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Ciobica ML, Botezatu AS, Galajda Z, Carsote M, Nistor C, Sandulescu BA. Minimally Invasive Surgical Approach in Granulomatosis with Polyangiitis Complicated with Intramural Descending Aorta Hematoma Followed by Aortic Wall Rupture. Diagnostics (Basel) 2025; 15:144. [PMID: 39857028 PMCID: PMC11763346 DOI: 10.3390/diagnostics15020144] [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: 11/20/2024] [Revised: 01/02/2025] [Accepted: 01/06/2025] [Indexed: 01/27/2025] Open
Abstract
Background and Clinical Significance: Granulomatosis with polyangiitis (GPA) represents a rare autoimmune disease with granulomatous inflammation, tissue necrosis, and systemic vasculitis of the small and medium blood vessels. Although the clinical elements vary, aortic involvement is exceptional and it represents a challenge that requires a rapid intervention with the potential of displaying a fulminant evolution. Case Presentation: We report a 64-year-old male with an 18-year history of GPA who presented atypical low back pain. Following ultrasound and computed tomography exams, the initial suspicion was an intramural descending aorta hematoma, surrounded by a peri-aortic sleeve suggesting a chronic inflammation. Serial non-invasive assessments revealed a progressive lesion within the next 10 to 12 days to an aortic wall rupture, despite the absence of previous aneurysmal changes. The peri-aortic fibrous inflammatory sleeve was life-saving, and emergency minimally invasive surgery was successful, including the massive improvement in back pain. Conclusions: To our knowledge, this is a very rare scenario in GPA; we found only 18 other cases (the oldest report being from 1994). An interventional approach was mentioned in a few cases as seen in this instance. Glucocorticoid medication for GPA might act as a potential contributor to symptomatic osteoporotic fractures which require a prompt differential diagnosis. Unusual aortic manifestations (such as intramural aortic hematoma or aortic wall rupture) are difficult to recognize since the index of clinical suspicion is rather low. A prompt intervention may be life-saving and a multidisciplinary team is mandatory. Minimally invasive surgical correction of the aortic event represents an optimum management in the modern era. Such cases add to the limited data we have so far with respect to unusual outcomes in long-standing GPAs.
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Affiliation(s)
- Mihai-Lucian Ciobica
- Department of Internal Medicine and Gastroenterology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (M.-L.C.); (B.-A.S.)
- Department of Internal Medicine I and Rheumatology, “Dr. Carol Davila” Central Military University Emergency Hospital, 010825 Bucharest, Romania
| | - Alexandru-Sebastian Botezatu
- Department of Angiography and Cardiac Catheterization, “Dr. Carol Davila” Central Military Emergency University Hospital, 010825 Bucharest, Romania;
| | - Zoltan Galajda
- Clinical Department of Cardiovascular Surgery, “Dr. Carol Davila” Central Military Emergency University Hospital, 010825 Bucharest, Romania;
| | - Mara Carsote
- Department of Endocrinology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
- Department of Clinical Endocrinology V, “C.I. Parhon” National Institute of Endocrinology, 011863 Bucharest, Romania
| | - Claudiu Nistor
- Department 4-Cardio-Thoracic Pathology, Thoracic Surgery II Discipline, “Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania
- Thoracic Surgery Department, “Dr. Carol Davila” Central Military University Emergency Hospital, 010242 Bucharest, Romania
| | - Bianca-Andreea Sandulescu
- Department of Internal Medicine and Gastroenterology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania; (M.-L.C.); (B.-A.S.)
- Department of Internal Medicine I and Rheumatology, “Dr. Carol Davila” Central Military University Emergency Hospital, 010825 Bucharest, Romania
- PhD Doctoral School, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania
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Monghal V, Puéchal X, Smets P, Vandergheynst F, Michel M, Diot E, Ramdani Y, Moulinet T, Dhote R, Hautcoeur A, Lelubre C, Dominique S, Lebourg L, Melboucy S, Wauters N, Carlotti A, Cachin F, Ebbo M, Jourde-Chiche N, Iudici M, Aumaitre O, Andre M, Terrier B, Trefond L. Large-vessel involvement in ANCA-associated vasculitis: A multicenter case-control study. Semin Arthritis Rheum 2024; 67:152475. [PMID: 38810568 DOI: 10.1016/j.semarthrit.2024.152475] [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: 01/22/2024] [Revised: 04/08/2024] [Accepted: 04/15/2024] [Indexed: 05/31/2024]
Abstract
OBJECTIVE Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management. METHODS This multicenter case-control (1:2) study included patients with AAV according to the ACR/EULAR classification and LVI as defined by the Chapel Hill nomenclature, together with controls matched for age, sex, and AAV type. RESULTS We included 26 patients, 15 (58 %) of whom were men, with a mean age of 56.0 ± 17.1 years. The patients had granulomatosis with polyangiitis (n = 20), or microscopic polyangiitis (n = 6). The affected vessels included the aorta (n = 18; 69 %) supra-aortic trunks (n = 9; 35 %), lower-limb arteries (n = 5; 19 %), mesenteric arteries (n = 5; 19 %), renal arteries (n = 4; 15 %), and upper-limb arteries (n = 2; 8 %). Imaging showed wall thickening (n = 10; 38 %), perivascular inflammation (n = 8; 31 %), aneurysms (n = 5; 19 %), and stenosis (n = 4; 15 %). Comparisons with the control group revealed that LVI was significantly associated with neurological manifestations (OR=3.23 [95 % CI: 1.11-10.01, p = 0.03]), but not with cardiovascular risk factors (OR=0.70 [95 % CI: 0.23-2.21, p = 0.60]), or AAV relapse (OR=2.01 [95 % CI: 0.70-5.88, p = 0.16]). All patients received corticosteroids, in combination with an immunosuppressant in 24 (92 %), mostly cyclophosphamide (n = 10, 38 %) or rituximab (n = 9, 35 %). CONCLUSION Regardless of distinctions based on vessel size, clinicians should consider LVI as a potential manifestation of AAV, with the aorta commonly affected. The risk of developing LVI appears to be greater for clinical phenotypes of AAV with neurological involvement. Standard AAV treatment can be used to manage LVI.
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Affiliation(s)
- V Monghal
- Médecine Interne, Centre Hospitalier Universitaire Gabriel-Montpied, 63000 Clermont-Ferrand, France
| | - X Puéchal
- National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP). Centre, Université Paris Cité, Paris, France
| | - P Smets
- Médecine Interne, Centre Hospitalier Universitaire Gabriel-Montpied, 63000 Clermont-Ferrand, France
| | - F Vandergheynst
- General Internal Medicine Department, Erasme University Hospital, Brussels, Belgium
| | - M Michel
- Centre de Référence des Cytopénies Auto-Immunes de l'Adulte, Service de Médecine Interne, CHU Henri Mondor, AP-HP, Université Paris-Est Créteil, Créteil, France
| | - E Diot
- Service de Médecine Interne Immunologie Clinique, CHU de Tours, F-37032 Tours, France
| | - Y Ramdani
- Service de Médecine Interne Immunologie Clinique, CHU de Tours, F-37032 Tours, France
| | - T Moulinet
- Systemic and Autoimmune Rare Diseases, Nancy University Hospital, UMR 7365, IMoPA, Lorraine University, CNRS, Vandoeuvre-lès-Nancy, France
| | - R Dhote
- Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris (APHP), Avicenne Hospital, Paris, France
| | - A Hautcoeur
- Department of Internal Medicine, Assistance Publique-Hôpitaux de Paris (APHP), Avicenne Hospital, Paris, France
| | - C Lelubre
- Department of Internal Medicine, CHU-Charleroi Marie-Curie, 6042 Charleroi, Belgium
| | - S Dominique
- CHU Rouen, Department of Pulmonology, F-76000 Rouen, France
| | - L Lebourg
- Department of Nephrology, Kidney Transplantation and Hemodialysis, Rouen University Hospital, Rouen, France
| | - S Melboucy
- Service de médecine interne et polyvalente, centre hospitalier de Saint-Quentin, 1, avenue Michel de l'Hospital, 02100, Saint-Quentin, France
| | - N Wauters
- General Internal Medicine Department, Erasme University Hospital, Brussels, Belgium
| | - A Carlotti
- Department of Pathology, Assistance Publique - Hôpitaux de Paris, Cochin Hospital, 75014, Paris, France
| | - F Cachin
- Service de Médecine Nucléaire, Centre Jean PERRIN, Clermont-Ferrand, France
| | - M Ebbo
- Département de Medecine Interne, CHU Timone, AP-HM, Aix Marseille Université, France
| | - N Jourde-Chiche
- Aix-Marseille Université, Faculté des Sciences Médicales et Paramédicales, C2VN, INRA 1260, INSERM 1263, AP-HM Hôpital de la Conception, Centre de Néphrologie et Transplantation Rénale, Marseille, France
| | - M Iudici
- National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP). Centre, Université Paris Cité, Paris, France
| | - O Aumaitre
- Médecine Interne, Centre Hospitalier Universitaire Gabriel-Montpied, 63000 Clermont-Ferrand, France; Université Clermont Auvergne Inserm U1071, Microbes, Intestin, Inflammation et Susceptibilité de l'Hôte (M2iSH), USC-INRA 2018, Clermont-Ferrand, France
| | - M Andre
- Médecine Interne, Centre Hospitalier Universitaire Gabriel-Montpied, 63000 Clermont-Ferrand, France; Université Clermont Auvergne Inserm U1071, Microbes, Intestin, Inflammation et Susceptibilité de l'Hôte (M2iSH), USC-INRA 2018, Clermont-Ferrand, France
| | - B Terrier
- National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP). Centre, Université Paris Cité, Paris, France
| | - L Trefond
- Médecine Interne, Centre Hospitalier Universitaire Gabriel-Montpied, 63000 Clermont-Ferrand, France; Université Clermont Auvergne Inserm U1071, Microbes, Intestin, Inflammation et Susceptibilité de l'Hôte (M2iSH), USC-INRA 2018, Clermont-Ferrand, France.
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Kaymakci MS, Elfishawi MM, Langenfeld HE, Hanson AC, Crowson CS, Bois MC, Ghaffar U, Koster MJ, Specks U, Warrington KJ. Large vessel involvement in antineutrophil cytoplasmic antibody-associated vasculitis. Rheumatology (Oxford) 2024; 63:1682-1689. [PMID: 37672018 DOI: 10.1093/rheumatology/kead467] [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: 06/08/2023] [Revised: 08/16/2023] [Accepted: 08/19/2023] [Indexed: 09/07/2023] Open
Abstract
OBJECTIVES ANCA-associated vasculitis (AAV) is currently categorized under the small vessel vasculitides. There is limited knowledge about large vessel involvement in AAV (L-AAV), mainly described in case reports and small series. L-AAV can involve temporal arteries (TA-AAV), aorta (A-AAV), and periaortic soft tissue (PA-AAV). We sought to characterize the features of patients with L-AAV. METHODS Patients older than 18 years at diagnosis of TA-AAV, A-AAV and PA-AAV seen at the Mayo Clinic, Rochester between 1 January 2000 and 31 December 2021 were identified through a proprietary medical text search algorithm. Patients were included if diagnosed with L-AAV, fulfilled 2022 ACR/EULAR classification criteria for GPA, MPA or EGPA, had positive ANCA test results, and had more than one outpatient or inpatient visit. RESULTS The study cohort consists of 36 patients with L-AAV. Of those, 23 had p-ANCA and/or MPO-ANCA, and 13 had c-ANCA and/or PR3-ANCA. Mean (s.d.) age at AAV diagnosis was 63.4 (12.79) years; 20 (56%) were male. Seventeen patients had TA-AAV, 10 had A-AAV and 9 had PA-AAV. Most patients (n = 25, 69%) were diagnosed with large vessel vasculitis and AAV within a 1-year timespan. Twenty-five (69%) patients had histopathological confirmation of AAV diagnosis in a location other than temporal artery, aorta or periaortic soft tissue. Glucocorticoids (36/36), rituximab (19/36) and methotrexate (18/36) were the most frequent treatments. CONCLUSION This is the largest single-centre cohort of patients with L-AAV to date. AAV can involve large arteries, albeit infrequent. AAV-targeted therapy should be considered in patients with L-AAV.
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Affiliation(s)
- Mahmut S Kaymakci
- Division of Rheumatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA
| | - Mohanad M Elfishawi
- Division of Rheumatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA
| | | | - Andrew C Hanson
- Department of Quantitative Health Sciences, Mayo Clinic, Rochester, MN, USA
| | - Cynthia S Crowson
- Division of Rheumatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA
- Department of Quantitative Health Sciences, Mayo Clinic, Rochester, MN, USA
| | - Melanie C Bois
- Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
| | - Umar Ghaffar
- Division of Rheumatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA
| | - Matthew J Koster
- Division of Rheumatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA
| | - Ulrich Specks
- Division of Pulmonary and Critical Care, Department of Medicine, Mayo Clinic, Rochester, MN, USA
| | - Kenneth J Warrington
- Division of Rheumatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA
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He L, Gao X, Meng F, Chen J, Shi Q, Zhao L, Yang J, Zhou Q, Wu H, Li W, Li Z. Peripheral T cell lymphoma initially presenting in lung biopsies: A diagnostic challenge. Pathol Res Pract 2024; 256:155234. [PMID: 38471291 DOI: 10.1016/j.prp.2024.155234] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2023] [Revised: 02/23/2024] [Accepted: 03/01/2024] [Indexed: 03/14/2024]
Abstract
BACKGROUND Primary or secondary pulmonary involvement by peripheral T cell lymphoma (PTCL) is rare and difficult to diagnose particularly via lung biopsies. METHODS 22 cases of PTCL diagnosed initially in lung biopsies between January 2006 and November 2020 were retrospectively reviewed followed at Nanjing Drum Tower Hospital and the First Affiliated Hospital of Zhengzhou University, respectively, including clinical manifestations, baseline biochemical indexes, images, histological findings and other available ancillary studies such as immunostaining, Epstein-Barr virus encoded RNA (EBER) in situ hybridization and T-cell receptor rearrangement analysis upon diagnosis. RESULTS The median age of these patients was 59 years old (range: 29-82 years) at diagnosis. The majority of them complained of fever, cough and fatigue. Computed tomography scans mainly revealed multiple ill-defined nodules/masses of various sizes and densities with or without air bronchogram. Microscopically, most lesions showed lymphoid cells with clear cytoplasm and irregular nuclear contours diffusely infiltrating alveolar septa or alveolar spaces in an inflammatory background. Several cases had a predominance of small neoplastic cells (n = 4) with atypical, irregular nuclei. One case showed a diffuse monotonous pattern of growth. Angioinvasion and necrosis were not uncommon findings. The neoplastic cells in all cases were positive for one or more T-cell markers, and negative for B-cell-lineage antigens and EBER. 19 out of 22 patients had complete follow-up information, and 17 patients were dead at the last follow-up. CONCLUSIONS Pulmonary involvement by PTCL is rare with dismal outcome. Aggressive clinical course and several clinicopathologic clues, albeit unspecific, may alert the pathologists of the possibilities of pulmonary PTCLs.
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Affiliation(s)
- Lu He
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Xianzheng Gao
- Department of Pathology, the First Affiliated Hospital of Zhengzhou University, 1 Jianshe East Road, Zhengzhou 450052, China
| | - Fanqing Meng
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Jieyu Chen
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Qianyun Shi
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Linyue Zhao
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Jun Yang
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Qiang Zhou
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Hongyan Wu
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
| | - Wencai Li
- Department of Pathology, the First Affiliated Hospital of Zhengzhou University, 1 Jianshe East Road, Zhengzhou 450052, China.
| | - Zhiwen Li
- Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China.
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Wang J, Xu Y, Wang G. ANCA-associated vasculitis with abdominal aortic aneurysm : Case report and literature review. Vascular 2023; 31:1082-1085. [PMID: 35593219 DOI: 10.1177/17085381221100384] [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] [Indexed: 11/17/2022]
Abstract
INTRODUCTION ANCA-associated vasculitis is an autoimmune disease that usually involves the small vessel walls. It is difficult to find the presence of ANCA-associated vasculitis in the great arteries, especially the thoracic and abdominal aorta. METHODS This is an 86-year-old Chinese man with ANCA-associated vasculitis and abdominal aortic aneurysm who presented with epigastric pain. Considering his age and physical condition, the patient was treated with methylprednisolone and mycophenolate mofetil instead of surgery. RESULTS The patient's epigastric pain symptoms were relieved after 2 months of conservative treatment. Imaging at follow-up 2 years later showed signs of aneurysm enlargement because of irregular medication. CONCLUSIONS Patients with ANCA-associated vasculitis combined with aortic aneurysm often require surgical management. But for patients with stable disease and poor physical conditions, conservative treatment is also an effective treatment method, which can bring benefits to the patient's survival.
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Affiliation(s)
- Jiawei Wang
- Department of Urology, Zhuzhou Hospital Affiliated to Xiangya Medical College, Central South University, Zhuzhou, China
| | - Yong Xu
- Department of Urology, Zhuzhou Hospital Affiliated to Xiangya Medical College, Central South University, Zhuzhou, China
| | - Gang Wang
- Department of Rheumatology and Immunology, Zhuzhou Hospital Affiliated to Xiangya Medical College, Central South University, Zhuzhou, China
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Kuske L, Khalifa A, Wibisono A, Bräsen JH, Witte T. MPO-ANCA-positive granulomatosis with polyangiitis and concurrent IgG4-related disease with periaortitis and tubulointerstitial nephritis: A case report of a new overlap syndrome? Int J Rheum Dis 2023; 26:1821-1825. [PMID: 36973170 DOI: 10.1111/1756-185x.14680] [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: 11/24/2022] [Revised: 03/04/2023] [Accepted: 03/13/2023] [Indexed: 03/29/2023]
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that was first recognized as a unique disease entity in the early 2000s. Its diagnosis is based on specific pathologic, serologic, and clinical features, and the exclusion of several differential diagnoses, such antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, emerging evidence suggests that these 2 conditions may overlap in some cases. Here, we report a new case of overlapping IgG4-RD and AAV. The patient was diagnosed with IgG4-RD owing to the presence of periaortitis and IgG4 positive tubulointerstitial nephritis. Myeloperoxidase (MPO)-ANCA positivity, chronic paranasal sinusitis, and glomerulonephritis with granuloma led to a concurrent diagnosis of MPO-ANCA-positive granulomatosis with polyangiitis. Our case supports the hypothesis that diagnoses of IgG4-RD and AAV are not mutually exclusive but can overlap. It can be assumed that an overlap with IgG4-RD typically affects the granulomatous form of AAV, suggesting a common pathophysiological pathway for these 2 conditions.
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Affiliation(s)
- Luise Kuske
- Department of Rheumatology, Medical School Hannover, Hannover, Germany
| | | | - Alexander Wibisono
- Department of Nuclear Medicine, Medical School Hannover, Hannover, Germany
| | | | - Torsten Witte
- Department of Rheumatology, Medical School Hannover, Hannover, Germany
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Beketova TV. Non-infectious diseases of the aorta and large arteries. TERAPEVT ARKH 2022; 94:695-703. [DOI: 10.26442/00403660.2022.05.201500] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2022] [Accepted: 06/17/2022] [Indexed: 11/22/2022]
Abstract
This article describes the various forms of inflammatory lesions of the aorta and large arteries, including chronic periaortitis, as well as the diagnostic methods are considered. Large vessel vasculitis represent the most common entities, however, there is also an association with other rheumatological or inflammatory diseases, drug-induced or paraneoplastic entities. Instrumental imaging modalities play an important role in the diagnosis.
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Virmani R, Sato Y, Sakamoto A, Romero ME, Butany J. Aneurysms of the aorta: ascending, thoracic, and abdominal and their management. Cardiovasc Pathol 2022. [DOI: 10.1016/b978-0-12-822224-9.00009-8] [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: 10/17/2022] Open
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Eosinophilic Granulomatosis Polyangiitis (EGPA) Masquerading as a Mycotic Aneurysm of the Abdominal Aorta: Case Report and Review of Literature. Case Rep Rheumatol 2021; 2021:7093607. [PMID: 34552804 PMCID: PMC8452388 DOI: 10.1155/2021/7093607] [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] [Received: 08/08/2021] [Accepted: 09/01/2021] [Indexed: 11/17/2022] Open
Abstract
Introduction Aortic involvement leading to aortitis in eosinophilic granulomatosis polyangiitis (EGPA) is infrequent, and only 2 cases have been reported so far in the literature. Even more so, aortic aneurysm, secondary to EGPA, has never been reported and remains a diagnostic and therapeutic challenge. Case Presentation. We present a 63-year-old Caucasian male patient with a prior diagnosis of EGPA presenting with abdominal pain, nausea, and loose stools to the emergency department. Physical examination showed periumbilical tenderness. He had no peripheral eosinophilia but had high C-reactive protein and procalcitonin levels. CT abdomen revealed a mycotic aneurysm involving the infrarenal abdominal aorta. The patient declined surgical repair initially and was treated with IV antibiotics only. Unfortunately, 24 hours later, the aneurysm ruptured, leading to emergent axillofemoral bypass surgery. Surgical biopsy showed aortitis, periaortitis, and active necrotizing vasculitis. Conclusion Abdominal aneurysms should be considered a complication of EGPA, and earlier immunosuppressive therapy should be considered to prevent further complications.
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Gravos A, Katsifa K, Tselioti P, Grammatikopoulou V, Sakellaridis K, Kanakaki S, Tsapas C, Destounis A, Moschouris H, Athanasiadou I, Chatzivasiloglou F, Ivanova E, Prekates A. Ruptured arterial aneurysm in Wegener's granulomatosis: a case report. J Med Case Rep 2021; 15:343. [PMID: 34247654 PMCID: PMC8274053 DOI: 10.1186/s13256-021-02955-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2020] [Accepted: 06/14/2021] [Indexed: 12/24/2022] Open
Abstract
Background Aneurysm formation is a possible, but rare, complication of granulomatosis with polyangiitis, known as Wegener’s granulomatosis. Urgent diagnosis and therapy is very important because a ruptured aneurysm could be life threatening. Case presentation We, therefore, present the case of a 63-year-old Greek man who was diagnosed with granulomatosis with polyangiitis and retroperitoneal hematoma due to ruptured aneurysm in renal artery and upper pancreaticoduodenal artery. His clinical course was complicated by acute renal failure and acute respiratory failure due to alveolar hemorrhage. Emergency coil embolization was performed. Postembolization recovery was uneventful; no bleeding occurred. The patient underwent mechanical ventilation and continuous veno-venous hemofiltration and received combined immunosuppression and supportive therapy, but eventually died 30 days after admission to hospital from severe septic shock and multiple organ failure. Conclusion Endovascular treatment is the therapy of choice, especially for patients with ruptured aneurysms that are hemodynamically stable. Early diagnosis is very important, as urgent embolization and early initiation of immunosuppression therapy are the treatment of choice.
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Affiliation(s)
- A Gravos
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece.
| | - K Katsifa
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - P Tselioti
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - V Grammatikopoulou
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - K Sakellaridis
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - S Kanakaki
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - C Tsapas
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - A Destounis
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - H Moschouris
- Radiology Department, Tzaneio General Hospital of Piraeus, Kamatero, Greece
| | - I Athanasiadou
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - F Chatzivasiloglou
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - E Ivanova
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
| | - A Prekates
- Intensive Care Unit (ICU), Tzaneio General Hospital of Piraeus, Dodonis 26, Kamatero, PC: 13451, Greece
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Cho U, Kim SK, Ko JM, Yoo J. Unusual presentation of granulomatosis with polyangiitis causing periaortitis and consequent subclavian steal syndrome: A case report. World J Clin Cases 2021; 9:1433-1438. [PMID: 33644212 PMCID: PMC7896677 DOI: 10.12998/wjcc.v9.i6.1433] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2020] [Revised: 12/30/2020] [Accepted: 01/08/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation. The formation of a large vessel lesion in GPA patients has been scarcely reported, and it can cause confusion in the diagnosis.
CASE SUMMARY A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior. An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe. Both nodules showed central necrosis. Also, there was a periaortic mass occluding the branching porting of the subclavian artery. He had positive anti-neutrophil cytoplasmic antibodies (ANCAs), but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative. The patient also developed symptoms of subclavian vein syndrome during the follow-up. Wedge resection of the lung revealed necrotizing vasculitis, destructive parenchymal abscess and surrounding granuloma, and therefore diagnosed of GPA. The patient started on methotrexate and steroid therapy with a relief of symptomatic.
CONCLUSION Here, we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome, which has never been previously described. This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.
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Affiliation(s)
- Uiju Cho
- Department of Pathology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
| | - Sung-Kyung Kim
- Department of Internal Medicine, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
| | - Jeong Min Ko
- Department of Radiology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
| | - Jinyoung Yoo
- Department of Hospital Pathology, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul 06591, South Korea
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12
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Tariq E, Nishanth K, Arshid A, Miqdad M, Cancarevic I. Aortic Involvement in Antineutrophil Cytoplasmic Antibodies Vasculitis, a Coincidence or a Real Association? Cureus 2020; 12:e9690. [PMID: 32923282 PMCID: PMC7486114 DOI: 10.7759/cureus.9690] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a type of small-vessel vasculitis. It is unusual for ANCA to involve aorta. However, multiple cases have been found where ANCA involved large vessels, particularly the aorta. Among vasculitides, aortic vasculitis is a part of Takayasu arteritis (TAK). In this review article, we tried to find the mechanism behind the aortic involvement in AAV. PubMed was used as a primary search engine, and all the available cases of aortic, as well as large-vessel involvement in ANCA-associated vasculitis, were thoroughly reviewed. Very limited data was available that could provide the mechanism behind this involvement. It is observed that ANCA-associated aortitis is more common in immunocompromised people; however, cases in previously healthy individuals have also been found. Pathogenesis of ANCA-related aortitis is different from Takayasu arteritis and is more close to ANCA-associated small vasculitis. ANCA-related aortitis involves the aorta through the same mechanism as it uses to involve small vessels. This rare manifestation of ANCA-associated vasculitis could be life-threatening but has a good prognosis if timely diagnosed and treated. ANCA-associated vasculitis must be considered as a differential diagnosis while treating a case of aortitis. We believe that there is a need to revise the classification of different types of vasculitides, and physicians should be aware of the possible overlap between different forms of vasculitides.
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Affiliation(s)
- Ezza Tariq
- Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.,Medicine, Nishtar Medical College, Multan, PAK
| | - Katukuri Nishanth
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Assam Arshid
- Surgery, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Mohammed Miqdad
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, SAU
| | - Ivan Cancarevic
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
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13
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Kawashima H, Utsugi A, Shibamiya A, Iida K, Mimura N, Ohashi H, Hase R, Kawakami M, Yanagisawa T, Hiraguri M. Consideration concerning similarities and differences between ANCA-associated vasculitis and IgG-4-related diseases: case series and review of literature. Immunol Res 2019; 67:99-107. [PMID: 30734199 DOI: 10.1007/s12026-019-9070-7] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related diseases (IgG4-RD) are regarded as entirely different disease types with different etiological mechanisms. However, we experienced two cases that had clinical features of both AAV and IgG4-RD. The first case is an 81-year-old woman who showed periaortitis and retroperitoneal fibrosis and periarteritis with elevation of myeloperoxidase-anti-neutrophil cytoplasmic antibody and IgG4 levels. The second case is a 63-year-old woman who had dura mater, ear, nose, lung, and kidney involvement with serum negative for ANCA and elevated IgG4. Renal biopsy revealed tubulointerstitial nephritis involving IgG4+ plasma cells (IgG4+/IgG+ cell ratio of ≥ 40%). On the other hand, lung biopsy showed features of granulomatosis with polyangiitis (GPA). These two cases suggested that AAV and IgG4-RD might overlap. To investigate the similarities and differences between AAV and IgG4-RD, we retrospectively analyzed 13 cases of typical GPA, a subtype of AAV, and 13 cases of typical IgG4-RD at our hospital for comparison of clinical features and found some differences that can be useful in the differential diagnosis between the two diseases. Although AAV and IgG4-RD are distinguishable based on characteristic findings in many cases, the diagnosis can be unclear in rare cases, in which clinicians should consider possible coexistence of AAV and IgG4-RD when performing further workup. Here, we discuss the similarities and differences between AAV and IgG4-RD on the basis of our results and past literature.
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Affiliation(s)
- Hirotoshi Kawashima
- Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan.
| | - Atsunari Utsugi
- Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Asuka Shibamiya
- Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Kazuma Iida
- Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Norihiro Mimura
- Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Hirohumi Ohashi
- Department of Nephrology, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Ryota Hase
- Department of Infectious Diseases, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Makio Kawakami
- Department of Pathology, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Takao Yanagisawa
- Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
| | - Masaki Hiraguri
- Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan
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14
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Yoshimoto R, Tanaka K, Kawahata T, Takatori S, Takatori K, Eguchi K, Fujishiro D, Kodama S, Kobayashi A, Okamoto K, Yuzawa S, Ota T, Makino Y. Unusual manifestations of giant cell arteritis and granulomatosis with polyangiitis. Immunol Med 2019; 42:94-98. [PMID: 31469613 DOI: 10.1080/25785826.2019.1657377] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022] Open
Abstract
Giant cell arteritis (GCA) is a type of large vessel vasculitis, and it involves the aorta, large vessels and terminal branches of the external carotid artery, especially the temporal artery. Temporal artery biopsy is a simple tool for the diagnosis of vasculitis, however, the histopathological findings do not always differentiate between the small-vessel vasculitis and GCA. We report the case of 72-year-old male who initially had a clinical diagnosis of GCA, then in the course of treatment, diagnostic histopathological approach revealed the necrotizing vasculitis with bronchocentric granulomatosis in the inflammatory nodule of the lung. The manifestations of patients with systemic vasculitis represent the disorders of multiple organ systems thus are diverse and may vary through the course of the disease. Presentation of unexpected features such as insufficient response to antibiotics, sinusitis, runny nose, discomfort of frontal region or pachymeningitis which anticipates re-evaluation of systemic vasculitis that may lead us to an appropriate diagnosis and the treatment.
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Affiliation(s)
- Ryota Yoshimoto
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Kitaru Tanaka
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Tomoki Kawahata
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Sayaka Takatori
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Kyohei Takatori
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Kohei Eguchi
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Daisuke Fujishiro
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Satoru Kodama
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Atsushi Kobayashi
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Kensaku Okamoto
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Sayaka Yuzawa
- Department of Pathology, Asahikawa Medical University Hospital , Hokkaido , Japan
| | - Tsuguhito Ota
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
| | - Yuichi Makino
- Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University , Hokkaido , Japan
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15
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Pan L, Yan JH, Gao FQ, Li H, Han SS, Cao GH, Lv CJ, Wang XZ. Case report of a 28-year-old man with aortic dissection and pulmonary shadow due to granulomatosis with polyangiitis. BMC Pulm Med 2019; 19:122. [PMID: 31286925 PMCID: PMC6615146 DOI: 10.1186/s12890-019-0884-9] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/02/2018] [Accepted: 06/24/2019] [Indexed: 12/25/2022] Open
Abstract
Background Granulomatosis with polyangiitis (GPA) is characterised by the main violation of the upper and lower respiratory tract and kidney. GPA is considered a systemic vasculitis of medium-sized and small blood vessels where aortic involvement is extremely rare. Case presentation A 28-year-old male was admitted to the hospital due to 4 h of chest pain. Computed tomography scan of the aorta showed a thickened aortic wall, pulmonary lesions, bilateral pleural effusion and pericardial effusion. The aortic dissection should be considered. An emergency operation was performed on the patient. Surgical biopsies obtained from the aortic wall showed destructive changes, visible necrosis, granulation tissue hyperplasia and a large number of acute and chronic inflammatory cells. Nearly a year later, the patient was re-examined for significant pulmonary lesions. His laboratory studies were significantly positive for anti-neutrophilic antibody directed against proteinase 3. Finally, the diagnosis of GPA was obviously established. Conclusions Although GPA rarely involves the aorta, we did not ignore the fact that GPA may involve large blood vessels. In addition, GPA should be included in the systemic vasculitis that can give rise to aortitis and even aortic dissection. Electronic supplementary material The online version of this article (10.1186/s12890-019-0884-9) contains supplementary material, which is available to authorized users.
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Affiliation(s)
- Lei Pan
- Department of Respiratory and Critical Care Medicine, Binzhou Medical University Hospital, 661 Yellow River Road, Binzhou, 256603, China
| | - Jun-Hong Yan
- Department of Ultrasonography, Binzhou Medical University Hospital, Binzhou, 256603, China
| | - Fu-Quan Gao
- Department of Respiratory and Critical Care Medicine, Binzhou Medical University Hospital, 661 Yellow River Road, Binzhou, 256603, China
| | - Hong Li
- Department of Pathology, Binzhou Medical University Hospital, Binzhou, 256603, China
| | - Sha-Sha Han
- Department of Respiratory and Critical Care Medicine, Binzhou Medical University Hospital, 661 Yellow River Road, Binzhou, 256603, China
| | - Guo-Hong Cao
- Department of Respiratory and Critical Care Medicine, Binzhou Medical University Hospital, 661 Yellow River Road, Binzhou, 256603, China
| | - Chang-Jun Lv
- Department of Respiratory and Critical Care Medicine, Binzhou Medical University Hospital, 661 Yellow River Road, Binzhou, 256603, China
| | - Xiao-Zhi Wang
- Department of Respiratory and Critical Care Medicine, Binzhou Medical University Hospital, 661 Yellow River Road, Binzhou, 256603, China.
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16
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Skeik N, Hari G, Nasr R. Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review. Rheumatol Int 2019; 39:1983-1988. [PMID: 31222438 DOI: 10.1007/s00296-019-04343-3] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2018] [Accepted: 06/09/2019] [Indexed: 12/26/2022]
Abstract
Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a systemic necrotizing small vessel vasculitis primarily affecting elderly patients. Neutrophil apoptosis and release of pro-inflammatory mediators promote small vessel inflammation and hence multi-organ disease. It rarely affects larger vessels with extremely rare aortic involvement. Diagnosis is made based on clinical presentation, tissue biopsy of affected organ, as well as immunofluorescence and ELISA assays for ANCA. Management includes immunosuppression (e.g., glucocorticoids, cyclophosphamide and rituximab) and supportive therapy. We present a rare case of a younger patient with AAV involving the aorta. The patient's diagnosis was supported by clinical presentation, systemic organ involvement, strongly positive c-ANCA, and skin as well as aortic tissue biopsy results. After failing multiple immunosuppressants, he responded well to rituximab with improved symptoms, inflammatory markers, and imaging findings. Based on our literature review, we were only able to find ten cases of ANCA-related vasculitis involving the aorta. This is the first reported case of successful treatment of AAV-related aortitis using rituximab. Our case report and literature review provide insight into treatment of severe cases of AAV with aortic involvement.
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Affiliation(s)
- Nedaa Skeik
- Minneapolis Heart Institute®, at Abbott Northwestern Hospital, Part of Allina Health, Mail Route: 33300, 920 E. 28th Street, Suite 300, Minneapolis, MN, 55407, USA.
| | - Gopika Hari
- Minneapolis Heart Institute®, at Abbott Northwestern Hospital, Part of Allina Health, Mail Route: 33300, 920 E. 28th Street, Suite 300, Minneapolis, MN, 55407, USA
| | - Rawad Nasr
- Hennepin Healthcare, University of Minnesota, Minneapolis, MN, USA
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17
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Tomosugi T, Takahashi T, Kawase Y, Yoshida K, Hayashi S, Sugiyama T, Shimizu M, Shoka M, Sawaki K, Onishi E, Hayashi N, Matsushita H, Okochi O. Accessory left gastric artery aneurysms in granulomatosis with polyangiitis: a case report and literature review. NAGOYA JOURNAL OF MEDICAL SCIENCE 2018; 79:75-83. [PMID: 28303064 PMCID: PMC5346623 DOI: 10.18999/nagjms.79.1.75] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Aneurysm formation is a potential complication of granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis. It is a very rare complication, but immediate diagnosis and therapy should be performed because an aneurysm can be life-threatening if it ruptures. An accessory left gastric artery (ALGA) is also a rare variant gastric artery that may obtain its blood supply from the left hepatic artery and left gastric artery. We herein describe a 57-year-old Japanese man who was diagnosed with GPA complicated by aneurysm rupture in an ALGA. Emergency surgery was performed after failure of arterial coil embolization to interrupt blood flow in the ALGA. The patient underwent partial resection of the lesser omentum, which contained all aneurysms. During partial resection of the lesser omentum, both the left gastric artery and ALGA were ligated because they were thought to be feeders of the aneurysms. Postoperative recovery was uneventful; no bleeding or recurrence of the aneurysms occurred. Immediate diagnosis and therapy should be performed for patients with GPA with symptoms of vascular ischemia or aortitis. Endovascular intervention is the first-choice therapy especially for hemodynamically stable patients with ruptured aneurysms or aneurysms located on variant arteries, which may have multiple blood supplies. In the present case, although endovascular treatment failed, the approach described herein was helpful during open surgery.
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Affiliation(s)
| | | | | | - Koichi Yoshida
- Department of Surgery, Tosei General Hospital, Seto, Japan
| | - Shogo Hayashi
- Department of Surgery, Tosei General Hospital, Seto, Japan
| | | | | | - Michita Shoka
- Department of Surgery, Tosei General Hospital, Seto, Japan
| | - Kohichi Sawaki
- Department of Surgery, Tosei General Hospital, Seto, Japan
| | - Eiji Onishi
- Department of Surgery, Tosei General Hospital, Seto, Japan
| | - Naomi Hayashi
- Department of Surgery, Tosei General Hospital, Seto, Japan
| | | | - Osamu Okochi
- Department of Surgery, Tosei General Hospital, Seto, Japan
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18
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Aortic Aneurysm as a Complication of Granulomatosis with Polyangiitis Successfully Treated with Prednisolone and Cyclophosphamide: A Case Report and Review of the Literature. Case Rep Rheumatol 2018; 2018:9682801. [PMID: 29971178 PMCID: PMC6008675 DOI: 10.1155/2018/9682801] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2017] [Revised: 04/21/2018] [Accepted: 05/15/2018] [Indexed: 12/23/2022] Open
Abstract
A 57-year-old Japanese man was admitted to the hospital with back pain and fever, multiple lung nodules, and abdominal aortic aneurysm (AAA). Laboratory tests performed at admission showed an increased proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) level. Video-associated thoracoscopic lung biopsy was performed; pathologic examination showed granulation tissue with necrosis and multinucleated giant cells. The diagnosis of granulomatosis with polyangiitis (GPA) was confirmed on the basis of the clinical presentation, laboratory findings, and lung biopsy. All symptoms were ameliorated, and the serum level of PR3-ANCA declined following treatment with prednisolone and cyclophosphamide. Although the association of GPA with AAA is rare, GPA may be included among the large vessel vasculitides that can give rise to aortic aneurysm.
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19
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Kaga H, Komatsuda A, Saito M, Nara M, Omokawa A, Togashi M, Okuyama S, Wakui H, Takahashi N. Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Complicated by Periaortitis and Cranial Hypertrophic Pachymeningitis: A Report of an Autopsy Case. Intern Med 2018; 57:107-113. [PMID: 29021484 PMCID: PMC5799067 DOI: 10.2169/internalmedicine.8751-16] [Citation(s) in RCA: 2] [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] [Indexed: 11/19/2022] Open
Abstract
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic inflammatory disorder categorized as small-vessel vasculitis. We herein report an elderly Japanese man with AAV (granulomatosis with polyangiitis affecting the eyes, nose, lungs, and kidneys) who also showed periaortitis at the diagnosis and developed cranial hypertrophic pachymeningitis (HP) during steroid maintenance therapy. His consciousness disturbance caused by HP improved after steroid pulse therapy, but he died of aspiration pneumonia. Autopsy findings showed giant cells in the thickened pachymeninges and obsolete inflammatory lesions in the aortic adventitia and renal tubulointerstitium. This is the first case of AAV complicated by periaortitis and cranial HP.
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Affiliation(s)
- Hajime Kaga
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | - Atsushi Komatsuda
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | - Masaya Saito
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | - Mizuho Nara
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | - Ayumi Omokawa
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | - Masaru Togashi
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | - Shin Okuyama
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | - Hideki Wakui
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
- Department of Life Science, Akita University Graduate School of Engineering Science, Japan
| | - Naoto Takahashi
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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20
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Cinar I, Wang H, Stone JR. Clinically isolated aortitis: pitfalls, progress, and possibilities. Cardiovasc Pathol 2017; 29:23-32. [PMID: 28500877 DOI: 10.1016/j.carpath.2017.04.003] [Citation(s) in RCA: 48] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2017] [Revised: 04/20/2017] [Accepted: 04/20/2017] [Indexed: 12/12/2022] Open
Abstract
Non-infectious aortitis may be caused by several distinct systemic rheumatologic diseases. In some patients, aortitis is identified either pathologically or radiologically in the absence of clinical evidence of a systemic vasculitis. By consensus nomenclature, such cases are referred to as clinically isolated aortitis (CIA). Some systemic disorders may initially present as CIA including giant cell arteritis (GCA), IgG4-related disease, infectious aortitis, and granulomatosis with polyangiitis. CIA most commonly occurs in women of European descent over the age of 50 and, thus, mirrors the gender, age, and geographic distribution of GCA. CIA most often demonstrates a granulomatous/giant cell pattern of inflammation (GPI), and CIA-GPI is pathologically indistinguishable from aortitis due to GCA. In many cases, CIA may be a manifestation of extracranial GCA. CIA is being identified both pathologically in resected aortic tissue and radiologically by computed tomography scanning, magnetic resonance imaging, and fluorodeoxyglucose positron emission tomography. However, there appears to be significant differences between pathologically defined CIA and radiologically defined CIA. Multiple studies have shown that patients with CIA are at increased risk for subsequent aortic events (new aneurysms or dissections) and thus it is recommended to monitor these patients with periodic aortic imaging. While the data is currently limited, there is increasing evidence that at least some patients with CIA may benefit from immunosuppressive therapy.
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Affiliation(s)
- Ilkay Cinar
- Department of Pathology, Prof. Dr. A. Ilhan Ozdemir Research Hospital, Giresun University, Giresun, Turkey
| | - He Wang
- Department of Pathology and Laboratory Medicine, Lewis Katz School of Medicine, Temple University, Philadelphia, PA, USA
| | - James R Stone
- Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
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21
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González Revilla EM, Fernandez AA, Ramirez MTR, Pardo SC, Moragues MAJ. Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis. Respir Med Case Rep 2016; 19:121-4. [PMID: 27672552 PMCID: PMC5030371 DOI: 10.1016/j.rmcr.2016.08.009] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2015] [Revised: 08/20/2016] [Accepted: 08/24/2016] [Indexed: 12/19/2022] Open
Abstract
Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures. We present the case of a 74-year-old man with a past history of infrarenal abdominal aortic aneurysm. He consulted for abdominal pain. Cavitating pulmonary nodules and retroperitoneal fibrosis with periaortic alterations were detected on computed tomography. Laboratory investigations revealed that the patient was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and necrotizing granulomas were observed on biopsies of the lung lesions and retroperitoneal tissue. The patient was diagnosed with GPA and treatment was started with glucocorticoids and immunosuppressive agents, which led to a significant clinical and radiological improvement over the following months.
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22
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Ladich E, Butany J, Virmani R. Aneurysms of the Aorta. Cardiovasc Pathol 2016. [DOI: 10.1016/b978-0-12-420219-1.00005-7] [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: 10/22/2022] Open
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23
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Stone JR, Bruneval P, Angelini A, Bartoloni G, Basso C, Batoroeva L, Buja LM, Butany J, d'Amati G, Fallon JT, Gittenberger-de Groot AC, Gouveia RH, Halushka MK, Kelly KL, Kholova I, Leone O, Litovsky SH, Maleszewski JJ, Miller DV, Mitchell RN, Preston SD, Pucci A, Radio SJ, Rodriguez ER, Sheppard MN, Suvarna SK, Tan CD, Thiene G, van der Wal AC, Veinot JP. Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases. Cardiovasc Pathol 2015; 24:267-78. [PMID: 26051917 DOI: 10.1016/j.carpath.2015.05.001] [Citation(s) in RCA: 230] [Impact Index Per Article: 23.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2015] [Revised: 05/11/2015] [Accepted: 05/11/2015] [Indexed: 10/23/2022] Open
Abstract
Inflammatory diseases of the aorta include routine atherosclerosis, aortitis, periaortitis, and atherosclerosis with excessive inflammatory responses, such as inflammatory atherosclerotic aneurysms. The nomenclature and histologic features of these disorders are reviewed and discussed. In addition, diagnostic criteria are provided to distinguish between these disorders in surgical pathology specimens. An initial classification scheme is provided for aortitis and periaortitis based on the pattern of the inflammatory infiltrate: granulomatous/giant cell pattern, lymphoplasmacytic pattern, mixed inflammatory pattern, and the suppurative pattern. These inflammatory patterns are discussed in relation to specific systemic diseases including giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis (Wegener's), rheumatoid arthritis, sarcoidosis, ankylosing spondylitis, Cogan syndrome, Behçet's disease, relapsing polychondritis, syphilitic aortitis, and bacterial and fungal infections.
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Affiliation(s)
| | | | | | | | | | | | - L Maximilian Buja
- University of Texas Health Science Center at Houston, Houston, TX, USA
| | | | | | | | | | | | | | | | | | - Ornella Leone
- Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
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24
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Ozaki T, Maeshima K, Kiyonaga Y, Torigoe M, Imada C, Hamasaki H, Haranaka M, Ishii K, Shibata H. Large-vessel involvement in granulomatosis with polyangiitis successfully treated with rituximab: A case report and literature review. Mod Rheumatol 2015; 27:699-704. [PMID: 25736357 DOI: 10.3109/14397595.2015.1021950] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
Granulomatosis with polyangiitis (GPA) is primary necrotizing vasculitis, which predominantly affects small to medium vessels. Herein, we describe a case of a 60-year-old female with GPA who developed inflammatory wall thickening localized in the aortic arch, upper abdominal aorta, and pulmonary artery. The wall thickening in the large vessels and other GPA lesions such as lung nodules and orbital mass had failed to respond to high-dose glucocorticoids combined with cyclophosphamide; however, all were successfully treated with rituximab. Our literature review identified 24 cases of large-vessel involvement associated with GPA. Luminal stenosis, occlusion, or wall thickening were observed in 8, periaortitis in 11, and aneurysms in 5 cases. The most commonly affected vessel was the abdominal aorta (12 cases), followed by the thoracic aorta (6 cases), subclavian artery (4 cases), and internal carotid artery (4 cases). Glucocorticoids were used in 23 cases, 20 of which received combination therapy with cyclophosphamide. Surgical or endovascular therapies were performed in 10 cases with aneurysmal dilatation. This is the first case showing the potential efficacy of rituximab for refractory large-vessel involvement associated with GPA.
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Affiliation(s)
- Takashi Ozaki
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Keisuke Maeshima
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Yasuhiro Kiyonaga
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Masataka Torigoe
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Chiharu Imada
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Hajime Hamasaki
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Miwa Haranaka
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Koji Ishii
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
| | - Hirotaka Shibata
- a Department of Endocrinology , Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University , Yufu , Oita , Japan
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25
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Maleszewski JJ. Inflammatory ascending aortic disease: Perspectives from pathology. J Thorac Cardiovasc Surg 2015; 149:S176-83. [DOI: 10.1016/j.jtcvs.2014.07.046] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/21/2014] [Accepted: 07/24/2014] [Indexed: 12/11/2022]
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Ohta N, Waki T, Fukase S, Suzuki Y, Kurakami K, Aoyagi M, Kakehata S. Aortic aneurysm rupture as a rare complication of granulomatosis with polyangiitis: a case report. J Med Case Rep 2013; 7:202. [PMID: 23889996 PMCID: PMC3750224 DOI: 10.1186/1752-1947-7-202] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2012] [Accepted: 03/05/2013] [Indexed: 12/03/2022] Open
Abstract
Introduction Granulomatosis with polyangiitis is characterized by systemic inflammation of medium and small blood vessels. Aortic involvement in granulomatosis with polyangiitis is extremely rare. As far as we know this is the first reported case of successful treatment in a patient with granulomatosis with polyangiitis complicated with aortic aneurysm rupture. Case presentation We describe a case of granulomatosis with polyangiitis in a 38-year-old Japanese man who developed an aortic aneurysm rupture 22 years after disease onset. The patient was operated on and a J-graft was inserted. He recovered uneventfully. Conclusion Recommendations in regard to, and consideration of, aortic involvement should be kept in mind in the long-term careful follow up of granulomatosis with polyangiitis.
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Affiliation(s)
- Nobuo Ohta
- Department of Otolaryngology, Yamagata University Faculty of Medicine, 2-2-2 Iida-nishi, Yamagata, 990-23, Japan.
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27
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Une aortite dans une maladie de Wegener. Rev Med Interne 2012. [DOI: 10.1016/j.revmed.2012.03.270] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
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28
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Fujii K, Hidaka Y. Churg-Strauss syndrome complicated by chronic periaortitis: a case report and review of the literature. Intern Med 2012; 51:109-12. [PMID: 22214634 DOI: 10.2169/internalmedicine.51.5985] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
We present a case of Churg-Strauss syndrome complicated by chronic periaortitis. A 68-year-old man presented with wheezing, dyspnea, purpurae, and numbness of the extremities. Antineutrophil cytoplasmic antibodies were absent; however, eosinophilia, a pulmonary infiltrative shadow on chest X-ray, eosinophilic vasculitis on histologic examination of skin and kidney, and mononeuritis multiplex were detected. Churg-Strauss syndrome was diagnosed. Contrast-enhanced abdominal computed tomography revealed a periaortic soft tissue mass extending from the subphrenic abdominal aorta to the proximal area of the bilateral iliac arteries. This indicated chronic periaortitis, probably caused by vasculitic activities. Both disorders improved with steroid therapy.
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Affiliation(s)
- Kenji Fujii
- Center for Rheumatic Disease, Iizuka Hospital, Japan.
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29
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30
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Hydronephrosis caused by a relapse of granulomatosis with polyangiitis (Wegener's). Mod Rheumatol 2011; 22:616-20. [PMID: 22065104 DOI: 10.1007/s10165-011-0554-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/20/2011] [Accepted: 10/20/2011] [Indexed: 10/15/2022]
Abstract
We describe a case of relapsed granulomatosis with polyangiitis (Wegener's) (GPA) that presented with abdominal pain. (18)F-fluoro-2-deoxy-D: -glucose positron emission tomography (FDG-PET)/computed tomography (CT) clearly depicted an inflammation of the left peri-iliac arterial soft tissue, which was thought to be the cause of the ureteral obstruction and hydronephrosis. Our case shows that peri-iliac arterial inflammation occurs in GPA and causes hydronephrosis. In addition, FDG-PET/CT is a useful tool for management of this systemic inflammatory disease.
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31
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Ünlü Ç, Willems M, Ten Berge IJ, Legemate DA. Aortitis with aneurysm formation as a rare complication of Wegener's granulomatosis. J Vasc Surg 2011; 54:1485-7. [DOI: 10.1016/j.jvs.2011.05.014] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2011] [Revised: 05/02/2011] [Accepted: 05/03/2011] [Indexed: 12/28/2022]
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32
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Epidural spinal tumor and periaortitis as rare complications of Wegener's granulomatosis. Mod Rheumatol 2011; 21:678-83. [PMID: 21691846 DOI: 10.1007/s10165-011-0456-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2011] [Accepted: 04/05/2011] [Indexed: 10/18/2022]
Abstract
This case report describes findings in a 61-year-old woman who manifested scleritis, small pulmonary nodules, otitis media, periaortitis, and progressive epidural spinal tumor, associated with elevated serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) levels. She was clinically diagnosed with Wegener's granulomatosis, although vasculitis was not diagnosed due to the lack of typical histological findings. We discuss the differential diagnosis in this patient, and the association of MPO-ANCA with periaortitis or epidural spinal tumor.
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33
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Salvarani C, Calamia KT, Matteson EL, Hunder GG, Pipitone N, Miller DV, Warrington KJ. Vasculitis of the gastrointestinal tract in chronic periaortitis. Medicine (Baltimore) 2011; 90:28-39. [PMID: 21200184 DOI: 10.1097/md.0b013e318207231e] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
The term "chronic periaortitis" (CP), proposed by Mitchinson in 1984, comprises 3 main entities: idiopathic retroperitoneal fibrosis (IRF), inflammatory abdominal aortic aneurysms (IAAAs), and perianeurysmal retroperitoneal fibrosis (PRF).The presence of constitutional symptoms, high acute-phase reactants, positive autoantibodies, and associated autoimmune diseases suggests a systemic inflammatory process. Histopathologic findings show vasculitis with fibrinoid necrosis involving the aortic vasa vasorum as well as the small and medium retroperitoneal vessels.We reviewed the medical records of 608 patients with a diagnosis of vasculitis involving the gastrointestinal (GI) tract at the Mayo Clinic between January 1996 and December 2007. Only patients with biopsy-proven or typical angiographic findings of vasculitis localized to the GI tract were included.Five patients were identified with evidence of CP (1 patient with PRF, 1 with IRF, and 3 with IAAAs). Three patients were men, and the median age at diagnosis was 49 years. The diagnosis of GI vasculitis and CP was made simultaneously in 4 patients. At the time of onset, all patients had abdominal pain and constitutional manifestations; the median erythrocyte sedimentation rate was 62.5 mm/1 h (range, 20-86 mm/1 h). All patients had evidence of mesenteric vasculitis at angiography. Three patients also had associated renal artery stenoses. Abdominal computed tomography showed spleen infarcts in 2 patients, bowel wall thickening in 1, and liver infarction in 1. Two patients underwent surgical intervention for acute abdomen; there was histologic evidence of small bowel infarcts and infarction of the spleen and liver in 1. Oral prednisone was administered to all 5 patients (median starting dose, 60 mg/d; range, 25-80 mg/d). Three patients also received immunosuppressive agents, 1 tamoxifen, and 1 anti-tumor necrosis factor therapy. All patients had at least 1 relapse or recurrence of vasculitis, but at last visit, GI vasculitis and CP were in remission in all 5 patients.This study provides evidence that GI manifestations due to mesenteric vasculitis may be associated with CP. Vasculitic involvement of the renal arteries is also frequently present in these patients. Aggressive immunosuppressive treatment should be promptly initiated to forestall abdominal complications. These findings reinforce the hypothesis that a vasculitic process plays an important role in the pathogenesis of CP.
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Affiliation(s)
- Carlo Salvarani
- From Unità di Reumatologia (CS, NP), Arcispedale S. Maria Nuova, Reggio Emilia, Italy; Division of Rheumatology (KTC), Mayo Clinic, Jacksonville, Florida; Division of Rheumatology (ELM, GGH, KJW), and Division of Anatomic Pathology (DVM), Mayo Clinic, Rochester, Minnesota
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Abstract
This review focuses on recent developments in the treatment of inflammatory neuropathies arising from immune dysregulation, rather than from infectious causes. The dysimmune inflammatory neuropathies are diseases of the peripheral nerves that have varying etiologies and may respond to immunomodulatory therapies. They are characterized by inflammatory changes in the nerve with associated destruction of myelin and axons. The underlying immune mechanisms are better understood in some of these conditions than others. Correct diagnosis and treatment is important to prevent clinical progression. Randomized controlled trials of some treatments in the more common inflammatory neuropathies have clarified their effectiveness; however, there are still groups of patients who are resistant to currently available treatments and for whom little effective treatment is available. Newer, targeted biologics and larger controlled trials of existing and novel therapies in these conditions offer promise of improved morbidity and mortality in this group of diseases.
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Affiliation(s)
- Elspeth J Hutton
- Centre for Neuromuscular Diseases and Department of Molecular Neuroscience, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
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35
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Durai R, Agrawal R, Piper K, Brohi K. Wegener's granulomatosis presenting as an abdominal aortic aneurysm: a case report. CASES JOURNAL 2009; 2:9346. [PMID: 20066062 PMCID: PMC2804726 DOI: 10.1186/1757-1626-2-9346] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/30/2009] [Accepted: 12/18/2009] [Indexed: 12/23/2022]
Abstract
Introduction Aortic aneurysm is not common in young patient. When a young patient presents with abdominal aortic aneurysm, there may be an underlying cause. Case presentation Here, we describe a case of a 33-year-old gentleman who presented with flu like illness, chest and abdominal pains following a tooth extraction. A chest X-ray and subsequent computerised tomogram of the chest and abdomen demonstrated lung nodules and an abdominal aortic aneurysm. The aneurysm was repaired and his serology was positive for Wegener's granulomatosis. A nasal mucosal biopsy confirmed WG. He was treated with oral steroids and cyclophosphamide. His graft leaked and had to be replaced with a synthetic graft. Two months after his re-operation, he remains well. Conclusion Whenever a young patient presents with an abdominal aortic aneurysm, an underlying connective disease should be excluded because early steroid/immunosuppressive treatment may prevent the development of further aneurysms.
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Affiliation(s)
- Rajaraman Durai
- Department of Vascular Surgery, The Royal London Hospital, Whitechapel Road, Whitechapel, London, E1 1BB, UK
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36
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Minnee RC, van den Berk GEL, Groeneveld JO, van Dijk J, Turkcan K, Visser MJ, Vahl AC. Aortic aneurysm and orchitis due to Wegener's granulomatosis. Ann Vasc Surg 2009; 23:786.e15-9. [PMID: 19748223 DOI: 10.1016/j.avsg.2009.06.011] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2008] [Revised: 02/16/2009] [Accepted: 06/08/2009] [Indexed: 12/30/2022]
Abstract
We present a patient with Wegener's granulomatosis (WG) with involvement of the abdominal aorta, testis, peripheral nerve system, and skin. A 51-year-old man presented at our outpatient clinic with lower back pain. He had a history of smoking, hypertension, and an embryonal carcinoma of the left testis, treated 13 years ago with orchidectomy and chemotherapy. One month earlier, he underwent a partial orchidectomy of the right testis due to testicular swelling. Abdominal computed tomography showed a 3.8 cm wide aneurysm of the distal part of the aorta with inflammation. One week later he was admitted to the hospital with numbness of his hands and feet. Physical examination showed signs of peripheral microemboli. Serological laboratory tests revealed elevated antineutrophil cytoplasmic antibody titers with positive reactions against proteinase-3, indicating Wegener's disease. The chest X-ray was normal. Pathological examination of the right testis showed necrotizing vasculitis of a small artery. He was treated with cyclophosphamide and prednisolone. WG with extrapulmonary involvement occurs infrequently, and reports of manifestations of WG in aorta, testis, the peripheral nerve system, and skin are even more uncommon. Small- and medium-vessel vasculitis can precede large-vessel vasculitis or occur in the absence of small-vessel involvement. Therefore, WG should be included in the work-up of large-vessel vasculitis, which can give rise to periaortic inflammation.
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Affiliation(s)
- R C Minnee
- Department of Vascular Surgery, Onze Lieve Vrouwe Gasthuis, Amsterdam, the Netherlands
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37
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Abstract
The inflammatory neuropathies are a large diverse group of immune-mediated neuropathies that are amenable to treatment and may be reversible. Their accurate diagnosis is essential for informing the patient of the likely course and prognosis of the disease, informing the treating physician of the appropriate therapy and informing the scientific community of the results of well-targeted, designed and performed clinical trials. With the advent of biological therapies able to manipulate the immune response more specifically, an understanding of the pathogenesis of these conditions is increasingly important. This review presents a broad overview of the pathogenesis, diagnosis and therapy of inflammatory neuropathies, concentrating on the most commonly encountered conditions.
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Affiliation(s)
- M P T Lunn
- Centre for Neuromuscular Disease and Department of Molecular Neuroscience, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
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38
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Hervier B, Masseau A, Bossard C, Agard C, Hamidou M. Vasa-vasoritis of the aorta and fatal myocarditis in fulminant Churg-Strauss syndrome. Rheumatology (Oxford) 2008; 47:1728-9. [PMID: 18775968 DOI: 10.1093/rheumatology/ken329] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
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Arlet JB, Le Thi Huong D, Marinho A, Cluzel P, Wechsler B, Piette JC. Arterial Aneurysms in Wegener’s Granulomatosis: Case Report and Literature Review. Semin Arthritis Rheum 2008; 37:265-8. [PMID: 17888497 DOI: 10.1016/j.semarthrit.2007.07.004] [Citation(s) in RCA: 32] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/18/2007] [Revised: 06/22/2007] [Accepted: 07/17/2007] [Indexed: 01/17/2023]
Abstract
OBJECTIVE Arterial aneurysms are characteristic of medium-size vessel vasculitis but are a very unusual feature of Wegener's granulomatosis (WG). We describe a typical WG case, complicated by arterial aneurysms and review previously reported cases. METHODS Medline database search of cases published between January 1978 and July 2006, in English, reporting arterial aneurysms complicating WG. RESULTS Five years after diagnosis, a 29-year-old man with typical WG developed macro- and microaneurysms located on branches of the hepatic and renal arteries during a disease relapse. The main symptoms were abdominal pain, vomiting, and altered general status. He was successfully treated by coil embolization in combination with prednisone, intravenous mycophenolate mofetil, and high-dose immunoglobulins. Twelve additional cases of WG complicated by arterial aneurysms are reported in the English literature. This represents a life-threatening complication since rupture occurred in half of the patients. CONCLUSIONS Although small-vessel injury predominates in WG, inflammation of medium-size arteries may occur and lead to aneurysm formation. Abdominal angiography should be recommended when unexplained abdominal pain occurs during a WG flare.
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Affiliation(s)
- Jean-Benoit Arlet
- Department of Internal Medicine, La Pitié-Salpétrière Hospital, Paris, France.
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40
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Breynaert C, Cornelis T, Stroobants S, Bogaert J, Vanhoof J, Blockmans D. Letter To the Editor. Lupus 2008; 17:72-4. [DOI: 10.1177/0961203307084172] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Affiliation(s)
| | | | | | - J. Bogaert
- Radiology Department, University Hospital Gasthuisberg, Leuven, Belgium
| | - J. Vanhoof
- Rheumatology Department, Biomedical Research Institute, University Hasselt, Belgium
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Vaglio A, Greco P, Corradi D, Palmisano A, Martorana D, Ronda N, Buzio C. Autoimmune aspects of chronic periaortitis. Autoimmun Rev 2006; 5:458-64. [PMID: 16920572 DOI: 10.1016/j.autrev.2006.03.011] [Citation(s) in RCA: 81] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2006] [Accepted: 03/12/2006] [Indexed: 11/17/2022]
Abstract
Chronic periaortitis (CP) includes idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis. These entities are characterised by a fibro-inflammatory tissue which develops around the abdominal aorta and the iliac arteries, and spreads into the surrounding retroperitoneum to entrap adjacent structures such as the ureters. CP often affects patients with advanced atherosclerosis, and several lines of evidence support the view that it could result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta such as oxidised-low density lipoproteins and ceroid. However, because most CP patients also suffer from constitutional symptoms and show elevated acute-phase reactant levels, positive autoantibodies and, in some cases, autoimmune diseases affecting other organs, CP may also be considered a manifestation of a systemic autoimmune disease. CP is usually diagnosed using computed tomography or magnetic resonance imaging, but retroperitoneal biopsy may also be necessary; positron emission tomography is useful in assessing the full extent of the disease and the metabolic activity of the retroperitoneal tissue. Ureterolysis and aneurysm repair are frequently performed, but the inflammatory and chronic-relapsing nature of the disease often compels the use of medical therapy, which is based on steroids and immunosuppressants.
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Affiliation(s)
- Augusto Vaglio
- Department of Clinical Medicine, Nephrology and Health Science, University of Parma, Italy.
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Chirinos JA, Corrales VF, Lichtstein DM. ANCA-associated large vessel compromise. Clin Rheumatol 2005; 25:111-2. [PMID: 15980936 DOI: 10.1007/s10067-005-1139-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2004] [Revised: 12/17/2004] [Accepted: 12/17/2004] [Indexed: 10/25/2022]
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