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Yang J, Steffens A, Olson AL, Anderson A, Basra G, Veeranki P, de Andrade JA. Supplemental oxygen therapy use among patients with fibrosing interstitial lung disease in the United States. Respir Res 2025; 26:80. [PMID: 40022082 PMCID: PMC11871663 DOI: 10.1186/s12931-025-03139-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2024] [Accepted: 02/06/2025] [Indexed: 03/03/2025] Open
Abstract
BACKGROUND Supplemental oxygen therapy is commonly prescribed in clinical practice for patients with fibrosing interstitial lung disease (ILD) to reduce breathlessness and increase physical capacity. Only a few studies have evaluated the incidence of oxygen therapy use, with evidence lacking in its use among fibrosing ILD subtypes including patients with idiopathic pulmonary fibrosis (IPF) and non-IPF ILD. This study aimed to estimate incidence of oxygen therapy and factors associated with oxygen therapy initiation. METHODS This non-interventional study used US administrative claims and electronic health record data from 01 October 2015 to 30 June 2022. Patients aged ≥ 18 years with newly diagnosed fibrosing ILD (≥ 2 fibrosing ILD diagnoses in any position on different dates of service within 365 days) were included; the index date was the first date with ILD diagnosis. Patients were followed until the earlier of health plan disenrollment, death, or end of study period. Oxygen therapy use was evaluated among patients without evidence of oxygen therapy before the index date, stratified by the underlying fibrosing disease (i.e., IPF vs. non-IPF ILD). Factors associated with oxygen therapy use were evaluated using Cox proportional hazards regression. RESULTS A total of 114,921 patients (IPF n = 5,555; non-IPF ILD n = 109,366) newly diagnosed with fibrosing ILD were included in the study. The mean (standard deviation) age of patients with ILD was 66.9 (14.2) years, and 47.2% were male. Patients were followed for a mean of 24 months after ILD diagnosis, during which 38% of fibrosing ILD patients initiated oxygen therapy; a higher proportion of patients with IPF initiated oxygen therapy compared to those with non-IPF ILD (68% and 36%, respectively). Factors associated with oxygen therapy initiation included IPF, higher Charlson comorbidity scores, and comorbidities that impair respiratory capacity. CONCLUSIONS The study findings demonstrate a substantial proportion of patients with fibrosing ILD initiated oxygen therapy following initial ILD diagnosis, with higher rates of oxygen therapy initiation observed among patients with IPF compared with non-IPF ILD. Respiratory comorbidities were key factors associated with increased initiation of oxygen therapy.
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Affiliation(s)
- Joseph Yang
- Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA.
| | | | - Amy L Olson
- Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA
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2
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Matteson EL, Bendstrup E, Strek ME, Dieudé P. Clinical Course of Interstitial Lung Disease in Patients With Rheumatoid Arthritis. ACR Open Rheumatol 2024; 6:836-845. [PMID: 39243209 PMCID: PMC11638131 DOI: 10.1002/acr2.11736] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2023] [Revised: 03/13/2024] [Accepted: 03/22/2024] [Indexed: 09/09/2024] Open
Abstract
Interstitial lung disease (ILD) is a frequent manifestation of rheumatoid arthritis (RA) that is associated with high mortality. RA-ILD may initially be asymptomatic, and lung function may be markedly impaired by the time it is diagnosed. The course of RA-ILD is highly variable, with some patients experiencing no discernable progression or a slow decline, whereas others experience more rapid deterioration. Some patients develop progressive pulmonary fibrosis, which is associated with high mortality. Although risk factors for the progression of RA-ILD have been identified, including older age, worse lung function, and a usual interstitial pneumonia pattern on high-resolution computed tomography, it is not possible to predict the course of RA-ILD in an individual patient. The association between RA disease activity and progression of RA-ILD remains unclear. Regular monitoring is important to enable the prompt identification of progression and early intervention to preserve lung function. The management of RA-ILD requires a multidisciplinary and individualized approach, taking account of the severity and progression of articular and lung disease, risk factors for the progression of RA-ILD, and the patient's preferences, and may include immunosuppression, antifibrotic therapy, and supportive care.
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Affiliation(s)
| | | | | | - Philippe Dieudé
- Assistance Publique‐Hôpitaux de Paris Cité, Bichat‐Claude Bernard University Hospital, INSERM UMR1152, University of ParisParisFrance
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3
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Menson KE, Dowman L. Pulmonary Rehabilitation for Diseases Other Than COPD. J Cardiopulm Rehabil Prev 2024; 44:425-431. [PMID: 39388147 DOI: 10.1097/hcr.0000000000000915] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/15/2024]
Abstract
Review the current literature regarding pulmonary rehabilitation (PR) for non-chronic obstructive pulmonary disease (COPD) diagnoses and what the evidence is regarding expected outcomes based on disease manifestations. Literature search was performed using PubMed database from March 2024 to June 2024. Terms included "pulmonary rehabilitation" and "exercise training" in conjunction with key words "interstitial lung disease (ILD)," "idiopathic pulmonary fibrosis," "asthma," "bronchiectasis," "post-acute sequalae of SARS-CoV-2 (PASC)," "long COVID," "pulmonary hypertension (PH)," and "lung cancer." Results were filtered for English language, randomized controlled trial, clinical trial, observational trial, meta-analysis, and guidelines. Emphasis was placed on more recent publications since prior reviews, where applicable. The abundance of literature involved ILD, where studies have demonstrated significant improvements in exercise capacity, health-related quality of life (HRQoL), and dyspnea, despite heterogeneity of diseases; benefits are similar to those seen with COPD. Those with milder disease have more sustained benefits longer term. Patients with asthma benefit in severe disease, lower exercise activity, elevated body mass index, or when comorbid conditions are present, and breathing exercises can improve symptoms of breathlessness. Patients with PASC have a multitude of symptoms and lack benefits in HRQoL measurements; PR improves performance on post-COVID-19 functional status scale, a more comprehensive measurement of symptoms. Those with bronchiectasis benefit from PR when airflow limitation or exacerbations are impacting symptoms and HRQoL. Those with stable PH can improve their exertional capacity without change in disease severity. PR reduces perioperative complications in those with lung cancer and preserve fitness during treatment.
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Affiliation(s)
- Katherine E Menson
- Author Affiliations: Division of Pulmonary and Critical Care Medicine, University of Vermont, Burlington, Vermont (Dr Menson); and Respiratory Care Group, Monash University, Melbourne, Victoria, Australia (Dr Dowman)
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Zhang X, Igor B, Elena D, Olga R, Glazachev O. Prevalence of occupational hypersensitivity pneumonitis: a systematic review and meta-analysis. INTERNATIONAL JOURNAL OF ENVIRONMENTAL HEALTH RESEARCH 2024; 34:3891-3908. [PMID: 38544315 DOI: 10.1080/09603123.2024.2333021] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/27/2024] [Accepted: 03/16/2024] [Indexed: 10/19/2024]
Abstract
In this meta-analysis, we aimed to evaluate the prevalence of occupational hypersensitivity pneumonitis (OHP) among different occupations globally. Our search was conducted on MEDLINE via PubMed, Scopus, Web of Science, and Cochrane CENTRAL from inception to September 2023. Eligible studies were observational in nature and focused on several specific occupations. A total of 46 articles were included (n = 2,826,420 participants). The overall prevalence of OHP was found to be 4.2% (95% CI: 2.1% to 8.0%), but this varied significantly based on occupation and geographic location. Printers had the highest OHP prevalence at 57.14%, followed by tobacco workers (26.32%), and water-related workers (24.10%). South America showed the highest prevalence of 16.71%, compared to Asia (15.19%), and North America (8.52%). Significant variations in OHP prevalence by occupation and region were found, with the highest rates in printers and tobacco workers. Age and smoking were identified as contributing factors to the prevalence variability.
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Affiliation(s)
- Xinliang Zhang
- Department of Normal Physiology, I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
| | - Bukhtiyarov Igor
- Department of Normal Physiology, I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
- Department of Occupational Medicine, Izmerov Research Institute of Occupational Health, Moscow, Russia
| | - Dudnik Elena
- Department of Normal Physiology, I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
| | - Rumyantseva Olga
- Department of Occupational Medicine, Izmerov Research Institute of Occupational Health, Moscow, Russia
| | - Oleg Glazachev
- Department of Normal Physiology, I.M.Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
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Mohammed SM, Al-Saedi HFS, Mohammed AQ, Amir AA, Radi UK, Sattar R, Ahmad I, Ramadan MF, Alshahrani MY, Balasim HM, Alawadi A. Mechanisms of Bleomycin-induced Lung Fibrosis: A Review of Therapeutic Targets and Approaches. Cell Biochem Biophys 2024; 82:1845-1870. [PMID: 38955925 DOI: 10.1007/s12013-024-01384-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/21/2024] [Indexed: 07/04/2024]
Abstract
Pulmonary toxicity is a serious side effect of some specific anticancer drugs. Bleomycin is a well-known anticancer drug that triggers severe reactions in the lungs. It is an approved drug that may be prescribed for the treatment of testicular cancers, Hodgkin's and non-Hodgkin's lymphomas, ovarian cancer, head and neck cancers, and cervical cancer. A large number of experimental studies and clinical findings show that bleomycin can concentrate in lung tissue, leading to massive oxidative stress, alveolar epithelial cell death, the proliferation of fibroblasts, and finally the infiltration of immune cells. Chronic release of pro-inflammatory and pro-fibrotic molecules by immune cells and fibroblasts leads to pneumonitis and fibrosis. Both fibrosis and pneumonitis are serious concerns for patients who receive bleomycin and may lead to death. Therefore, the management of lung toxicity following cancer therapy with bleomycin is a critical issue. This review explains the cellular and molecular mechanisms of pulmonary injury following treatment with bleomycin. Furthermore, we review therapeutic targets and possible promising strategies for ameliorating bleomycin-induced lung injury.
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Affiliation(s)
- Shaimaa M Mohammed
- Department of Pharmacy, Al- Mustaqbal University College, 51001, Hilla, Babylon, Iraq
| | | | | | - Ahmed Ali Amir
- Department of Medical Laboratories Technology, Al-Nisour University College, Baghdad, Iraq
| | - Usama Kadem Radi
- College of Pharmacy, National University of Science and Technology, Nasiriyah, Dhi Qar, Iraq
| | - Ruaa Sattar
- Al-Hadi University College, Baghdad, 10011, Iraq
| | - Irfan Ahmad
- Department of Clinical Laboratory Sciences, College of Applied Medical Sciences, King Khalid University, Abha, Saudi Arabia
| | | | - Mohammad Y Alshahrani
- Department of Clinical Laboratory Sciences, College of Applied Medical Sciences, King Khalid University, Abha, Saudi Arabia.
| | - Halah Majeed Balasim
- Department of Medical Laboratory Technologies, Al Rafidain University College, Bagdad, Iraq
| | - Ahmed Alawadi
- College of technical engineering, the Islamic University, Najaf, Iraq
- College of technical engineering, the Islamic University of Al Diwaniyah, Al Diwaniyah, Iraq
- College of technical engineering, the Islamic University of Babylon, Hilla, Iraq
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6
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Aronson KI, Rajan M, Varadarajan J, Paul TK, Swigris JJ, Krishnan JK, Kaner RJ, Martinez FJ, Safford MM, Pinheiro LC. Development and initial validation of a disease-specific instrument to measure health-related quality of life in hypersensitivity pneumonitis. ERJ Open Res 2024; 10:00155-2024. [PMID: 39104956 PMCID: PMC11299010 DOI: 10.1183/23120541.00155-2024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2024] [Accepted: 04/10/2024] [Indexed: 08/07/2024] Open
Abstract
Rationale and objective Disease-specific health-related quality of life (HRQOL) instruments enable us to capture domains that are most relevant to specific patient populations and are useful when a more individualised approach to patient assessment is desired. In this study, we assessed the validity and reliability of the first instrument specifically developed to measure HRQOL in hypersensitivity pneumonitis (HP). Methods A 39-item HP-HRQOL instrument and several anchors were collected from a cohort of patients with HP. Exploratory factor analysis and item reduction were utilised to construct a shortened version of the instrument. Several validity and reliability analyses were conducted on this version of the HP-HRQOL. Measurements and main results 59 patients with HP completed the study. The revised HP-HRQOL instrument comprises 15 items composing two factors (domains): 1) impacts on daily life; and 2) mental wellbeing. Internal consistency reliability was strong for Factor 1 (Cronbach's α=0.94, 95% CI 0.92-0.96) and Factor 2 (Cronbach's α=0.89, 95% CI 0.85-0.94). Test-retest reliability was strong (ICC 0.94, 95% CI 0.89-0.97). The HP-HRQOL strongly correlated with other validated patient-reported outcome measures and moderately correlated with % predicted forced vital capacity. The HP-HRQOL distinguished between those with different severities of HP as determined by lung function and supplemental oxygen use. Conclusions The HP-HRQOL, the first patient-reported outcome instrument specific to adults with HP, possesses strong validity and reliability characteristics for measuring disease-specific HRQOL and distinguishes among patients with different severities of disease.
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Affiliation(s)
- Kerri I. Aronson
- Division of Pulmonary and Critical Care, Weill Cornell Medicine, New York, NY, USA
| | - Mangala Rajan
- Division of General Internal Medicine, Department of Medicine, Weill Cornell Medicine, New York, NY, USA
| | - Janani Varadarajan
- Division of Pulmonary and Critical Care, Weill Cornell Medicine, New York, NY, USA
| | - Tessy K. Paul
- Division of Pulmonary and Critical Care Medicine, University of Virginia, Charlottesville, VA, USA
| | - Jeffrey J. Swigris
- Center for Interstitial Lung Disease, National Jewish Health, Denver, CO, USA
| | - Jamuna K. Krishnan
- Division of Pulmonary and Critical Care, Weill Cornell Medicine, New York, NY, USA
| | - Robert J. Kaner
- Division of Pulmonary and Critical Care, Weill Cornell Medicine, New York, NY, USA
- Department of Genetic Medicine, Weill Cornell Medicine New York, NY, USA
| | - Fernando J. Martinez
- Division of Pulmonary and Critical Care, Weill Cornell Medicine, New York, NY, USA
| | - Monika M. Safford
- Division of General Internal Medicine, Department of Medicine, Weill Cornell Medicine, New York, NY, USA
| | - Laura C. Pinheiro
- Division of General Internal Medicine, Department of Medicine, Weill Cornell Medicine, New York, NY, USA
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Amin R, Vaishali K, Maiya GA, Mohapatra AK, Sinha MK, Bhat A, Bommasamudram T, Acharya V, Gore S. Patient education for individuals with Interstitial Lung Disease: A scoping review. F1000Res 2024; 13:405. [PMID: 38895701 PMCID: PMC11184280 DOI: 10.12688/f1000research.147340.1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/12/2024] [Indexed: 06/21/2024] Open
Abstract
Objectives Interstitial Lung Disease (ILD) is a severe and rapidly progressing disease with a high fatality rate. Patient education (PE) has been demonstrated to promote long-term adherence to exercise and lifestyle improvements by assisting patients in developing self-management techniques. Our scoping review's goal was to chart out the prevailing level of research about the content, processes, and effectiveness of PE for patients with ILD. Methods The relevant databases were searched using the rules provided by Arksey and O'Malley in 2005 and the Joanna Briggs Institute reviewers' manual 2015: an approach for JBI scoping reviews. Individuals with ILD, published in English between the years of inception and 2020, and describing PE administered by various healthcare practitioners were among the 355 studies found and reviewed. Thirteen studies met these criteria. Results PE delivery process, delivery techniques, quality of life assessments, common PE themes, and healthcare professional participation were all recognized and cataloged. Conclusion Despite the fact that healthcare professionals (physicians, nurses, and physiotherapists) provide PE to patients with ILD regularly, the PE provided varies greatly (contents of PE, process of delivery and delivery techniques). During the scoping review, a significant variation in the themes was addressed. They could not provide any evidence-based specific recommendations for all healthcare practitioners due to the studies' heterogeneity and lack of effectiveness measures.
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Affiliation(s)
- Revati Amin
- Physiotherapy, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India
| | - K. Vaishali
- Physiotherapy, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India
| | - G. Arun Maiya
- Physiotherapy, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India
| | - Aswini Kumar Mohapatra
- Respiratory Medicine, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India
| | - Mukesh Kumar Sinha
- Physiotherapy, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India
| | - Anup Bhat
- Physiotherapy, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India
| | - Tulasiram Bommasamudram
- Exercise and Sports Science, Manipal College of Health Professions, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India
| | - Vishak Acharya
- Pulmonary Medicine, Kasturba Medical College, Manipal Academy of Higher Education, Mangalore, Karnataka, 575001, India
| | - Shweta Gore
- MGH Institute of Health Professions, Boston, Massachusetts, 02129, USA
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Mackintosh JA, Keir G, Troy LK, Holland AE, Grainge C, Chambers DC, Sandford D, Jo HE, Glaspole I, Wilsher M, Goh NSL, Reynolds PN, Chapman S, Mutsaers SE, de Boer S, Webster S, Moodley Y, Corte TJ. Treatment of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis: A position statement from the Thoracic Society of Australia and New Zealand 2023 revision. Respirology 2024; 29:105-135. [PMID: 38211978 PMCID: PMC10952210 DOI: 10.1111/resp.14656] [Citation(s) in RCA: 6] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2023] [Accepted: 12/11/2023] [Indexed: 01/13/2024]
Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive disease leading to significant morbidity and mortality. In 2017 the Thoracic Society of Australia and New Zealand (TSANZ) and Lung Foundation Australia (LFA) published a position statement on the treatment of IPF. Since that time, subsidized anti-fibrotic therapy in the form of pirfenidone and nintedanib is now available in both Australia and New Zealand. More recently, evidence has been published in support of nintedanib for non-IPF progressive pulmonary fibrosis (PPF). Additionally, there have been numerous publications relating to the non-pharmacologic management of IPF and PPF. This 2023 update to the position statement for treatment of IPF summarizes developments since 2017 and reaffirms the importance of a multi-faceted approach to the management of IPF and progressive pulmonary fibrosis.
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Affiliation(s)
- John A. Mackintosh
- Department of Respiratory MedicineThe Prince Charles HospitalBrisbaneQueenslandAustralia
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
| | - Gregory Keir
- Department of Respiratory MedicinePrincess Alexandra HospitalBrisbaneQueenslandAustralia
| | - Lauren K. Troy
- Department of Respiratory and Sleep MedicineRoyal Prince Alfred HospitalCamperdownNew South WalesAustralia
- University of SydneySydneyNew South WalesAustralia
| | - Anne E. Holland
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
- Department of PhysiotherapyThe Alfred HospitalMelbourneVictoriaAustralia
- Department of Respiratory Research@AlfredCentral Clinical School, Monash UniversityMelbourneVictoriaAustralia
| | - Christopher Grainge
- Department of Respiratory MedicineJohn Hunter HospitalNewcastleNew South WalesAustralia
| | - Daniel C. Chambers
- Department of Respiratory MedicineThe Prince Charles HospitalBrisbaneQueenslandAustralia
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
| | - Debra Sandford
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
- Department of Thoracic MedicineCentral Adelaide Local Health NetworkAdelaideSouth AustraliaAustralia
- University of AdelaideAdelaideSouth AustraliaAustralia
| | - Helen E. Jo
- Department of Respiratory and Sleep MedicineRoyal Prince Alfred HospitalCamperdownNew South WalesAustralia
- University of SydneySydneyNew South WalesAustralia
| | - Ian Glaspole
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
- Department of Respiratory MedicineThe Alfred HospitalMelbourneVictoriaAustralia
| | - Margaret Wilsher
- Department of Respiratory MedicineTe Toka Tumai AucklandAucklandNew Zealand
| | - Nicole S. L. Goh
- Department of Respiratory MedicineAustin HospitalMelbourneVictoriaAustralia
- Institute for Breathing and SleepMelbourneVictoriaAustralia
- University of MelbourneMelbourneVictoriaAustralia
| | - Paul N. Reynolds
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
- Department of Thoracic MedicineCentral Adelaide Local Health NetworkAdelaideSouth AustraliaAustralia
- University of AdelaideAdelaideSouth AustraliaAustralia
| | - Sally Chapman
- Institute for Respiratory Health, University of Western AustraliaNedlandsWestern AustraliaAustralia
| | - Steven E. Mutsaers
- Department of Respiratory MedicineFiona Stanley HospitalMurdochWestern AustraliaAustralia
| | - Sally de Boer
- Department of Respiratory MedicineTe Toka Tumai AucklandAucklandNew Zealand
| | - Susanne Webster
- Department of Respiratory and Sleep MedicineRoyal Prince Alfred HospitalCamperdownNew South WalesAustralia
| | - Yuben Moodley
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
- Institute for Respiratory Health, University of Western AustraliaNedlandsWestern AustraliaAustralia
- Department of Respiratory MedicineFiona Stanley HospitalMurdochWestern AustraliaAustralia
| | - Tamera J. Corte
- Centre of Research Excellence in Pulmonary FibrosisCamperdownNew South WalesAustralia
- Department of Respiratory and Sleep MedicineRoyal Prince Alfred HospitalCamperdownNew South WalesAustralia
- University of SydneySydneyNew South WalesAustralia
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9
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Lee JYT, Tikellis G, Dowman L, Jones AW, Hoffman M, Mellerick CR, Malaguti C, Khor YH, Holland AE. Self-management interventions for people with pulmonary fibrosis: a scoping review. Eur Respir Rev 2023; 32:230092. [PMID: 37914193 PMCID: PMC10618910 DOI: 10.1183/16000617.0092-2023] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2023] [Accepted: 09/04/2023] [Indexed: 11/03/2023] Open
Abstract
BACKGROUND The most effective method for encouraging self-management in individuals with pulmonary fibrosis (PF) is unclear. This review aimed to identify common self-management components, the outcome measures used and the impact of these components in PF. METHODS A scoping review was conducted according to the Joanna Briggs Institute Manual for Evidence Synthesis using Medline, Embase, PsychInfo, CINAHL and the Cochrane Central Register of Controlled Trials. Eligible studies included those with educational, behavioural or support components aimed at facilitating self-management among adults with PF and employed quantitative and/or qualitative methods. RESULTS 87 studies were included. Common self-management components included education (78%), managing physical symptoms (66%) and enhancing psychosocial wellbeing (54%). Components were predominantly delivered in a pulmonary rehabilitation setting (71%). No studies tested a PF-specific self-management package. Common outcome measures were 6-min walk distance (60%), St George's Respiratory Questionnaire (37%) and the Medical Research Council Dyspnoea scale (34%). Clinically significant improvements in these outcomes were seen in ≥50% of randomised controlled trials. Qualitative data highlighted the importance of healthcare professional and peer support and increased confidence in managing PF. CONCLUSION Self-management components are commonly incorporated into pulmonary rehabilitation programmes rather than being offered as standalone packages. Future research should focus on testing PF-specific self-management packages and employ standardised outcome assessments that include self-efficacy and health-related behaviours.
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Affiliation(s)
- Joanna Y T Lee
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
| | - Gabriella Tikellis
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
| | - Leona Dowman
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Department of Respiratory and Sleep Medicine, Austin Health, Melbourne, Australia
| | - Arwel W Jones
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
| | - Mariana Hoffman
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Institute for Breathing and Sleep, Melbourne, Australia
| | - Christie R Mellerick
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Department of Physiotherapy, Alfred Health, Melbourne, Australia
| | - Carla Malaguti
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Federal University of Juiz de Fora, Juiz de Fora, Brazil
| | - Yet H Khor
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Department of Respiratory and Sleep Medicine, Austin Health, Melbourne, Australia
- Institute for Breathing and Sleep, Melbourne, Australia
- Department of Respiratory and Sleep Medicine, Alfred Health, Melbourne, Australia
| | - Anne E Holland
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Institute for Breathing and Sleep, Melbourne, Australia
- Department of Physiotherapy, Alfred Health, Melbourne, Australia
- Department of Respiratory and Sleep Medicine, Alfred Health, Melbourne, Australia
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10
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Koduri G, Solomon JJ. Identification, Monitoring, and Management of Rheumatoid Arthritis-Associated Interstitial Lung Disease. Arthritis Rheumatol 2023; 75:2067-2077. [PMID: 37395725 DOI: 10.1002/art.42640] [Citation(s) in RCA: 33] [Impact Index Per Article: 16.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/22/2022] [Revised: 05/23/2023] [Accepted: 06/27/2023] [Indexed: 07/04/2023]
Abstract
Interstitial lung disease (ILD) is a frequent complication of rheumatoid arthritis (RA) that is associated with a significant increase in mortality. Several risk factors for the development of ILD in patients with RA have been identified, but ILD can still develop in the absence of these risk factors. Screening tools for RA-ILD are required to facilitate early detection of RA-ILD. Close monitoring of patients with RA-ILD for progression is crucial to enable timely implementation of treatment strategies to improve outcomes. Patients with RA are commonly treated with immunomodulatory therapies, although their efficacy in slowing the progression of RA-ILD remains the subject of debate. Clinical trials have shown that antifibrotic therapies slow decline in lung function in patients with progressive fibrosing ILDs, including patients with RA-ILD. The management of patients with RA-ILD should be based on multidisciplinary evaluation of the severity and progression of their ILD and the activity of their articular disease. Close collaboration between rheumatologists and pulmonologists is essential to optimize patient care.
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Affiliation(s)
- Gouri Koduri
- Southend University Hospital NHS Foundation Trust, Chelmsford, UK
- Anglia Ruskin University, Chelmsford, UK
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11
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Løkke A, Castello L, Pinheiro Martins P, Soulard S, Hilberg O. Burden of Disease and Productivity Loss in the European Economic Area in Patients Affected by Fibrosing Interstitial Lung Disease. Adv Ther 2023; 40:5502-5518. [PMID: 37837527 PMCID: PMC10611590 DOI: 10.1007/s12325-023-02701-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2023] [Accepted: 09/29/2023] [Indexed: 10/16/2023]
Abstract
INTRODUCTION Progression of fibrosis in interstitial lung diseases (ILD) has been associated with poor prognosis, lower quality of life for patients and caregivers, and higher healthcare costs. This study estimated the burden of disease and productivity loss of progressively fibrosing ILD, focusing on progressive pulmonary fibrosis other than idiopathic pulmonary fibrosis (non-IPF PPF) and systemic sclerosis-associated ILD (SSc-ILD) in the European Economic Area (EEA). METHODS An economic model was built to estimate the clinical burden of SSc-ILD and non-IPF PPF. The model was based on published data on disease prevalence and disease burden (in terms of comorbidities, exacerbations, and deaths) as well as on productivity loss (in terms of sick days, early retirement, permanent disability, and job loss). Aggregate income loss was obtained by multiplying productivity loss by the median daily income in each country/area of investigation. A sensitivity analysis was performed to test the impact of the variability of the model assumptions. RESULTS In the whole EEA, a total of 86,794 and 13,221 individuals were estimated to be affected by non-IPF PPF and SSc-ILD, respectively. Estimated annual sick days associated with the diseases were 3,952,604 and 672,172, early retirements were 23,174 and 5341, permanently disabled patients were 41,748 and 4037, and job losses were 19,789 and 2617 for non-IPF PPF and SSc-ILD, respectively. Annual exacerbations were estimated to be 22,401-31,181 and 1259-1753, while deaths were 5791-6171 and 572-638 in non-IPF PPF and SSc-ILD, respectively. The estimated annual aggregate income loss in EEA, accounting for losses due to annual sick days, early retirements, and permanently disabled patients, was €1433 million and €220 million in non-IPF PPF and SSc-ILD, respectively. The productivity loss due to job losses was €194 million and €26 million in non-IPF PPF and SSc-ILD, respectively. The main driver of aggregate income loss variability was the prevalence. CONCLUSION The impact of non-IPF PPF and SSc-ILD on society is definitely non-negligible. Actions to reduce the burden on our societies are highly needed.
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Affiliation(s)
- Anders Løkke
- Department of Medicine, Lillebaelt Hospital, Vejle, Denmark
- Department of Regional Health Research, University of Southern Denmark, Odense, Denmark
| | | | | | | | - Ole Hilberg
- Department of Medicine, Lillebaelt Hospital, Vejle, Denmark
- Department of Regional Health Research, University of Southern Denmark, Odense, Denmark
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12
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Oliveira A, Fabbri G, Gille T, Bargagli E, Duchemann B, Evans R, Pinnock H, Holland AE, Renzoni E, Ekström M, Jones S, Wijsenbeek M, Dinh-Xuan AT, Vagheggini G. Holistic management of patients with progressive pulmonary fibrosis. Breathe (Sheff) 2023; 19:230101. [PMID: 37719243 PMCID: PMC10501708 DOI: 10.1183/20734735.0101-2023] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/11/2023] [Accepted: 07/18/2023] [Indexed: 09/19/2023] Open
Abstract
Progressive pulmonary fibrosis (PF) is a complex interstitial lung disease that impacts substantially on patients' daily lives, requiring personalised and integrated care. We summarise the main needs of patients with PF and their caregivers, and suggest a supportive care approach. Individualised care, education, emotional and psychological support, specialised treatments, and better access to information and resources are necessary. Management should start at diagnosis, be tailored to the patient's needs, and consider end-of-life care. Pharmacological and non-pharmacological interventions should be individualised, including oxygen therapy and pulmonary rehabilitation, with digital healthcare utilised as appropriate. Further research is needed to address technical issues related to oxygen delivery and digital healthcare. Educational aims To identify the main needs of patients with PF and their caregivers.To describe the components of a comprehensive approach to a supportive care programme for patients with PF.To identify further areas of research to address technical issues related to the management of patients with PF.
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Affiliation(s)
- Ana Oliveira
- Lab 3R Respiratory Research and Rehabilitation Laboratory, School of Health Sciences, University of Aveiro (ESSUA), IBMED Aveiro PT, Aveiro, Portugal
- School of Rehabilitation Science, Faculty of Health Science, McMaster University, Hamilton, ON, Canada
| | - Gaia Fabbri
- Respiratory Diseases and Lung Transplantation Unit, Department of Medical and Surgical Sciences and Neuro-Sciences, University of Siena, Siena, Italy
| | - Thomas Gille
- Inserm U1272 “Hypoxia & the Lung”, UFR SMBH Léonard de Vinci, Université Sorbonne Paris Nord, Bobigny, France
- Service Physiologie et Explorations Fonctionnelles, Hôpitaux Universitaires de Paris Seine-Saint-Denis, Assistance Publique – Hôpitaux de Paris, Bobigny, France
| | - Elena Bargagli
- Respiratory Diseases and Lung Transplantation Unit, Department of Medical and Surgical Sciences and Neuro-Sciences, University of Siena, Siena, Italy
| | - Boris Duchemann
- Inserm U1272 “Hypoxia & the Lung”, UFR SMBH Léonard de Vinci, Université Sorbonne Paris Nord, Bobigny, France
- Service d'oncologie médicale et thoracique, Hôpitaux Universitaires de Paris Seine-Saint-Denis, Assistance Publique – Hôpitaux de Paris, Bobigny, France
| | - Rachel Evans
- Department of Respiratory Sciences, University of Leicester, Glenfield Hospital, Leicester, UK
| | - Hilary Pinnock
- Allergy and Respiratory Research Group, Usher Institute, The University of Edinburgh, Edinburgh, UK
| | - Anne E. Holland
- Central Clinical School, Monash University and Department of Physiotherapy, Alfred Health, Melbourne, Australia
| | - Elisabetta Renzoni
- Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas’ NHS Foundation Trust, London, UK
- Margaret Turner Warwick Centre for Fibrosing Lung Diseases, NHLI, Imperial College, London, UK
| | - Magnus Ekström
- Lund University, Faculty of Medicine, Department of Clinical Sciences Lund, Respiratory Medicine, Allergology and Palliative Medicine, Lund, Sweden
| | - Steve Jones
- European Pulmonary Fibrosis Federation, Brussels, Belgium
| | - Marlies Wijsenbeek
- Centre for Expertise for Interstitial Lung Disease and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands
| | - Anh Tuan Dinh-Xuan
- Service de Physiologie-Explorations Fonctionnelles, Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Université Paris Cité, Paris, France
| | - Guido Vagheggini
- Department of Internal Medicine and Medical Specialties, Respiratory Failure Pathway, Azienda USL Toscana Nordovest, Pisa, Italy
- Fondazione Volterra Ricerche ONLUS, Volterra (PI), Italy
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13
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Zeng Q, Jiang D. Global trends of interstitial lung diseases from 1990 to 2019: an age-period-cohort study based on the Global Burden of Disease study 2019, and projections until 2030. Front Med (Lausanne) 2023; 10:1141372. [PMID: 37554509 PMCID: PMC10404716 DOI: 10.3389/fmed.2023.1141372] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2023] [Accepted: 06/19/2023] [Indexed: 08/10/2023] Open
Abstract
BACKGROUND Interstitial lung diseases (ILDs) are indispensable components of chronic respiratory diseases and global health challenges. We aimed to explore the global long-term changes in the prevalence, mortality, and disability-adjusted life years (DALYs) of ILDs; investigate the independent effect of age, period, and cohort; and project the disease burden over the next decade. METHODS Data were retrieved from the Global Burden of Disease (GBD) database 2019. The joinpoint regression model was used to calculate the average annual percent change (AAPC). An age-period-cohort (APC) analysis was employed to measure the independent effect of age, period, and cohort. The Bayesian age-period-cohort (BAPC) model was used to project the global epidemiological trends until 2030. RESULTS From 1990 to 2019, the age-standardized prevalence rate (ASPR), age-standardized mortality rate (ASMR), and age-standardized disability-adjusted life years (DALYs) rate (ASDR) of interstitial lung disease and pulmonary sarcoidosis (ILD) slightly increased from 52.66 per 100,000 [95% uncertainty interval (UI) 44.49 to 61.07] to 57.62 per 100,000 (95% UI 49.42 to 65.67), from 1.76 per 100,000 (95% UI 1.41 to 2.22) to 2.17 per 100,000 (95% UI 1.5 to 2.62), and from 41.57 per 100,000 (95% UI 33.93 to 51.92) to 46.45 per 100,000 (95% UI 35.12 to 54.98), whereas the ASPR, ASMR, and ASDR of pneumoconiosis decreased. High social-demographic index (SDI) regions possessed the highest ASPR, whereas low-middle SDI regions had the highest ASMR and ASDR, followed by low-SDI regions in ILD. Middle-SDI regions reported the highest ASPR, ASMR, and ASDR in pneumoconiosis. The age effect showed that the rate ratio (RR) was high in older adults. Period effect indicated that the RR of prevalence increased over time, whereas the RR of mortality and DALYs decreased in men but increased in women. The cohort effect exhibited that the more recent birth cohort had a higher RR than the previous cohort in prevalence. We projected that ASPR, ASMR, and ASDR would stabilize with little variation over the next decade. CONCLUSION The global burden of ILDs remains relatively severe, especially among older adults, in low- and middle-SDI regions. Effective measurements are expected to improve this situation.
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Aronson KI, Martin-Schwarze AM, Swigris JJ, Kolenic G, Krishnan JK, Podolanczuk AJ, Kaner RJ, Martinez FJ, Safford MM, Pinheiro LC. Validity and Reliability of the Fatigue Severity Scale in a Real-World Interstitial Lung Disease Cohort. Am J Respir Crit Care Med 2023; 208:188-195. [PMID: 37099412 PMCID: PMC10395489 DOI: 10.1164/rccm.202208-1504oc] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2022] [Accepted: 04/25/2023] [Indexed: 04/27/2023] Open
Abstract
Rationale: Fatigue is a common and debilitating symptom for people living with interstitial lung disease (ILD). Studies on fatigue in ILD are limited, and little headway has been made toward developing interventions targeting the alleviation of fatigue. A barrier to progress is a lack of knowledge around the performance characteristics of a patient-reported outcome measure to assess fatigue in patients with ILD. Objectives: To assess the validity and reliability of the Fatigue Severity Scale (FSS) for measuring fatigue in a national cohort of patients with ILD. Methods: FSS scores and several anchors were measured in 1,881 patients from the Pulmonary Fibrosis Foundation Patient Registry. Anchors included the Short Form 6D Health Utility Index (SF-6D) score and a single vitality question from the SF-6D; the University of California, San Diego, Shortness of Breath Questionnaire; FVC; DlCO; and 6-minute-walk distance. Internal consistency reliability, concurrent validity, and known-groups validity were assessed. Structural validity was assessed using confirmatory factor analysis. Measurements and Main Results: The FSS demonstrated high internal consistency (Cronbach's α = 0.96). There were moderate to strong correlations between FSS score and patient-reported anchors (vitality question from the SF-6D [r = 0.55] and University of California, San Diego, Shortness of Breath Questionnaire total score [r = 0.70]) and weak correlations between FSS score and physiological measures (FVC [r = -0.24], percentage predicted DlCO [r = -0.23], and 6-minute-walk distance [r = -0.29]). Higher mean FSS scores, indicating greater fatigue, were observed among patients using supplemental oxygen, those prescribed steroids, and those with lower percentage predicted FVC and percentage predicted DlCO. The confirmatory factor analysis results suggest that the nine questions of the FSS reflect one dimension of fatigue. Conclusions: Fatigue is an important patient-centered outcome in ILD that is poorly correlated with physiological measures of disease severity, including lung function and walk distance. These findings further support the need for a reliable and valid measure of patient-reported fatigue in ILD. The FSS possesses acceptable performance characteristics for assessing fatigue and distinguishing different degrees of fatigue among patients with ILD.
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Affiliation(s)
| | - Adam M. Martin-Schwarze
- Statistical Analysis of Biomedical and Educational Research Group, University of Michigan, Ann Arbor, Michigan; and
| | | | - Giselle Kolenic
- Statistical Analysis of Biomedical and Educational Research Group, University of Michigan, Ann Arbor, Michigan; and
| | | | | | - Robert J. Kaner
- Division of Pulmonary and Critical Care
- Department of Genetic Medicine, and
| | | | - Monika M. Safford
- Division of General Internal Medicine, Weill Cornell Medical College, New York, New York
| | - Laura C. Pinheiro
- Division of General Internal Medicine, Weill Cornell Medical College, New York, New York
| | - the Pulmonary Fibrosis Foundation
- Division of Pulmonary and Critical Care
- Department of Genetic Medicine, and
- Division of General Internal Medicine, Weill Cornell Medical College, New York, New York
- Statistical Analysis of Biomedical and Educational Research Group, University of Michigan, Ann Arbor, Michigan; and
- National Jewish Health, Denver, Colorado
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15
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Khor YH, Cottin V, Holland AE, Inoue Y, McDonald VM, Oldham J, Renzoni EA, Russell AM, Strek ME, Ryerson CJ. Treatable traits: a comprehensive precision medicine approach in interstitial lung disease. Eur Respir J 2023; 62:2300404. [PMID: 37263752 PMCID: PMC10626565 DOI: 10.1183/13993003.00404-2023] [Citation(s) in RCA: 15] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/08/2023] [Accepted: 05/16/2023] [Indexed: 06/03/2023]
Abstract
Interstitial lung disease (ILD) is a diverse group of inflammatory and fibrotic lung conditions causing significant morbidity and mortality. A multitude of factors beyond the lungs influence symptoms, health-related quality of life, disease progression and survival in patients with ILD. Despite an increasing emphasis on multidisciplinary management in ILD, the absence of a framework for assessment and delivery of comprehensive patient care poses challenges in clinical practice. The treatable traits approach is a precision medicine care model that operates on the premise of individualised multidimensional assessment for distinct traits that can be targeted by specific interventions. The potential utility of this approach has been described in airway diseases, but has not been adequately considered in ILD. Given the similar disease heterogeneity and complexity between ILD and airway diseases, we explore the concept and potential application of the treatable traits approach in ILD. A framework of aetiological, pulmonary, extrapulmonary and behavioural and lifestyle treatable traits relevant to clinical care and outcomes for patients with ILD is proposed. We further describe key research directions to evaluate the application of the treatable traits approach towards advancing patient care and health outcomes in ILD.
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Affiliation(s)
- Yet H Khor
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Department of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Australia
- Institute for Breathing and Sleep, Heidelberg, Australia
- Faculty of Medicine, University of Melbourne, Melbourne, Australia
| | - Vincent Cottin
- National Coordinating Reference Centre for Rare Pulmonary Diseases, OrphaLung, Louis Pradel Hospital, Hospices Civils de Lyon, ERN-LUNG, Lyon, France
- UMR 754, Claude Bernard University Lyon 1, INRAE, Lyon, France
| | - Anne E Holland
- Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia
- Institute for Breathing and Sleep, Heidelberg, Australia
- Department of Respiratory and Sleep Medicine, Alfred Health, Melbourne, Australia
- Department of Physiotherapy, Alfred Health, Melbourne, Australia
| | - Yoshikazu Inoue
- Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai City, Japan
| | - Vanessa M McDonald
- National Health and Medical Research Council Centre for Research Excellence in Treatable Traits, New Lambton Heights, Australia
- Asthma and Breathing Research Centre, Hunter Medical Research Institute, New Lambton Heights, Australia
- School of Nursing and Midwifery, College of Health, Medicine and Wellbeing, The University of Newcastle, Callaghan, Australia
| | - Justin Oldham
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA
- Department of Epidemiology, University of Michigan, Ann Arbor, MI, USA
| | - Elisabetta A Renzoni
- Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK
- Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK
| | - Anne Marie Russell
- Exeter Respiratory Innovation Centre, University of Exeter, Exeter, UK
- Royal Devon University Hospitals, NHS Foundation Trust, Devon, UK
- Faculty of Medicine, Imperial College Healthcare NHS Trust, London, UK
| | - Mary E Strek
- Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL, USA
| | - Christopher J Ryerson
- Department of Medicine, University of British Columbia, Vancouver, BC, Canada
- Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada
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16
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Karampitsakos T, Wijsenbeek M, Herazo-Maya JD, Tzouvelekis A, Kreuter M. Interstitial lung diseases: an overview. RARE DISEASES OF THE RESPIRATORY SYSTEM 2023. [DOI: 10.1183/2312508x.10017322] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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17
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Farrell J, Ho L. Management of Patients with Systemic Sclerosis-Associated Interstitial Lung Disease: A Focus on the Role of the Pharmacist. INTEGRATED PHARMACY RESEARCH AND PRACTICE 2023; 12:101-112. [PMID: 37163188 PMCID: PMC10164394 DOI: 10.2147/iprp.s399518] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2022] [Accepted: 03/23/2023] [Indexed: 05/11/2023] Open
Abstract
Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc), which becomes fibrosing and progressive in some patients. Regular monitoring of patients with SSc-ILD is important to assess progression and inform treatment decisions. Therapy for SSc-ILD may include immunomodulatory and antifibrotic therapies. Therapeutic decisions should be made on a case-by-case basis, ideally following multidisciplinary discussion. Most patients with SSc-ILD have several organ manifestations of SSc or comorbidities and are taking a complex medication regimen. Patients with SSc are particularly susceptible to gastrointestinal side-effects of medications due to the gastrointestinal manifestations of the disease. Pharmacists play an important role in the management of patients with SSc-ILD by assisting patients with access to medications, optimizing medication regimens, and advising on alternative dosage forms. Pharmacists can also contribute to patient education to help patients better understand their treatment and how to prevent and manage potential side effects.
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Affiliation(s)
- Jessica Farrell
- Albany College of Pharmacy and Health Sciences and Albany Medical Center Division of Rheumatology, Albany, NY, USA
| | - Lawrence Ho
- Center for Interstitial Lung Disease, University of Washington, Seattle, WA, USA
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18
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Paixão C, Almeida S, Ferreira PG, Mendes MA, Brooks D, Marques A. Lifestyle integrated functional exercise for people with interstitial lung disease (iLiFE): A mixed-methods feasibility study. Heart Lung 2023; 60:20-27. [PMID: 36878103 DOI: 10.1016/j.hrtlng.2023.02.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2022] [Revised: 02/15/2023] [Accepted: 02/19/2023] [Indexed: 03/07/2023]
Abstract
BACKGROUND People with interstitial lung disease (ILD) present low levels of physical activity (PA) and spend most of their time at home, especially in advanced stages of the disease. The Lifestyle Integrated Functional Exercise for people with ILD (iLiFE) embedding PA in patients' daily routines was developed and implemented. OBJECTIVES This study aimed to explore the feasibility of iLiFE. METHODS A pre/post mixed-methods feasibility study was conducted. Feasibility of iLiFE was determined by participant recruitment/retention, adherence, feasibility of outcome measures and adverse events. Measures of PA, sedentary behaviour, balance, muscle strength, functional performance/capacity, exercise capacity, impact of the disease, symptoms (i.e., dyspnoea, anxiety, depression, fatigue and cough) and health-related quality of life were collected at baseline and post-intervention (12-weeks). Semi-structured interviews with participants were conducted in-person immediately after iLiFE. Interviews were audio-recorded, transcribed and analysed by deductive thematic analysis. RESULTS Ten participants (5♀, 77±3y; FVCpp 77.1 ± 4.4, DLCOpp 42.4 ± 6.6) were included, but only nine completed the study. Recruitment was challenging (30%) and retention high (90%). iLiFE was feasible, with excellent adherence (84.4%) and no adverse events. Missing data were associated with one dropout and non-compliance with the accelerometer (n = 1). Participants reported that iLiFE contributed to (re)gain control in their daily life, namely through improving their well-being, functional status and motivation. Weather, symptoms, physical impairments and lack of motivation were identified as threats to keep an active lifestyle. CONCLUSIONS iLiFE seems to be feasible, safe and meaningful for people with ILD. A randomised controlled trial is needed to strengthen these promising findings.
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Affiliation(s)
- Cátia Paixão
- Lab3R - Respiratory Research and Rehabilitation Laboratory, School of Health Sciences, University of Aveiro (ESSUA), Aveiro, Portugal; iBiMED - Institute of Biomedicine, University of Aveiro, Aveiro, Portugal; Department of Medical Sciences, University of Aveiro, Aveiro, Portugal
| | - Sara Almeida
- Lab3R - Respiratory Research and Rehabilitation Laboratory, School of Health Sciences, University of Aveiro (ESSUA), Aveiro, Portugal
| | - Pedro G Ferreira
- ILD Outpatient Clinic, Pulmonology Department - Centro Hospitalar Universitario de Coimbra (CHUC), Coimbra, Portugal; Faculty of Medicine of University of Coimbra (FMUC), Coimbra, Portugal- Coimbra, Portugal
| | - M Aurora Mendes
- Lab3R - Respiratory Research and Rehabilitation Laboratory, School of Health Sciences, University of Aveiro (ESSUA), Aveiro, Portugal; Pulmonology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal
| | - Dina Brooks
- School of Rehabilitation Science, McMaster University, Hamilton, Canada; West Park Healthcare Centre, Toronto, Canada - Hamilton, Canada
| | - Alda Marques
- Lab3R - Respiratory Research and Rehabilitation Laboratory, School of Health Sciences, University of Aveiro (ESSUA), Aveiro, Portugal; iBiMED - Institute of Biomedicine, University of Aveiro, Aveiro, Portugal.
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Kilgore K, Leinfelder J, Campbell J, Wayne PM, Hallowell RW, Barakat A. A Tai Chi Class Collaboratively Developed for Persons With Interstitial and Other Lung Diseases: An Ethnographic Investigation. GLOBAL ADVANCES IN INTEGRATIVE MEDICINE AND HEALTH 2023; 12:27536130231206122. [PMID: 37901846 PMCID: PMC10605663 DOI: 10.1177/27536130231206122] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 07/09/2023] [Revised: 09/02/2023] [Accepted: 09/18/2023] [Indexed: 10/31/2023]
Abstract
Background Participating in physical activity (PA) can be challenging for persons with chronic and significant lung disease due to the multifaceted disruptive effects of their symptoms and variable disease course. Objectives Our study investigates a novel approach to increasing PA by collaboratively and adaptively developing a Tai Chi (TC) class for and by persons with lung diseases and explores participants' perceptions of their experiences in the co-developed TC class. Methods We initiated a collaboration between the Interstitial Lung Disease (ILD) Collaborative and the Tai Chi Foundation to develop a TC class appropriate for persons with ILD and other lung diseases. The TC class was offered online, during the early phases of the COVID-19 pandemic, when pulmonary patients were isolated socially. TC class sessions were held twice weekly for 12 weeks with 12 participants. Ethnographic field methods were used to collect observations and conduct interviews with teachers and students. The Social Ecological Model (SEM) for understanding factors in intrapersonal, interpersonal, social, and organizational contexts was used to explore ways in which wellness practices, particularly those involving changes in health behaviors, can be collaboratively conceived, and developed by persons with the lived experience of illness and community organizations that are sensitive to their personal and social contexts. The constant comparative method was used for data analysis. Results Our findings include the importance of (1) creating a supportive class environment, characterized by interactive and reciprocal relationships among students and teachers; (2) alternating segments of movement and meditation to avoid fatigue and breathlessness; (3) cultivating sensory awareness and body trust, resting when needed and rejoining the movements when ready; (4) increasing the capacity to meditate through deepening presence and renewing the vital connection with inner and outer sources of energy; (5) reducing, through meditative movement, the persistent anxiety, isolation, and sense of loss that accompany chronic disease diagnosis and progression. Conclusion We documented a collaboration between the TC and pulmonary communities to design a TC class for persons with chronic and significant lung disease. We employed the SEM to provide insights into how teachers, informed by their students, can use effective pedagogical skills to create core curricula with modifications appropriate for a specific population.
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Affiliation(s)
- Karen Kilgore
- Interstitial Lung Disease Collaborative, Pulmonary Care and Research Collaborative, Boston, MA, USA
- Osher Center for Integrative Medicine, Brigham and Women’s Hospital, Boston, MA, USA
| | | | | | - Peter M. Wayne
- Osher Center for Integrative Medicine, Brigham and Women’s Hospital, Boston, MA, USA
- Harvard Medical School, Boston, MA, USA
| | - Robert W. Hallowell
- Harvard Medical School, Boston, MA, USA
- Division of Pulmonary and Critical Care Medicine, Massachusetts General Hospital, Boston, MA, USA
| | - Aliaa Barakat
- Interstitial Lung Disease Collaborative, Pulmonary Care and Research Collaborative, Boston, MA, USA
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20
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Patel H, Shah JR, Patel DR, Avanthika C, Jhaveri S, Gor K. Idiopathic pulmonary fibrosis: Diagnosis, biomarkers and newer treatment protocols. Dis Mon 2022:101484. [DOI: 10.1016/j.disamonth.2022.101484] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
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21
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Wijsenbeek M, Suzuki A, Maher TM. Interstitial lung diseases. Lancet 2022; 400:769-786. [PMID: 35964592 DOI: 10.1016/s0140-6736(22)01052-2] [Citation(s) in RCA: 177] [Impact Index Per Article: 59.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2021] [Revised: 03/14/2022] [Accepted: 06/03/2022] [Indexed: 02/07/2023]
Abstract
Over 200 interstitial lung diseases, from ultra rare to relatively common, are recognised. Most interstitial lung diseases are characterised by inflammation or fibrosis within the interstitial space, the primary consequence of which is impaired gas exchange, resulting in breathlessness, diminished exercise tolerance, and decreased quality of life. Outcomes vary considerably for each of the different interstitial lung diseases. In some conditions, spontaneous reversibility or stabilisation can occur, but unfortunately in many people with interstitial lung disease, especially in those manifesting progressive pulmonary fibrosis, respiratory failure and death are a sad reality. Over the past 3 years, the field of interstitial lung disease has had important advances, with the approval of drugs to treat systemic sclerosis-associated interstitial lung disease, interstitial lung disease-associated pulmonary hypertension, and different forms of progressive pulmonary fibrosis. This Seminar provides an update on epidemiology, pathogenesis, presentation, diagnosis, disease course, and management of the interstitial lung diseases that are most frequently encountered in clinical practice. Furthermore, we describe how developments have led to a shift in the classification and treatment of interstitial lung diseases that exhibit progressive pulmonary fibrosis and summarise the latest practice-changing guidelines. We conclude with an outline of controversies, uncertainties, and future directions.
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Affiliation(s)
- Marlies Wijsenbeek
- Center for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, Netherlands.
| | - Atsushi Suzuki
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Aichi, Japan
| | - Toby M Maher
- Hastings Centre for Pulmonary Research and Division of Pulmonary, Critical Care and Sleep Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA; National Heart and Lung Institute, Imperial College London, London, UK
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22
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Towards Treatable Traits for Pulmonary Fibrosis. J Pers Med 2022; 12:jpm12081275. [PMID: 36013224 PMCID: PMC9410230 DOI: 10.3390/jpm12081275] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2022] [Revised: 07/24/2022] [Accepted: 07/29/2022] [Indexed: 11/17/2022] Open
Abstract
Interstitial lung diseases (ILD) are a heterogeneous group of disorders, of which many have the potential to lead to progressive pulmonary fibrosis. A distinction is usually made between primarily inflammatory ILD and primarily fibrotic ILD. As recent studies show that anti-fibrotic drugs can be beneficial in patients with primarily inflammatory ILD that is characterized by progressive pulmonary fibrosis, treatment decisions have become more complicated. In this perspective, we propose that the ‘treatable trait’ concept, which is based on the recognition of relevant exposures, various treatable phenotypes (disease manifestations) or endotypes (shared molecular mechanisms) within a group of diseases, can be applied to progressive pulmonary fibrosis. These targets for medical intervention can be identified through validated biomarkers and are not necessarily related to specific diagnostic labels. Proposed treatable traits are: cigarette smoking, occupational, allergen or drug exposures, excessive (profibrotic) auto- or alloimmunity, progressive pulmonary fibrosis, pulmonary hypertension, obstructive sleep apnea, tuberculosis, exercise intolerance, exertional hypoxia, and anxiety and depression. There are also several potential traits that have not been associated with relevant outcomes or for which no effective treatment is available at present: air pollution, mechanical stress, viral infections, bacterial burden in the lungs, surfactant-related pulmonary fibrosis, telomere-related pulmonary fibrosis, the rs35705950 MUC5B promoter polymorphism, acute exacerbations, gastro-esophageal reflux, dyspnea, and nocturnal hypoxia. The ‘treatable traits’ concept can be applied in new clinical trials for patients with progressive pulmonary fibrosis and could be used for developing new treatment strategies.
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Physiotherapy management of interstitial lung disease. J Physiother 2022; 68:158-164. [PMID: 35753970 DOI: 10.1016/j.jphys.2022.06.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/06/2022] [Accepted: 06/13/2022] [Indexed: 11/24/2022] Open
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Khanna SA, Nance JW, Suliman SA. Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis. Curr Rheumatol Rep 2022; 24:166-173. [PMID: 35499699 PMCID: PMC9399070 DOI: 10.1007/s11926-022-01067-5] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/18/2022] [Indexed: 12/27/2022]
Abstract
PURPOSE OF REVIEW Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc). We explore the importance of early detection, monitoring, and management of SSc-ILD. RECENT FINDINGS All patients with SSc are at risk of ILD and should be screened for ILD at diagnosis using a high-resolution computed tomography (HRCT) scan. Some patients with SSc-ILD develop a progressive phenotype characterized by worsening fibrosis on HRCT, decline in lung function, and early mortality. To evaluate progression and inform treatment decisions, regular monitoring is important and should include pulmonary function testing, evaluation of symptoms and quality of life, and, where indicated, repeat HRCT. Multidisciplinary discussion enables comprehensive evaluation of the available information and its implications for management. The first-line treatment for SSc-ILD is usually immunosuppression. The antifibrotic drug nintedanib has been approved for slowing lung function decline in patients with SSc-ILD. Optimal management of patients with SSc-ILD requires a multidisciplinary and patient-centered approach.
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Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, Kreuter M, Lynch DA, Maher TM, Martinez FJ, Molina-Molina M, Myers JL, Nicholson AG, Ryerson CJ, Strek ME, Troy LK, Wijsenbeek M, Mammen MJ, Hossain T, Bissell BD, Herman DD, Hon SM, Kheir F, Khor YH, Macrea M, Antoniou KM, Bouros D, Buendia-Roldan I, Caro F, Crestani B, Ho L, Morisset J, Olson AL, Podolanczuk A, Poletti V, Selman M, Ewing T, Jones S, Knight SL, Ghazipura M, Wilson KC. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2022; 205:e18-e47. [PMID: 35486072 PMCID: PMC9851481 DOI: 10.1164/rccm.202202-0399st] [Citation(s) in RCA: 1220] [Impact Index Per Article: 406.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023] Open
Abstract
Background: This American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana de Tórax guideline updates prior idiopathic pulmonary fibrosis (IPF) guidelines and addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases (ILDs) other than IPF. Methods: A committee was composed of multidisciplinary experts in ILD, methodologists, and patient representatives. 1) Update of IPF: Radiological and histopathological criteria for IPF were updated by consensus. Questions about transbronchial lung cryobiopsy, genomic classifier testing, antacid medication, and antireflux surgery were informed by systematic reviews and answered with evidence-based recommendations using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach. 2) Progressive pulmonary fibrosis (PPF): PPF was defined, and then radiological and physiological criteria for PPF were determined by consensus. Questions about pirfenidone and nintedanib were informed by systematic reviews and answered with evidence-based recommendations using the GRADE approach. Results:1) Update of IPF: A conditional recommendation was made to regard transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy in centers with appropriate expertise. No recommendation was made for or against genomic classifier testing. Conditional recommendations were made against antacid medication and antireflux surgery for the treatment of IPF. 2) PPF: PPF was defined as at least two of three criteria (worsening symptoms, radiological progression, and physiological progression) occurring within the past year with no alternative explanation in a patient with an ILD other than IPF. A conditional recommendation was made for nintedanib, and additional research into pirfenidone was recommended. Conclusions: The conditional recommendations in this guideline are intended to provide the basis for rational, informed decisions by clinicians.
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Koyauchi T, Suzuki Y, Sato K, Hozumi H, Karayama M, Furuhashi K, Fujisawa T, Enomoto N, Nakamura Y, Inui N, Yokomura K, Imokawa S, Nakamura H, Morita T, Suda T. Impact of end-of-life respiratory modalities on quality of dying and death and symptom relief in patients with interstitial lung disease: a multicenter descriptive cross-sectional study. Respir Res 2022; 23:79. [PMID: 35379240 PMCID: PMC8981636 DOI: 10.1186/s12931-022-02004-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2021] [Accepted: 03/24/2022] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Respiratory modalities applied at the end of life may affect the burden of distressing symptoms and quality of dying and death (QODD) among patients with end-stage interstitial lung disease (ILD); however, there have been few studies into respiratory modalities applied to these patients near death. We hypothesized that high-flow nasal cannula (HFNC) might contribute to improved QODD and symptom relief in patients with end-stage ILD. OBJECTIVES This multicenter study examined the proportion of end-of-life respiratory modalities in a hospital setting and explored its impact on QODD and symptom relief among patients dying with ILD. METHODS Consecutive patients with ILD who died in four participating hospitals in Japan from 2015 to 2019 were identified and divided into four groups according to end-of-life respiratory modality: conventional oxygen therapy (COT), HFNC, non-invasive ventilation (NIV), and invasive mechanical ventilation (IMV). In addition, a mail survey was performed to quantify the QODD and symptom relief at their end of life from a bereaved family's perspective. QODD and symptom relief were quantified using the Good Death Inventory (GDI) for patients with a completed bereavement survey. The impact of end-of-life respiratory modalities on QODD and symptom relief was measured by multivariable linear regression using COT as a reference. RESULTS Among 177 patients analyzed for end-of-life respiratory modalities, 80 had a completed bereavement survey. The most common end-of-life respiratory modality was HFNC (n = 76, 42.9%), followed by COT (n = 62, 35.0%), NIV (n = 27, 15.3%), and IMV (n = 12, 6.8%). Regarding the place of death, 98.7% of patients treated with HFNC died outside the intensive care unit. Multivariable regression analyses revealed patients treated with HFNC had a higher GDI score for QODD [partial regression coefficient (B) = 0.46, 95% CI 0.07-0.86] and domain score related to symptom relief (B = 1.37, 95% CI 0.54-2.20) than those treated with COT. CONCLUSION HFNC was commonly used in patients with end-stage ILD who died in the hospital and was associated with higher bereaved family ratings of QODD and symptom relief. HFNC might contribute to improved QODD and symptom relief in these patients who die in a hospital setting.
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Affiliation(s)
- Takafumi Koyauchi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan.
| | - Yuzo Suzuki
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Kazuki Sato
- Nursing for Advanced Practice, Division of Integrated Health Sciences, Nagoya University Graduate School of Medicine, Aichi, Japan
| | - Hironao Hozumi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Masato Karayama
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Kazuki Furuhashi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Tomoyuki Fujisawa
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Noriyuki Enomoto
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Yutaro Nakamura
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Naoki Inui
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
| | - Koshi Yokomura
- Department of Respiratory Medicine, Respiratory Disease Centre, Seirei Mikatahara Hospital, Shizuoka, Japan
| | - Shiro Imokawa
- Department of Respiratory Medicine, Iwata City Hospital, Shizuoka, Japan
| | - Hidenori Nakamura
- Department of Respiratory Medicine, Seirei Hamamatsu Hospital, Shizuoka, Hamamatsu, Japan
| | - Tatsuya Morita
- Department of Palliative and Supportive Care, Palliative Care Team and Seirei Hospice, Seirei Mikatahara Hospital, Shizuoka, Japan
| | - Takafumi Suda
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Higashi Ward, Hamamatsu, Shizuoka, 431-3192, Japan
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Wijsenbeek M, Molina-Molina M, Chassany O, Fox J, Galvin L, Geissler K, Hammitt KM, Kreuter M, Moua T, O'Brien EC, Slagle AF, Krasnow A, Reaney M, Baldwin M, Male N, Rohr KB, Swigris J, Antoniou K. Developing a conceptual model of symptoms and impacts in progressive fibrosing interstitial lung disease to evaluate patient-reported outcome measures. ERJ Open Res 2022; 8:00681-2021. [PMID: 35509443 PMCID: PMC9062300 DOI: 10.1183/23120541.00681-2021] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2021] [Accepted: 03/04/2022] [Indexed: 12/04/2022] Open
Abstract
Background An understanding of the experience of patients with progressive fibrosing interstitial lung disease (PF-ILD) is needed to select appropriate patient-reported outcome measures (PROMs) to evaluate treatment effect in clinical trials. Methods A systematic literature review was conducted to develop a preliminary conceptual model of the symptoms experienced by patients with PF-ILD and the impacts the disease has on them. An online survey and consensus meetings were then conducted with 12-14 stakeholders (patients, clinicians, regulatory and payer advisors) to refine the conceptual model and critically appraise how key concepts should be measured by PROMs. PROMs assessed included Living with Idiopathic Pulmonary Fibrosis, Living with Pulmonary Fibrosis, the King's Brief Interstitial Lung Disease questionnaire, Cough and Sputum Assessment Questionnaire, Evaluating Respiratory Symptoms, Leicester Cough Questionnaire, Functional Assessment of Chronic Illness Therapy (Dyspnoea/Fatigue) and St George's Respiratory Questionnaire for Idiopathic Pulmonary Fibrosis. Results The literature review identified 36 signs/symptoms and 43 impacts directly or indirectly related to pulmonary aspects of PF-ILD. The most relevant symptoms identified by participants included shortness of breath on exertion, fatigue and cough; relevant impacts included effects on physical functioning, activities of daily living and emotional wellbeing. These are presented in a conceptual model. Consensus opinion was that existing PROMs need further modification and validation before use in clinical trials. Conclusions The conceptual model improves understanding of the symptoms and impacts that living with PF-ILD has on patients' wellbeing. It can help to inform the choice of PROMs in clinical trials and highlight aspects to assess in the clinical care of patients with PF-ILD.
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Affiliation(s)
- Marlies Wijsenbeek
- Dept of Respiratory Medicine, Erasmus University Medical Centre, Rotterdam, The Netherlands
| | - Maria Molina-Molina
- Dept of Pneumology, Unit of Interstitial Lung Diseases, University Hospital of Bellvitge, Institute for Biomedical Research (IDIBELL), Barcelona, Spain
| | - Olivier Chassany
- Patient-Reported Outcomes Research Unit, Université de Paris, Paris, France
- Health Economics Clinical Trial Unit (URC-ECO), Hotel-Dieu Hospital, AP-HP, Paris, France
| | - John Fox
- Foxworthy Healthcare Consulting, Ada, MI, USA
| | - Liam Galvin
- European Idiopathic Pulmonary Fibrosis and Related Disorders Federation, Overijse, Belgium
| | | | | | - Michael Kreuter
- Center for Interstitial and Rare Lung Diseases, Department of Pneumology, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Heidelberg, Germany
| | - Teng Moua
- Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA
| | | | | | | | | | - Michael Baldwin
- Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany
| | - Natalia Male
- Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany
| | - Klaus B. Rohr
- Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany
| | | | - Katerina Antoniou
- Laboratory of Cellular and Molecular Pneumonology, Dept of Respiratory Medicine, School of Medicine, University of Crete, Crete, Greece
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Hamblin M, Prosch H, Vašáková M. Diagnosis, course and management of hypersensitivity pneumonitis. Eur Respir Rev 2022; 31:31/163/210169. [PMID: 35140104 DOI: 10.1183/16000617.0169-2021] [Citation(s) in RCA: 54] [Impact Index Per Article: 18.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2021] [Accepted: 11/21/2021] [Indexed: 11/05/2022] Open
Abstract
Hypersensitivity pneumonitis (HP) is a complex and heterogeneous interstitial lung disease (ILD) that occurs when susceptible individuals develop an exaggerated immune response to an inhaled antigen. In this review, we discuss the latest guidelines for the diagnostic evaluation of patients with suspected HP, the importance of identifying patients with fibrotic and progressive disease, and the evidence supporting the drugs commonly used in the treatment of HP. Differential diagnosis of HP can be challenging and requires a thorough exposure history, multidisciplinary discussion of clinical and radiologic data, and, in some cases, assessment of bronchoalveolar lavage lymphocytosis and histopathologic findings. Patients with HP may be categorised as having non-fibrotic or fibrotic HP. The presence of fibrosis is associated with worse outcomes. A proportion of patients with fibrotic HP develop a progressive phenotype, characterised by worsening fibrosis, decline in lung function and early mortality. There are no established guidelines for the treatment of HP. Antigen avoidance should be implemented wherever possible. Immunosuppressants are commonly used in patients with HP but have not been shown to slow the worsening of fibrotic disease. Nintedanib, a tyrosine kinase inhibitor, has been approved by the US Food and Drug Administration for slowing the progression of chronic fibrosing ILDs with a progressive phenotype, including progressive fibrotic HP. Non-pharmacological interventions, such as oxygen therapy, pulmonary rehabilitation and supportive care, may be important components of the overall care of patients with progressive HP.
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Affiliation(s)
- Mark Hamblin
- Department of Pulmonary and Critical Care Medicine, University of Kansas Medical Center, Kansas City, KS, USA
| | - Helmut Prosch
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
| | - Martina Vašáková
- Department of Respiratory Medicine, University Thomayer Hospital, Prague, Czech Republic
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Khan S, Llinas EJ, Danoff SK, Llinas RH, Marsh EB. The telemedicine experience: using principles of clinical excellence to identify disparities and optimize care. Medicine (Baltimore) 2022; 101:e29017. [PMID: 35451400 PMCID: PMC8913094 DOI: 10.1097/md.0000000000029017] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2021] [Accepted: 02/17/2022] [Indexed: 01/04/2023] Open
Abstract
The use of telemedicine has increased significantly during the Corona virus disease 2019 pandemic. This manuscript serves to identify the underlying principles of clinical excellence in telemedicine and to determine whether effective care practices can be generalized as a one-size-fits-all model or must instead be tailored to individual patient populations.A survey assessing care quality and patient satisfaction for patients using telemedicine was created and administered via email to 2 urban cohorts of varying demographics and socioeconomic backgrounds: a population of patients with prior stroke and cerebrovascular disease, and a cohort of patients followed for interstitial lung disease. Results were compared across groups to determine the generalizability of effective practices across populations.Individuals taking part in telemedicine were more likely to be White, more affluent, and woman, regardless of clinical diagnosis compared with a similar cohort of patients seen in-person the year prior. A lower-than-expected number of patients who were Black and of lower socioeconomic status followed up virtually, indicating potential barriers to access. Overall, patients who participated in televisits were satisfied with the experience and felt that the care met their medical needs; however, those who were older were more likely to experience technical difficulties and prefer in-person visits, while those with less education were less likely to feel that their questions were addressed in an understandable way.When thoughtfully designed, telemedicine practices can be an effective model for patient care, though implementation must consider population characteristics including age, education, and socioeconomic status, and strategies such as ease of access versus optimization of communication strategies should be tailored to meet individual patient needs.
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Affiliation(s)
- Sheena Khan
- Department of Neurology, The Johns Hopkins School of Medicine, Baltimore, MD
| | - Edward J. Llinas
- Department of Neurology, The Johns Hopkins School of Medicine, Baltimore, MD
| | - Sonye K. Danoff
- Division of Pulmonary and Critical Care Medicine, The Johns Hopkins School of Medicine, Baltimore, MD
| | - Rafael H. Llinas
- Department of Neurology, The Johns Hopkins School of Medicine, Baltimore, MD
| | - Elisabeth B. Marsh
- Department of Neurology, The Johns Hopkins School of Medicine, Baltimore, MD
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Morrow LE, Hilleman D, Malesker MA. Management of patients with fibrosing interstitial lung diseases. Am J Health Syst Pharm 2022; 79:129-139. [PMID: 34608488 PMCID: PMC8881211 DOI: 10.1093/ajhp/zxab375] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
Abstract
PURPOSE This article summarizes the appropriate use and pharmacology of treatments for fibrosing interstitial lung diseases, with a specific focus on the antifibrotic agents nintedanib and pirfenidone. SUMMARY The interstitial lung diseases are a heterogenous group of parenchymal lung disorders with a common feature-infiltration of the interstitial space with derangement of the normal capillary-alveolar anatomy. Diseases characterized by fibrosis of the interstitial space are referred to as the fibrosing interstitial lung diseases and often show progression over time: idiopathic pulmonary fibrosis is the most common fibrotic interstitial lung disease. Historically, therapies for fibrosing lung diseases have been limited in number, questionable in efficacy, and associated with potential harms. Food and Drug Administration (FDA) approval of the antifibrotic agents nintedanib and pirfenidone for idiopathic pulmonary fibrosis in 2014 heralded an era of reorganization of therapy for the fibrotic interstitial lung diseases. Subsequent investigations have led to FDA approval of nintedanib for systemic sclerosis-associated interstitial lung disease and interstitial lung diseases with a progressive phenotype. Although supportive care and pulmonary rehabilitation should be provided to all patients, the role(s) of immunomodulators and/or immune suppressing agents vary by the underlying disease state. Several agents previously used to treat fibrotic lung diseases (N-acetylcysteine, anticoagulation, and pulmonary vasodilators) lack efficacy or cause harm. CONCLUSION With the introduction of effective pharmacotherapy for fibrosing interstitial lung disease, pharmacists have an increasingly important role in the interdisciplinary team managing these patients.
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Affiliation(s)
- Lee E Morrow
- Creighton University School of Medicine, Omaha, NE
- Creighton University School of Pharmacy and Health Professions, Omaha, NE, USA
| | - Daniel Hilleman
- Creighton University School of Pharmacy and Health Professions, Omaha, NE
- Creighton University School of Medicine, Omaha, NE, USA
| | - Mark A Malesker
- Creighton University School of Pharmacy and Health Professions, Omaha, NE
- Creighton University School of Medicine, Omaha, NE, USA
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Kolonics-Farkas AM, Šterclová M, Mogulkoc N, Lewandowska K, Müller V, Hájková M, Kramer M, Jovanovic D, Tekavec-Trkanjec J, Studnicka M, Stoeva N, Littnerová S, Vašáková M. Differences in Baseline Characteristics and Access to Treatment of Newly Diagnosed Patients With IPF in the EMPIRE Countries. Front Med (Lausanne) 2022; 8:729203. [PMID: 35004713 PMCID: PMC8733326 DOI: 10.3389/fmed.2021.729203] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Accepted: 11/22/2021] [Indexed: 12/12/2022] Open
Abstract
Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with poor prognosis. The diagnosis and treatment possibilities are dependent on the health systems of countries. Hence, comparison among countries is difficult due to data heterogeneity. Our aim was to analyse patients with IPF in Central and Eastern Europe using the uniform data from the European Multipartner IPF registry (EMPIRE), which at the time of analysis involved 10 countries. Newly diagnosed IPF patients (N = 2,492, between March 6, 2012 and May 12, 2020) from Czech Republic (N = 971, 39.0%), Turkey (N = 505, 20.3%), Poland (N = 285, 11.4%), Hungary (N = 216, 8.7%), Slovakia (N = 149, 6.0%), Israel (N = 120, 4.8%), Serbia (N = 95, 3.8%), Croatia (N = 87, 3.5%), Austria (N = 55, 2.2%), and Bulgaria (N = 9, 0.4%) were included, and Macedonia, while a member of the registry, was excluded from this analysis due to low number of cases (N = 5) at this timepoint. Baseline characteristics, smoking habit, comorbidities, lung function values, CO diffusion capacity, high-resolution CT (HRCT) pattern, and treatment data were analysed. Patients were significantly older in Austria than in the Czech Republic, Turkey, Hungary, Slovakia, Israel, and Serbia. Ever smokers were most common in Croatia (84.1%) and least frequent in Serbia (39.2%) and Slovakia (42.6%). The baseline forced vital capacity (FVC) was >80% in 44.6% of the patients, between 50 and 80% in 49.3%, and <50% in 6.1%. Most IPF patients with FVC >80% were registered in Poland (63%), while the least in Israel (25%). A typical usual interstitial pneumonia (UIP) pattern was present in 67.6% of all patients, ranging from 43.5% (Austria) to 77.2% (Poland). The majority of patients received antifibrotic therapy (64.5%); 37.4% used pirfenidone (range 7.4–39.8% between countries); and 34.9% nintedanib (range 12.6–56.0% between countries) treatment. In 6.8% of the cases, a therapy switch was initiated between the 2 antifibrotic agents. Significant differences in IPF patient characteristics and access to antifibrotic therapies exist in EMPIRE countries, which needs further investigation and strategies to improve and harmonize patient care and therapy availability in this region.
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Affiliation(s)
| | - Martina Šterclová
- Department of Respiratory Diseases of the First Faculty of Medicine Charles University, University Thomayer Hospital, Prague, Czechia
| | - Nesrin Mogulkoc
- Department of Pulmonary Medicine, Ege University Medical School, Izmir, Turkey
| | - Katarzyna Lewandowska
- First Department of Pulmonary Diseases, Institute of Tuberculosis and Lung Diseases, Warsaw, Poland
| | - Veronika Müller
- Department of Pulmonology, Semmelweis University, Budapest, Hungary
| | - Marta Hájková
- Clinic of Pneumology and Phthisiology, University Hospital Bratislava, Bratislava, Slovakia
| | - Mordechai Kramer
- Rabin Medical Center, Institute of Pulmonary Medicine, Petah Tikva, Israel
| | | | | | | | | | - Simona Littnerová
- Faculty of Medicine, Institute of Biostatistics and Analyses, Masaryk University, Brno, Czechia
| | - Martina Vašáková
- Department of Respiratory Diseases of the First Faculty of Medicine Charles University, University Thomayer Hospital, Prague, Czechia
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Copeland CR, Lancaster LH. Management of Progressive Fibrosing Interstitial Lung Diseases (PF-ILD). Front Med (Lausanne) 2021; 8:743977. [PMID: 34722582 PMCID: PMC8548364 DOI: 10.3389/fmed.2021.743977] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/19/2021] [Accepted: 09/09/2021] [Indexed: 11/13/2022] Open
Abstract
Progressive fibrosing interstitial lung diseases (PF-ILD) consist of a diverse group of interstitial lung diseases (ILD) characterized by a similar clinical phenotype of accelerated respiratory failure, frequent disease exacerbation and earlier mortality. Regardless of underlying disease process, PF-ILD progresses through similar mechanisms of self-sustained dysregulated cell repair, fibroblast proliferation and alveolar dysfunction that can be therapeutically targeted. Antifibrotic therapy with nintedanib or pirfenidone slow lung function decline and are the backbone of treatment for IPF with an expanded indication of PF-ILD for nintedanib. Immunosuppression is utilized for some subtypes of PF-ILD, including connective tissue disease ILD and hypersensitivity pneumonitis. Inhaled treprostinil is a novel therapy that improves exercise tolerance in individuals with PF-ILD and concomitant World Health Organization (WHO) group 3 pulmonary hypertension. Lung transplantation is the only curative therapy and can be considered in an appropriate and interested patient. Supportive care, oxygen therapy when appropriate, and treatment of comorbid conditions are important aspects of PF-ILD management. This review summarizes the current data and recommendations for management of PF-ILD.
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Affiliation(s)
- Carla R Copeland
- Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States
| | - Lisa H Lancaster
- Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States
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Fisher JH, Cottin V. Care Delivery Models and Interstitial Lung Disease: The Role of the Specialized Center. Clin Chest Med 2021; 42:347-355. [PMID: 34024409 DOI: 10.1016/j.ccm.2021.03.013] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023]
Abstract
Comprehensive interstitial lung disease (ILD) care delivery models have several key components including diagnosis, treatment, monitoring, coordination with other health care providers, patient support/advocacy, education, and research. ILD is rapidly evolving, and specialized centers with ILD-specific expertise have emerged as ways to care for complex patients. The role of the specialized center in care delivery is multifaceted and aimed at improving patient care and advancing the field of ILD. Widespread access to specialized centers is a barrier to ILD care delivery worldwide. Creative and innovative strategies that leverage technology are needed to bridge gaps in ILD care.
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Affiliation(s)
- Jolene H Fisher
- Department of Medicine, University Health Network, University of Toronto, 9N-945 585 University Avenue, Toronto, Ontario M5G 2N2, Canada
| | - Vincent Cottin
- Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon 1, INRAE, IVPC, RespiFil, Radico-ILD and ERN-LUNG, F-69008, UMR754, 28 Avenue Doyen Lepine, Lyon Cedex 69677, France.
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Abstract
Nonidiopathic pulmonary fibrosis (non-IPF) progressive fibrotic interstitial lung diseases (PF-ILDs) are a heterogeneous group of ILDs, often challenging to diagnose, although an accurate diagnosis has significant implications for both treatment and prognosis. A subgroup of these patients experiences progressive deterioration in lung function, physical performance, and quality of life after conventional therapy. Risk factors for ILD progression include older age, lower baseline pulmonary function, and a usual interstitial pneumonia pattern. Management of non-IPF P-ILD is both pharmacologic and nonpharmacologic. Antifibrotic drugs, originally approved for IPF, have been considered in patients with other fibrotic ILD subtypes, with favorable results in clinical trials.
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Affiliation(s)
- Bridget F Collins
- Department of Medicine, Center for Interstitial Lung Diseases, University of Washington Medical Center, 1959 NE Pacific Street, Box 356166, Seattle, WA 98195-6166, USA.
| | - Fabrizio Luppi
- Department of Medicine and Surgery, University of Milan Bicocca; Pneumology Unit, Ospedale "S. Gerardo", ASST Monza, Monza, Italy
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35
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Abstract
Progress in the past 2 decades has led to widespread use of 2 medications to slow loss of lung function in patients with pulmonary fibrosis. Treatment of individual patients with currently available pharmacotherapies can be limited by side effects, and neither drug has a consistent effect on patient symptoms or function. Several promising new pharmacotherapies are under development. Comprehensive management of pulmonary fibrosis hinges on shared decision making. Patient and caregiver education, and early identification and management of symptoms and comorbidities, can help improve quality of life.
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Affiliation(s)
- Margaret L Salisbury
- Department of Medicine, Division of Allergy, Pulmonary and Critical Care, Vanderbilt University Medical Center, 1161 21st Avenue South, T-1209A Medical Center North, Nashville, TN 37232, USA.
| | - Marlies S Wijsenbeek
- Department of Respiratory Medicine, Centre for Interstitial Lung Diseases and Sarcoidosis, Erasmus Medical Center, University Medical Centre Rotterdam, Dr. Molewaterplein 40, Rotterdam 3015, GD, the Netherlands
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36
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Liu QW, Qin T, Hu B, Zhao YL, Zhu XL. Relationship between illness perception, fear of progression and quality of life in interstitial lung disease patients: A cross-sectional study. J Clin Nurs 2021; 30:3493-3505. [PMID: 33998090 DOI: 10.1111/jocn.15852] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2020] [Revised: 03/31/2021] [Accepted: 04/22/2021] [Indexed: 01/02/2023]
Abstract
AIMS AND OBJECTIVES This study aimed to investigate whether fear of progression mediates the association between illness perception and quality of life among interstitial lung disease patients. BACKGROUND So far, the physiological treatment of interstitial lung disease is limited. In addition to immunosuppressants such as glucocorticoids, two anti-fibrosis drugs (pirfenidone and nintedanib) have shown moderately beneficial effects on slowing the progression of interstitial lung disease fibrosis. However, none of these drugs has shown reliable or strong beneficial effects on improving quality of life. Psychological care and mental health support strategies focusing on improving patients' quality of life are particularly important. DESIGN A cross-sectional study. METHODS A convenience sample of patients suffering from interstitial lung disease were enrolled from August to December 2019. Data including sociodemographic and clinical characteristics, illness perception, fear of progression and quality of life were collected. The descriptive analysis and Pearson correlations were analysed by SPSS 26.0 (IBM Corp.). PROCESS v3.4 (by Andrew F. Hayes) macro was applied to analyse the mediating effects. We used the STROBE checklist to report the results. RESULTS Both illness perception and fear of progression were correlated with quality of life. Fear of progression mediated the association between illness perception and quality of life. The indirect effect was 0.121, and the proportion of intermediary effect in the main effect was 26.36%. CONCLUSION Interstitial lung disease patients experience relatively poor quality of life and fear of progression exerts a mediating role between illness perception and quality of life. RELEVANCE TO CLINICAL PRACTICE This study alerts medical staff to pay attention to negative illness perception and excessive fear, which is helpful to formulate effective interventions to manage interstitial lung disease patients' quality of life.
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Affiliation(s)
- Qing-Wei Liu
- Department of Medicine, School of Nursing, Qingdao University, Qingdao, China
| | - Tong Qin
- Department of Medicine, School of Nursing, Qingdao University, Qingdao, China
| | - Bo Hu
- Department of Thoracic Surgery, Municipal Hospital, Qingdao, China
| | - Ya-Ling Zhao
- Department of Medicine, School of Nursing, Qingdao University, Qingdao, China
| | - Xiu-Li Zhu
- Department of Medicine, School of Nursing, Qingdao University, Qingdao, China
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37
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Goodwin AT, Saini G. Supportive care of patients with fibrosing interstitial lung disease: answering a great clinical need. Breathe (Sheff) 2021; 16:200066. [PMID: 33447270 PMCID: PMC7792811 DOI: 10.1183/20734735.0066-2020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
The fibrosing interstitial lung diseases (F-ILDs) are a heterogeneous group, including idiopathic pulmonary fibrosis (IPF), connective tissue disease-associated interstitial lung disease, hypersensitivity pneumonitis and asbestosis, among others. The common feature of all F-ILDs is the irreversible replacement of normal lung parenchyma with scar tissue, resulting in impaired gas exchange and culminating in respiratory failure. Fibrosing interstitial lung disease (F-ILD) significantly reduces quality of life. F-ILD care includes symptom management, end-of-life planning and supportive measures, as well as antifibrotics. Patients and carers should be central to all care decisions.https://bit.ly/2ZAE2Ks
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Affiliation(s)
- Amanda T Goodwin
- Nottingham NIHR Respiratory Biomedical Research Centre, University of Nottingham, Nottingham, UK
| | - Gauri Saini
- Nottingham NIHR Respiratory Biomedical Research Centre, University of Nottingham, Nottingham, UK.,Nottingham University Hospital NHS Trust, Nottingham, UK
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38
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Specialty Palliative Care Program ILD. Respir Med 2021. [DOI: 10.1007/978-3-030-81788-6_16] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
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Palliative Care in Interstitial Lung Disease. Respir Med 2021. [DOI: 10.1007/978-3-030-81788-6_11] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
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40
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Goda K, Kenzaka T, Kuriyama K, Hoshijima M, Akita H. End-of-life home care of an interstitial pneumonia patient supported by high-flow nasal cannula therapy: A case report. World J Clin Cases 2020; 8:4853-4857. [PMID: 33195653 PMCID: PMC7642529 DOI: 10.12998/wjcc.v8.i20.4853] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2020] [Revised: 09/18/2020] [Accepted: 09/25/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND High-flow nasal cannula (HFNC) therapy and morphine continuous subcutaneous infusion (CSI) have been used to ameliorate dyspnea in non-cancer patients with end-stage respiratory diseases, including chronic obstructive pulmonary disease and interstitial pneumonia, primarily in hospital settings. However, it is rare to perform home-based medical treatment using these. We observe a case to assess the feasibility of this treatment strategy.
CASE SUMMARY Here, we report a case of a 75-year-old man who was diagnosed with interstitial pneumonia 11 years ago and was successfully nursed at home during his terminal phase for over 10 mo without hospitalization, by introducing domiciliary uses of HFNC and morphine CSI with a patient-controlled analgesia device.
CONCLUSION Active utilization of HFNC and morphine CSI with patient-controlled analgesia device would substantiate successful end-of-life palliative home care of idiopathic interstitial pneumonia patients.
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Affiliation(s)
- Ken Goda
- Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, Tamba 669-3495, Japan
- Division of Community Medicine and Career Development, Kobe University Graduate School of Medicine, Kobe 652-0032, Japan
- Department of Medicine, Tamba City mirune Clinic, Tamba 669-3464, Japan
| | - Tsuneaki Kenzaka
- Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, Tamba 669-3495, Japan
- Division of Community Medicine and Career Development, Kobe University Graduate School of Medicine, Kobe 652-0032, Japan
- Department of Medicine, Tamba City mirune Clinic, Tamba 669-3464, Japan
| | - Kyosuke Kuriyama
- Clinical Engineer, Nursing Department, Hyogo Prefectural Tamba Medical Center, Tamba 669-3495, Japan
| | - Masahiko Hoshijima
- Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, Tamba 669-3495, Japan
| | - Hozuka Akita
- Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, Tamba 669-3495, Japan
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41
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Managing Dyspnea in Individuals With Idiopathic Pulmonary Fibrosis. J Hosp Palliat Nurs 2020; 22:447-455. [PMID: 32956191 DOI: 10.1097/njh.0000000000000687] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Idiopathic pulmonary fibrosis is an unrelenting form of interstitial lung disease associated with a high symptom burden and reported low health-related quality of life. Clinicians have access to limited pharmacologic interventions to help slow the disease progression. Nonpharmacologic interventions are vital in managing dyspnea for these individuals, which is one of the most frequently reported factors that negatively impacts health-related quality of life. Common methods of symptom control include integration of pulmonary rehabilitation, supplemental oxygen, and interdisciplinary support, such as support groups, palliative care, and case conferences, into routine medical care. This literature review describes a multidisciplinary approach for managing dyspnea to improve health-related quality of life for those with idiopathic pulmonary fibrosis. Findings demonstrate that structured pulmonary rehabilitation programs, fast-track case conferences, and supplemental oxygen therapy are most effective. Further research is needed to demonstrate a clinically significant benefit of palliative care visits in the long term for these individuals.
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42
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Affiliation(s)
- Marlies Wijsenbeek
- From the Center for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC-University Medical Center Rotterdam, Rotterdam, the Netherlands (M.W.); and the Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, and Claude Bernard University - both in Lyon, France (V.C.)
| | - Vincent Cottin
- From the Center for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC-University Medical Center Rotterdam, Rotterdam, the Netherlands (M.W.); and the Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, and Claude Bernard University - both in Lyon, France (V.C.)
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43
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Khor YH, Harrison A, Robinson J, Goh NSL, Glaspole I, McDonald CF. Moderate resting hypoxaemia in fibrotic interstitial lung disease. Eur Respir J 2020; 57:13993003.01563-2020. [PMID: 32859672 DOI: 10.1183/13993003.01563-2020] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2020] [Accepted: 08/09/2020] [Indexed: 11/05/2022]
Affiliation(s)
- Yet H Khor
- Dept of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Australia.,Institute for Breathing and Sleep, Heidelberg, Australia.,Faculty of Medicine, University of Melbourne, Melbourne, Australia.,Dept of Respiratory Medicine, Alfred Health, Melbourne, Australia
| | - Amelia Harrison
- Dept of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Australia.,Institute for Breathing and Sleep, Heidelberg, Australia
| | - Julien Robinson
- Dept of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Australia.,Institute for Breathing and Sleep, Heidelberg, Australia
| | - Nicole S L Goh
- Dept of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Australia.,Institute for Breathing and Sleep, Heidelberg, Australia.,Faculty of Medicine, University of Melbourne, Melbourne, Australia.,Dept of Respiratory Medicine, Alfred Health, Melbourne, Australia
| | - Ian Glaspole
- Dept of Respiratory Medicine, Alfred Health, Melbourne, Australia.,Dept of Medicine, Monash University, Melbourne, Australia
| | - Christine F McDonald
- Dept of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Australia.,Institute for Breathing and Sleep, Heidelberg, Australia.,Faculty of Medicine, University of Melbourne, Melbourne, Australia
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44
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Hoffmann-Vold AM, Allanore Y, Bendstrup E, Bruni C, Distler O, Maher TM, Wijsenbeek M, Kreuter M. The need for a holistic approach for SSc-ILD - achievements and ambiguity in a devastating disease. Respir Res 2020; 21:197. [PMID: 32703199 PMCID: PMC7379834 DOI: 10.1186/s12931-020-01459-0] [Citation(s) in RCA: 34] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2020] [Accepted: 07/17/2020] [Indexed: 12/13/2022] Open
Abstract
Systemic sclerosis (SSc) is a multi-organ autoimmune disease with complex interactions between immune-mediated inflammatory processes and vascular pathology leading to small vessel obliteration, promoting uncontrolled fibrosis of skin and internal organs. Interstitial lung disease (ILD) is a common but highly variable manifestation of SSc and is associated with high morbidity and mortality. Treatment approaches have focused on immunosuppressive therapies, which have shown some efficacy on lung function. Recently, a large phase 3 trial showed that treatment with nintedanib was associated with a reduction in lung function decline. None of the conducted randomized clinical trials have so far shown convincing efficacy on other outcome measures including quality of life determined by patient reported outcomes. Little evidence is available for non-pharmacological treatment and supportive care specifically for SSc-ILD patients, including pulmonary rehabilitation, supplemental oxygen, symptom relief and adequate information. Improved management of SSc-ILD patients based on a holistic approach is necessary to support patients in maintaining as much quality of life as possible throughout the disease course and to improve long-term outcomes.
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Affiliation(s)
- Anna-Maria Hoffmann-Vold
- Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Pb 4950 Nydalen, 0424, Oslo, Norway.
- Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
| | - Yannick Allanore
- Rheumatology Department, Cochin Hospital, University of PARIS and INSERM U1016, Paris, France
| | - Elisabeth Bendstrup
- Center for Rare Lung Diseases, Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark
| | - Cosimo Bruni
- Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy
| | - Oliver Distler
- Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
| | - Toby M Maher
- Inflammation, Repair, and Development Section, National Heart and Lung Institute, Imperial College London, London, UK
- National Institute for Health Research Respiratory Clinical Research Facility, Royal Brompton Hospital, London, UK
- Keck School of Medicine, University of Southern California, 2020 Zonal Avenue, Los Angeles, California, USA
| | - Marlies Wijsenbeek
- Center for Interstitial lung disease and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
| | - Michael Kreuter
- Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, Heidelberg University Hospital and German Center for Lung Research, Heidelberg, Germany
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45
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Abstract
PURPOSE OF REVIEW Supplemental oxygen therapy is prescribed for management of hypoxaemia in patients with interstitial lung disease (ILD). This review summarizes current evidence and implications of the use of supplemental oxygen therapy at home and during exercise training in ILD. RECENT FINDINGS Despite the significance of hypoxaemia in patients with ILD, there is a lack of high-quality evidence to guide the use of oxygen therapy in this population. Recent studies suggest that ambulatory oxygen may improve symptoms and health-related quality of life in patients with ILD. Long-term oxygen therapy for resting hypoxaemia in ILD is recommended by international guidelines. Supplemental oxygen during exercise may augment training effects, whereas therapeutic effects of nocturnal oxygen therapy are yet to be evaluated in patients with ILD. Nevertheless, it is important to consider the potential burden imposed by oxygen therapy on patients' daily activities of living. SUMMARY Ambulatory oxygen may be considered in ILD patients with exertional hypoxaemia, with long-term oxygen therapy being a standard care for resting hypoxaemia. Trials are currently underway to clarify therapeutic potentials of supplemental oxygen for exertional hypoxaemia and during exercise training in ILD patients, with additional research needed for the evaluation of nocturnal oxygen therapy.
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46
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Abstract
Overnight pulse oximetry (OPO) has proven to be an effective and beneficial technique to determine the cardiorespiratory status of patients in both the inpatient and outpatient settings. It is a cheap, safe, reliable, simple, and accurate method of patient monitoring as compared to the expensive and labor-intensive method of multichannel polysomnography for detecting sleep-disordered breathing. It provides accurate information about patient's oxygenation status and also helps in monitoring the response to continuous positive airway pressure and in the surgical treatment of obstructive sleep apnea (OSA). Nocturnal hypoxemia portends a poor prognosis in patients of chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and neuromuscular diseases. OPO can help its early detection and management.
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Affiliation(s)
- Shruti Singh
- Division of Pulmonary, Critical Care and Sleep Medicine, Northwell Health, New Hyde Park, NY, USA
| | - Sara Z Khan
- Division of Pulmonary, Critical Care and Sleep Medicine, Northwell Health, New Hyde Park, NY, USA
| | - Dilbagh Singh
- Division of Pulmonary, Critical Care and Sleep Medicine, Northwell Health, New Hyde Park, NY, USA
| | - Sameer Verma
- Division of Pulmonary, Critical Care and Sleep Medicine, Northwell Health, New Hyde Park, NY, USA
| | - Arunabh Talwar
- Division of Pulmonary, Critical Care and Sleep Medicine, Northwell Health, New Hyde Park, NY, USA
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