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1
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Lequain H, Streichenberger N, Gallay L, Gerfaud-Valentin M, Fenouil T, Bonjour M, Roux KL, Jamilloux Y, Leblanc P, Sève P. Granulomatous myositis: characteristics and outcome from a monocentric retrospective cohort study. Neuromuscul Disord 2024; 42:5-13. [PMID: 39059057 DOI: 10.1016/j.nmd.2024.06.007] [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: 03/14/2024] [Revised: 06/22/2024] [Accepted: 06/25/2024] [Indexed: 07/28/2024]
Abstract
Granulomatous myositis is a clinical-pathological entity, which has been rarely reported, mostly described in sarcoidosis. Currently, no clear and simple prognostic factor has been identified to predict granulomatous myositis evolution. The clinical, anatomopathological, imaging, and biological characteristics of 26 patients with granulomatous myositis were retrospectively collected to describe clinical presentation and outcomes of this condition. Twenty-six patients with granulomatous myositis were included (14 males) with a median age of symptom onset of 65 years. 54 % of patients presented a severe form of the disease defined as a Rankin score ≥2 at last follow-up visit or a progressive form of the disease (no improvement under treatment). Etiology were sarcoidosis (n = 14), inclusion body myositis (n = 4), autoimmune disease (n = 1), hematological malignancy (n = 1), and idiopathic (n = 6). Distal deficit and amyotrophy were more frequent among those with a severe disease. Corticosteroids led to improvement in 75 % of cases, but 66 % of responders relapsed. Methotrexate appeared as a promising second line therapy with clinical improvement in 50 % of patients, and no relapse in responders. Granulomatous myositis is often a severe and difficult-to-treat disease in which patients frequently progress towards severe disability. The presence of muscle atrophy and distal weakness appears to be frequently associated with a severe form of the disease.
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Affiliation(s)
- Hippolyte Lequain
- Department of Internal Medicine, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Université Claude Bernard-Lyon 1, Lyon, France
| | - Nathalie Streichenberger
- Department of Pathology, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, Université Claude Bernard-Lyon1, Lyon, France; Faculté de Médecine Rockefeller, Institut NeuroMyoGène INMG-PGNM, Physiopathologie et Génétique du Neurone et du Muscle, UMR5261, INSERM U1315, Université Claude Bernard-Lyon1, Lyon, France
| | - Laure Gallay
- Department of Internal Medicine, Hôpital Édouard Herriot, Hospices Civils de Lyon, Lyon, France
| | - Mathieu Gerfaud-Valentin
- Department of Internal Medicine, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Université Claude Bernard-Lyon 1, Lyon, France
| | - Tanguy Fenouil
- Department of Pathology, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, Université Claude Bernard-Lyon1, Lyon, France
| | - Maxime Bonjour
- Service de Biostatistique, Hospices Civils de Lyon, Université Claude Bernard-Lyon 1, Lyon, France
| | - Karine Le Roux
- Department of Internal Medicine, Centre hospitalier Métropole Savoie, Aix-les-Bains, France
| | - Yvan Jamilloux
- Department of Internal Medicine, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Université Claude Bernard-Lyon 1, Lyon, France
| | - Pascal Leblanc
- Faculté de Médecine Rockefeller, Institut NeuroMyoGène INMG-PGNM, Physiopathologie et Génétique du Neurone et du Muscle, UMR5261, INSERM U1315, Université Claude Bernard-Lyon1, Lyon, France
| | - Pascal Sève
- Department of Internal Medicine, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Université Claude Bernard-Lyon 1, Lyon, France; Research on Healthcare Performance (RESHAPE), U129-INSERM, Université Claude Bernard-Lyon 1, Lyon, France.
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2
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Berner A, Coen M, Egervari K, Lobrinus JA, Grosjean A, Gressot P, Ribeiro Da Costa R, Farhoumand PD, Serratrice J. When Crouching Gait Reveals Crohn's Disease. Am J Med 2024; 137:414-416. [PMID: 38043886 DOI: 10.1016/j.amjmed.2023.11.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2023] [Accepted: 11/17/2023] [Indexed: 12/05/2023]
Affiliation(s)
- Amandine Berner
- Division of General Internal Medicine, Department of Medicine, Geneva University Hospitals, Switzerland.
| | - Matteo Coen
- Division of General Internal Medicine, Department of Medicine, Geneva University Hospitals, Switzerland; Unit of Development and Research in Medical Education, Faculty of Medicine, University of Geneva, Switzerland
| | - Kristof Egervari
- Division of Clinical Pathology, Diagnostic Department, Geneva University Hospitals, Switzerland
| | | | - Alicia Grosjean
- Division of General Internal Medicine, Department of Medicine, Geneva University Hospitals, Switzerland
| | - Pablo Gressot
- Division of Gastroenterology and Hepatology, Department of Medicine, Geneva University Hospitals, Switzerland
| | - Rui Ribeiro Da Costa
- Division of General Internal Medicine, Department of Medicine, Geneva University Hospitals, Switzerland
| | | | - Jacques Serratrice
- Division of General Internal Medicine, Department of Medicine, Geneva University Hospitals, Switzerland
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3
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Chompoopong P, Liewluck T. Granulomatous myopathy: Sarcoidosis and beyond. Muscle Nerve 2023; 67:193-203. [PMID: 36352751 DOI: 10.1002/mus.27741] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2022] [Revised: 10/09/2022] [Accepted: 10/11/2022] [Indexed: 11/11/2022]
Abstract
Non-necrotizing granulomatous inflammation is a rare but easily recognized histopathological finding in skeletal muscle biopsy. A limited number of diseases are known to be associated with non-necrotizing granulomatous myopathy. Once identified, a careful evaluation for evidence of extramuscular granulomatosis and other signs suggestive of sarcoidosis is warranted as about half of the patients have sarcoid myopathy. In addition, the presence of granulomatous myopathy should trigger a search for clinical and pathological clues of inclusion body myositis (IBM), which accounts for most of the remaining patients and can coexist with sarcoidosis. Recognizing the features of IBM in patients with granulomatous myopathy can potentially spare the patients from unnecessary exposure to immunosuppressive therapies. In patients whose granulomatous myopathy remain unexplained, further investigations should aim at identifying myasthenia gravis and other autoimmune disorders, especially those known to cause granulomatous inflammation in other organs. Laboratory investigations should include acetylcholine receptor, antimitochondrial, antineutrophil cytoplasmic, thyroglobulin, and thyroid peroxidase autoantibodies. In the appropriate clinical context, exposure to immune checkpoint inhibitors and chronic graft-vs-host disease can be causes of granulomatous myopathy. In cases of unexplained granulomatous myopathy, natural killer/T-cell lymphoma should be considered and careful histopathological examination for atypical cells and appropriate immunostaining is crucial. Identifying the etiology of granulomatous myopathy in each patient can guide appropriate treatment.
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4
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[Differential diagnosis of a vasculitic syndrome of the lower limb]. Z Rheumatol 2022; 81:871-880. [PMID: 34241690 DOI: 10.1007/s00393-021-01044-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/07/2021] [Indexed: 12/16/2022]
Abstract
Vasculitides can present with various clinical signs and symptoms. Besides disease-specific organ manifestations, the skin, peripheral nerves and musculature are frequently involved. The combination of elevated serological inflammatory markers, vasculitic skin lesions, active polyneuropathy and immobilizing myalgia of the lower limb musculature is highly suspicious for muscular polyarteritis nodosa (mPAN). Based on the case of a 63-year-old female patient with a vasculitic syndrome confined to the lower limb due to mPAN, important differential diagnoses of the these disease manifestations are discussed. Magnetic resonance imaging of the affected muscles and subsequent muscle biopsy (including skin and fascia) provide the relevant diagnostic data.
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5
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Catherine J, Kadhim H, Lambot F, Liefferinckx C, Meurant V, Otero Sanchez L. Crohn’s disease-related ‘gastrocnemius myalgia syndrome’ successfully treated with infliximab: A case report. World J Gastroenterol 2022; 28:755-762. [PMID: 35317272 PMCID: PMC8891723 DOI: 10.3748/wjg.v28.i7.755] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/30/2021] [Revised: 12/13/2021] [Accepted: 01/20/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Extra-intestinal manifestations in inflammatory bowel diseases (IBD) are frequent and involve virtually all organs. Conversely, the clinical characteristics and course of inflammatory myopathies in IBD remain poorly described and mostly related to orbital myositis. Moreover, alternative therapeutic strategies in non-responder patients to corticosteroid therapy must still be clarified.
CASE SUMMARY A 33-year-old woman with a history of unclassified colitis presented with acute bilateral calf pain. On admission, her clinical and biological examinations were non-specific. However, magnetic resonance imaging showed bilateral inflammatory changes in gastrocnemius muscles suggestive of myositis. Muscle biopsy confirmed the diagnosis of myositis and demonstrated an inflammatory infiltrate mainly located in the perimysial compartment including lympho-plasmocytic cells with the formation of several granulomatous structures while the endomysium was relatively spared. The combined clinical, biological and histomyopathological findings were concordant with the diagnosis of ‘gastrocnemius myalgia syndrome’ (GMS), a rare disorder associated with Crohn’s disease (CD). Ileocolonoscopy confirmed CD diagnosis and systemic corticosteroids (CS) therapy was started, resulting in a rapid clinical improvement. During CS tapering, however, she experienced a relapse of GMS together with a severe active ileocolitis. Infliximab was started and allowed a sustained remission of both conditions at the latest follow-up (20 mo).
CONCLUSION The GMS represent a rare CD-associated inflammatory myopathy for which anti-tumour necrosis factor-α therapy might be considered as an effective therapeutic option.
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Affiliation(s)
- Julien Catherine
- Institute for Medical Immunology, Université Libre de Bruxelles, Gosselies 6041, Belgium
- Department of Internal Medicine, C.U.B. Hôpital Erasme, Brussels 1070, Belgium
| | - Hazim Kadhim
- NeuroMyopathology Unit (Anatomic Pathology Service) and Reference Center for Neuromuscular Pathology, CHU Brugmann, Université Libre de Bruxelles, Brussels 1020, Belgium
| | - Frédéric Lambot
- Department of Internal Medicine, Centre Hospitalier Universitaire Tivoli, La Louvière 7100, Belgium
| | - Claire Liefferinckx
- Department of Gastroenterology, Hepatopancreatology and Digestive Oncology, C.U.B. Hôpital Erasme, Brussels 1070, Belgium
- Laboratory of Experimental Gastroenterology, Université Libre de Bruxelles, Brussels 1070, Belgium
| | - Virginie Meurant
- Department of Emergency Medicine, Centre Hospitalier Universitaire Tivoli, La Louvière 7100, Belgium
| | - Lukas Otero Sanchez
- Department of Gastroenterology, Hepatopancreatology and Digestive Oncology, C.U.B. Hôpital Erasme, Brussels 1070, Belgium
- Laboratory of Experimental Gastroenterology, Université Libre de Bruxelles, Brussels 1070, Belgium
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6
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Mertz P, Lannes B, Nadaj Pakleza A, Meyer A, Guffroy A. Bilateral gastrocnemius myositis: an extra-intestinal manifestation of Crohn's disease. Rheumatology (Oxford) 2021; 61:e35-e37. [PMID: 34534274 DOI: 10.1093/rheumatology/keab593] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2021] [Revised: 07/19/2021] [Accepted: 07/21/2021] [Indexed: 11/14/2022] Open
Affiliation(s)
- Philippe Mertz
- Department of Clinical Immunology and Internal Medicine, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Tertiary Center for Primary Immunodeficiencies, Strasbourg, France
| | - Béatrice Lannes
- Department of Pathology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
| | - Aleksandra Nadaj Pakleza
- Department of Neurology, National Reference Centre for Neuromuscular Diseases, Strasbourg, France
| | - Alain Meyer
- Department of Rheumatology and Department of Physiology, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Strasbourg, France
| | - Aurélien Guffroy
- Department of Clinical Immunology and Internal Medicine, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Tertiary Center for Primary Immunodeficiencies, Strasbourg, France
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7
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Affiliation(s)
- Eric M Drabble
- Department of SurgeryJohn Hunter Hospital, Lookout Road, New Lambton Heights, NSW 2305
| | - Jonathon S Gani
- Department of SurgeryJohn Hunter Hospital, Lookout Road, New Lambton Heights, NSW 2305
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Alhammad RM, Liewluck T. Myopathies featuring non-caseating granulomas: Sarcoidosis, inclusion body myositis and an unfolding overlap. Neuromuscul Disord 2018; 29:39-47. [PMID: 30578101 DOI: 10.1016/j.nmd.2018.10.007] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2018] [Revised: 10/23/2018] [Accepted: 10/31/2018] [Indexed: 11/18/2022]
Abstract
Granulomatous myopathies are etiologically heterogeneous myopathies, pathologically characterized by the presence of intramuscular granulomas. Treatment outcomes are variable. We aimed to identify prognostic factors of treatment outcomes in myopathies featuring non-caseating granulomas. Sixteen patients were identified (9 sarcoid myopathy, 6 inclusion body myositis, and 1 granulomatous myopathy of indeterminate cause) over a 21-year period. The median age at diagnosis was 67 years in sarcoid myopathy group, and 64 years in inclusion body myositis group. Three inclusion body myositis patients were initially diagnosed with sarcoid myopathy based on the presence of systemic features of sarcoidosis and findings on muscle biopsies, but subsequent biopsies performed because of treatment refractoriness, showed all canonical pathologic features of inclusion body myositis. We identified sarcoplasmic congophilic inclusions in 6 sarcoid myopathy patients without associated rimmed vacuoles or typical weakness pattern of inclusion body myositis. Four inclusion body myositis and 4 of 5 sarcoid myopathy patients with congophilic inclusions were refractory to immunotherapy. Our study portrays the overlapping clinical and pathological features of sarcoid myopathy and inclusion body myositis. The presence of sarcoplasmic congophilic inclusions in sarcoid myopathy may predict an unfavorable outcome of immunosuppressive therapy, but a larger prospective study is required to further validate this observation.
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Affiliation(s)
- Reem M Alhammad
- Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA; Section of Neurology, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia
| | - Teerin Liewluck
- Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
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9
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Osada A, Yamada H, Takehara S, Tozuka Y, Fukushima T, Oka H, Okazaki H, Nagaoka S. Gastrocnemius Myalgia as a Rare Initial Manifestation of Crohn's Disease. Intern Med 2018; 57:2001-2006. [PMID: 29491286 PMCID: PMC6096017 DOI: 10.2169/internalmedicine.0327-17] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
The initial symptoms of Crohn's disease (CD) sometimes present as extraintestinal lesions, which can be a diagnostic challenge for physicians. Painful legs, known as "gastrocnemius myalgia syndrome", are rare complications that often precede abdominal manifestations. We herein report the case of a 38-year-old man who presented with bilateral leg myalgia lasting for 4 months. Magnetic resonance imaging showed abnormal intensity, and a muscle biopsy revealed inflammatory cell infiltration. Abdominal symptoms appeared three months after the myalgia onset, and the diagnosis of CD was confirmed later by endoscopic and radiological findings. To our knowledge, this is the first description of gastrocnemius myalgia syndrome in Japan.
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Affiliation(s)
- Atsumu Osada
- Department of Rheumatology, Yokohama Minami Kyosai Hospital, Japan
| | - Hiroaki Yamada
- Department of Gastroenterology, Kosinkai Shiomidai Hospital, Japan
| | - Sayuri Takehara
- Department of Gastroenterology, Yokohama Minami Kyosai Hospital, Japan
| | | | - Taito Fukushima
- Department of Gastroenterology, Kanagawa Cancer Center, Japan
| | - Hiroyuki Oka
- Department of Gastroenterology, Yokohama Minami Kyosai Hospital, Japan
| | - Hiroshi Okazaki
- Department of Gastroenterology, Yokohama Minami Kyosai Hospital, Japan
| | - Shohei Nagaoka
- Department of Rheumatology, Yokohama Minami Kyosai Hospital, Japan
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10
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Khadilkar SV, Yadav RS, Patel BA. Idiopathic Inflammatory Myopathies. Neuromuscul Disord 2018. [DOI: 10.1007/978-981-10-5361-0_11] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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Abstract
There is a growing interest in the extraintestinal manifestations of common pediatric gastrointestinal diseases, such as inflammatory bowel disease and celiac disease. This article specifically focuses on the neurological symptoms that manifest because of these disorders and their treatments. Many neurological symptoms have been reported in association with these diseases, including neuropathy, myopathy, ataxia, headache, and seizures, among others. It is currently believed that these neurological symptoms are largely overlooked by practitioners and could be a red flag for earlier diagnosis. However, additional research, especially in the pediatric population, is warranted to further elaborate on the causality and pathophysiology of these neurological symptoms.
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Affiliation(s)
- Melissa Shapiro
- From the Section of Gastroenterology, Department of Pediatrics, Drexel University College of Medicine, St. Christopher's Hospital for Children, Philadelphia, PA
| | - David A Blanco
- From the Section of Gastroenterology, Department of Pediatrics, Drexel University College of Medicine, St. Christopher's Hospital for Children, Philadelphia, PA.
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Vadala di Prampero S, Marino M, Toso F, Avellini C, Nguyen V, Sorrentino D. Isolated Bilateral Gastrocnemius Myositis in Crohn Disease Successfully Treated with Adalimumab. Case Rep Gastroenterol 2016; 10:661-667. [PMID: 27920658 PMCID: PMC5126597 DOI: 10.1159/000448880] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2016] [Accepted: 08/01/2016] [Indexed: 12/25/2022] Open
Abstract
Extraintestinal manifestations are common in inflammatory bowel disease; however, muscular involvement in Crohn disease is rarely reported. We present a case of a 26-year-old male with ileocolonic Crohn disease who developed sudden tenderness in both calves. Doppler ultrasound was negative for deep vein thrombosis. Magnetic resonance imaging of the gastrocnemius muscle showed high intensity signal in the muscle fibers, and muscle biopsy demonstrated nonspecific lymphocytic myositis. Other relevant laboratory results included normal antineutrophil cytoplasmic antibodies and creatine kinase as well as elevated C-reactive protein, erythrocyte sedimentation rate, and anti-Saccharomyces cerevisiae IgG titer. The patient was in clinical remission, being treated with azathioprine 2.5 mg/kg. Prednisone 60 mg/day was initiated with rapid resolution of calf tenderness; however, tenderness soon returned when the dose was tapered to 10 mg/day. Subsequently, prednisone and azathioprine were discontinued, and adalimumab was started at standard induction and maintenance doses. The patient's symptoms resolved shortly after the first induction dose. A repeat magnetic resonance imaging of the calves – 3 months after starting adalimumab – showed complete resolution of muscle inflammation. To our knowledge, this is the first case of gastrocnemius myositis – a rare extraintestinal manifestation of Crohn disease – successfully treated with anti-tumor necrosis factor agents.
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Affiliation(s)
| | - Marco Marino
- Department of Pathology, University Hospital of Udine, Udine, Italy
| | - Francesco Toso
- Department of Pathology, University Hospital of Udine, Udine, Italy
| | - Claudio Avellini
- Department of Pathology, University Hospital of Udine, Udine, Italy
| | - Vu Nguyen
- Department of Internal Medicine. Virginia Tech Carilion School of Medicine, Roanoke, VA, USA
| | - Dario Sorrentino
- Department of Pathology, University Hospital of Udine, Udine, Italy; Department of Internal Medicine. Virginia Tech Carilion School of Medicine, Roanoke, VA, USA; Department of Clinical and Experimental Medical Sciences, University of Udine School of Medicine, Udine, Italy
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13
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Scalco RS, Brady S, Becker J, Gomes I, Holton JL, Staub HL. LETTER TO THE EDITOR Atypical Granulomatous Myositis and Pulmonary Sarcoidosis. Open Rheumatol J 2015; 9:57-9. [PMID: 26312107 PMCID: PMC4541420 DOI: 10.2174/1874312901409010057] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2015] [Revised: 05/27/2015] [Accepted: 06/09/2015] [Indexed: 12/02/2022] Open
Affiliation(s)
- Renata Siciliani Scalco
- Department of Neurology, Pontifical Catholic University of Rio Grande do Sul (PUCRS), Porto Alegre, Brazil ; MRC Centre for Neuromuscular Diseases and Division of Neuropathology, Institute of Neurology, University College London, London, UK
| | - Stefen Brady
- MRC Centre for Neuromuscular Diseases and Division of Neuropathology, Institute of Neurology, University College London, London, UK
| | - Jefferson Becker
- Department of Neurology, Pontifical Catholic University of Rio Grande do Sul (PUCRS), Porto Alegre, Brazil
| | - Irenio Gomes
- Department of Neurology, Pontifical Catholic University of Rio Grande do Sul (PUCRS), Porto Alegre, Brazil
| | - Janice L Holton
- MRC Centre for Neuromuscular Diseases and Division of Neuropathology, Institute of Neurology, University College London, London, UK
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Jakobiec FA, Rashid A, Lane KA, Kazim M. Granulomatous dacryoadenitis in regional enteritis (crohn disease). Am J Ophthalmol 2014; 158:838-844.e1. [PMID: 25036879 DOI: 10.1016/j.ajo.2014.07.011] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2014] [Revised: 07/10/2014] [Accepted: 07/11/2014] [Indexed: 12/25/2022]
Abstract
PURPOSE To evaluate the clinical and immunopathologic features of 2 patients with bilateral dacryoadenitis associated with regional enteritis. DESIGN Retrospective, clinicopathologic study. METHODS Clinical records, photographs, and imaging studies were reviewed and microscopic sections of lacrimal gland biopsy samples were critically re-evaluated. The microscopic slides were stained with hematoxylin and eosin, special stains for organisms, and a range of immunohistochemical biomarkers, including CD3, CD4, CD5, CD8, CD20, CD68, CD138, CD1a, and immunoglobulins Ig G, IgG4, and IgA. RESULTS Both patients were young women with a well-established diagnosis of regional enteritis. Histopathologic examination of biopsy samples disclosed moderate intraparenchymal fibrosis and lymphoplasmacytic infiltrates without lymphoid follicles. Small to medium intraparenchymal, noncaseating granulomas lacking multinucleated giant cells and, in 1 patient, CD68-positive and CD1a-negative palisading granulomas in widened interlobular fibrous septa were detected. Vasculitis and IgG4 plasma cells were not observed. Additional immunohistochemical studies revealed that CD8 T lymphocytes (suppressor or cytotoxic subset) predominated over CD4-positive T lymphocytes (helper cells) surrounding the necrobiotic foci and were intermixed with the CD68-positive histiocytes in the absence of CD20 B lymphocytes. Special stains for organisms demonstrated negative results. CONCLUSIONS Dacryoadenitis is the rarest form of ocular adnexal involvement in regional enteritis, which affects the orbit far more frequently than ulcerative colitis. It is a granulomatous process with the possibility of palisading necrobiotic foci. In contrast, ulcerative colitis causes an interstitial lymphocytic and nongranulomatous myositis. Sarcoidosis, Wegener granulomatosis, and pseudorheumatoid nodules must be ruled out. Treatment options entail a wide variety of agents with selection based on empirical considerations and tailored to the patient's symptoms.
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Affiliation(s)
- Frederick A Jakobiec
- David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts.
| | - Alia Rashid
- David G. Cogan Laboratory of Ophthalmic Pathology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts
| | | | - Michael Kazim
- Edward S. Harkness Eye Institute of the New York Hospital Presbyterian Medical Center, and Columbia University, New York, New York
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15
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Bodoki L, Nagy-Vincze M, Griger Z, Péter A, Dankó K. [Anti-NXP2-positive dermatomyositis associated with ulcerative colitis and celiac disease]. Orv Hetil 2014; 155:1033-8. [PMID: 24954145 DOI: 10.1556/oh.2014.29940] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
The authors discuss a rare case of a 25-year-old female patient having dermatomyositis associated with celiac disease and ulcerative colitis. The idiopathic inflammatory myopathies are systemic, chronic, immune-mediated diseases characterized by proximal, symmetrical muscle weakness. Many examples from the literature refer that celiac disease occurs more often in patients with myositis than in the general population, but its association with ulcerative colitis is a real rarity in the international literature.
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Affiliation(s)
- Levente Bodoki
- Debreceni Egyetem, Általános Orvostudományi Kar Belgyógyászati Intézet, Klinikai Immunológia Tanszék Debrecen Móricz Zsigmond krt. 22. 4032
| | - Melinda Nagy-Vincze
- Debreceni Egyetem, Általános Orvostudományi Kar Belgyógyászati Intézet, Klinikai Immunológia Tanszék Debrecen Móricz Zsigmond krt. 22. 4032
| | - Zoltán Griger
- Debreceni Egyetem, Általános Orvostudományi Kar Belgyógyászati Intézet, Klinikai Immunológia Tanszék Debrecen Móricz Zsigmond krt. 22. 4032
| | - Andrea Péter
- Debreceni Egyetem, Általános Orvostudományi Kar Kardiológiai Klinika Debrecen
| | - Katalin Dankó
- Debreceni Egyetem, Általános Orvostudományi Kar Belgyógyászati Intézet, Klinikai Immunológia Tanszék Debrecen Móricz Zsigmond krt. 22. 4032
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16
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Prieto-González S, Grau JM. Diagnosis and classification of granulomatous myositis. Autoimmun Rev 2014; 13:372-4. [PMID: 24424169 DOI: 10.1016/j.autrev.2014.01.017] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/13/2013] [Indexed: 11/30/2022]
Abstract
The term granulomatous myositis is applied to a myopathic syndrome associated with non-specific epithelioid granulomas in striated muscle. This rare entity is most frequently related to sarcoidosis, but other uncommon causes have been reported, including an idiopathic form only after systemic disorders known to cause similar myopathological abnormalities have been excluded. Symmetrical proximal or distal muscle weakness is the rule in the clinical presentation, sometimes associated with dysphagia. Although the clinical profile together with electromyography (EMG) studies may be useful, definite diagnosis requires pathological examination. Systemic glucocorticoids are the treatment of choice, but the clinical outcome is not always satisfactory.
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Affiliation(s)
- S Prieto-González
- Muscle Research Unit, Service of Internal Medicine, Institut Clinic de Medicina i Dermatologia, Hospital Clínic, Universitat de Barcelona, Spain.
| | - J M Grau
- Muscle Research Unit, Service of Internal Medicine, Institut Clinic de Medicina i Dermatologia, Hospital Clínic, Universitat de Barcelona, Spain.
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17
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Jasim S, Shaibani A. Nonsarcoid granulomatous myopathy: two cases and a review of literature. Int J Neurosci 2013; 123:516-20. [PMID: 23311755 DOI: 10.3109/00207454.2013.765871] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Granulomatous myopathy is an uncommon skeletal muscles disorder. It can develop in association with other granuloma-forming diseases and is then considered a secondary myopathy or, less frequently, a primary disorder for which no etiology is identified. Studies of granulomatous myopathies have focused on examining the differences between primary and secondary diseases. Herein, we describe two cases of nonsarcoid granulomatous myopathies, for which diagnostic work-up did not reveal an underlying granuloma-causing pathology. The patients exhibited similar histopathological characteristics in skeletal muscle biopsies. However, they had different clinical presentations and therapeutic responses. Specifically, one patient had distal muscle weakness with a poor response to immunosuppressive treatment, whereas the other had a more proximal muscle weakness distribution and a very good response to treatment with corticosteroids and azathioprine, resulting in remission. More studies are warranted to further characterize the clinical course and effect of different treatment modalities on nonsarcoid granulomatous myopathy.
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Affiliation(s)
- Sina Jasim
- Saint Louis University, Saint Louis, MO, USA
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18
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Abstract
Inflammatory bowel diseases, such as Crohn's disease, ulcerative colitis, autoantibody driven celiac disease and infectious Whipple's disease can all be associated with neurological symptoms. The neurological manifestation may occur even before the gastrointestinal symptoms or the enteropathic symptoms can even be absent as in celiac disease. These diseases can be caused by malresorption and lack of vitamins due to enteral inflammation as well as (auto-)immunological mechanisms and drug-associated side effects. Thus, inflammatory bowel diseases have to be considered in the differential diagnosis. In this review the most common neurological manifestations of these diseases will be described as well as the diagnostic approach.
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19
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Zois CD, Katsanos KH, Kosmidou M, Tsianos EV. Neurologic manifestations in inflammatory bowel diseases: current knowledge and novel insights. J Crohns Colitis 2010; 4:115-24. [PMID: 21122494 DOI: 10.1016/j.crohns.2009.10.005] [Citation(s) in RCA: 59] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/27/2009] [Revised: 10/26/2009] [Accepted: 10/27/2009] [Indexed: 02/08/2023]
Abstract
BACKGROUND Crohn's disease (CD) and ulcerative colitis (UC), widely known as inflammatory bowel diseases (IBD), are thought to result from an inappropriate activation of the mucosal immune system driven by intestinal bacterial flora. METHODS Although the extraintestinal manifestations of IBD are well documented, the association of IBD with neurologic and neuromuscular involvement is rare and often controversial, with sporadic and conflicting data on its prevalence and spectrum. In addition, a serious number of the latter manifestations may become life-threatening, playing a very important role in disease morbidity. To define the pattern of neurologic involvement in IBD, the most important manifestations in these patients have been reviewed, exploring also their clinical significance. RESULTS There is evidence that UC and CD can manifest both in the PNS and CNS. Thrombotic complications are common in IBD patients, but cerebral vascular involvement is rare. CONCLUSIONS Neurologic manifestations in IBD patients are more common than previously estimated and may follow a different pattern of involvement in CD and UC. Small numbers of patients currently preclude a better characterization of the clinical spectrum and a better understanding of pathogenesis.
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Affiliation(s)
- Christos D Zois
- 1st Department of Internal Medicine, Hepato-Gastroenterology Unit, Medical School, University of Ioannina, 451 10 Ioannina, Greece
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20
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Abstract
In recent years, there has been increasing recognition of the presence of gastrointestinal (GI) dysfunction in the setting of neurologic diseases. Parkinson's disease is a particularly well-known example, but GI dysfunction also may occur in multiple sclerosis, stroke, and in various myopathic and peripheral neuropathic processes. There is much less awareness, however, that primary GI diseases may also display neurologic dysfunction as part of their clinical picture. This article focuses on some of those disease processes. Illnesses primarily targeting the GI tract are addressed and examples of primary esophageal, gastric, and intestinal disease processes are described.
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Affiliation(s)
- Ronald F Pfeiffer
- Department of Neurology, University of Tennessee Health Science Center, 855 Monroe Avenue, Memphis, TN 38163, USA.
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21
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Abstract
BACKGROUND The inflammatory myopathies are a group of acquired diseases characterized by a proximal myopathy caused by an inflammatory infiltrate of the skeletal muscle. The three major diseases are dermatomyositis, polymyositis and inclusion body myositis. AIMS To review the gastrointestinal manifestations of myositis. METHODS Over 110 articles in the English literature were reviewed. RESULTS Dysphagia to solids and liquids occurs in patients with myositis. The pharyngo-oesophageal muscle tone is lost and therefore patients develop nasal speech, hoarseness, nasal regurgitation and aspiration pneumonia. There is tongue weakness, flaccid vocal cords, poor palatal motion and pooling of secretions in the distended hypopharynx. Proximal oesophageal skeletal muscle dysfunction is demonstrated by manometry with low amplitude/absent pharyngeal contractions and decreased upper oesophageal sphincter pressures. Patients exhibit markedly elevated creatine kinase and lactate dehydrogenase levels consistent with muscle injury. Myositis can be associated with inflammatory bowel disease, coeliac disease and interferon treatment of hepatitis C. Corticosteroids and other immunosuppressive drugs comprise the mainstay of treatment. Inclusion body myositis responds poorly to these agents and therefore a myotomy is usually indicated. CONCLUSION Myositis mainly involves the skeletal muscles in the upper oesophagus with dysphagia, along with proximal muscle weakness.
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Affiliation(s)
- E C Ebert
- Department of Medicine, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
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22
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Nava A, Orozco-Barocio G. Abordaje en el diagnóstico diferencial de las miopatías inflamatorias. ACTA ACUST UNITED AC 2009; 5 Suppl 3:32-4. [DOI: 10.1016/j.reuma.2009.07.003] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2009] [Revised: 07/02/2009] [Accepted: 07/05/2009] [Indexed: 11/24/2022]
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23
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Abstract
The inflammatory myopathies are a group of acquired diseases, characterized by an inflammatory infiltrate of the skeletal muscle. On the basis of clinical, immuno-pathological and demographic features, three major diseases can be identified: dermatomyositis (DM); polymyositis (PM); and inclusion body myositis (IBM). New diagnostic criteria have recently been introduced, which are crucial for discriminating between the three different subsets of inflammatory myopathies and for excluding other disorders. DM is a complement-mediated microangiopathy affecting skin and muscle. PM and IBM are T cell-mediated disorders, where CD8-positive cytotoxic T cells invade muscle fibres expressing MHC class I antigens, thus leading to fibre necrosis. In IBM, vacuolar formation with amyloid deposits are also present. This article summarizes the main clinical, laboratory, electrophysiological, immunological and histologic features as well as the therapeutic options of the inflammatory myopathies.
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Affiliation(s)
- C Briani
- University of Padova, Department of Neurosciences, Padova, Italy.
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24
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Shimoyama T, Tamura Y, Sakamoto T, Inoue K. Immune-mediated myositis in Crohn's disease. Muscle Nerve 2009; 39:101-5. [DOI: 10.1002/mus.21164] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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25
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Collins S, McKelvie P, Dennett X, Byrne E. Idiopathic granulomatous myositis: does the clinical spectrum include polymyalgia rheumatica? J Clin Neurosci 2008; 6:255-9. [PMID: 18639165 DOI: 10.1016/s0967-5868(99)90517-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/1997] [Accepted: 12/02/1997] [Indexed: 11/17/2022]
Abstract
Granulomatous inflammation restricted to muscle is an uncommon cause of myopathic syndromes. Two patients were diagnosed with idiopathic granulomatous polymyositis after appropriate investigations failed to reveal a systemic or alternative explanation for the granulomas seen in their muscle biopsy. One patient presented with a syndrome indistinguishable from polymyalgia rheumatica but both patients manifested disabling myalgias which were strikingly corticosteroid responsive. The two cases underscore the potential importance of muscle biopsy in polymyalgic states, the non-specificity of polymyalgia rheumatica as a syndrome which can be simulated by other disorders including granulomatous myositis, and the potential corticosteroid responsiveness of prominent, functionally limiting myalgias which can be seen in this disorder. Some controversy continues regarding the correct nosology of this disease, largely perpetuated by an awareness of the inherent limitations of current non-invasive evaluation techniques to confirm occult granulomatous involvement of non-myogenous organs.
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Affiliation(s)
- S Collins
- Department of Clinical Neuroscience, St Vincent's Hospital, Victoria Parade, Fitzroy, Victoria, Australia 3065
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26
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Mangone M, Spagnolo A, Capurso G, Marignani M, Panzuto F, Angeletti S, Ruggeri M, Menè P, Delle Fave G. Rhabdomyolysis due to severe hypokaliemia in a Crohn's disease patient after budesonide treatment. Dig Liver Dis 2007; 39:776-779. [PMID: 17049941 DOI: 10.1016/j.dld.2006.09.003] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/31/2006] [Revised: 08/29/2006] [Accepted: 09/05/2006] [Indexed: 12/11/2022]
Abstract
Patients with Crohn's disease may experience several non-digestive complications, including muscle disorders. Rabdomyolysis has rarely been reported in patients with inflammatory bowel disease, however a number of factors may cause muscular damage in this setting. We report the case of a young woman with Crohn's disease who developed a severe, symptomatic skeletal muscle damage associated with severe hypokaliemia. Reversal of the potassium levels to normal ranges led to clinical resolution. The possible causes that might have lead to hypokalemia development and subsequent rhabdomyolysis are discussed with special emphasis for the potential causative role of medical treatment, especially budesonide for which similar side effects have been previously reported. Physicians should be aware that hypokalemia is possible in the setting of Crohn's disease and muscle damage can present as a complication.
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Affiliation(s)
- M Mangone
- Digestive and Liver Disease Unit, II Medical School, S. Andrea Hospital, University La Sapienza, Rome, Italy
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27
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Qureshi JA, Staugaitis SM, Calabrese LH. Neutrophilic myositis: an extra-intestinal manifestation of ulcerative colitis. J Clin Rheumatol 2007; 8:85-8. [PMID: 17041328 DOI: 10.1097/00124743-200204000-00004] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
Myositis of the skeletal muscle is rarely seen as an extraintestinal manifestation of inflammatory bowel disease. We report a case of a 36-year-old man with ulcerative colitis who developed 3 episodes of steroid responsive myositis in a 4-month period. He presented with a blistering rash followed by pain and massive swelling in the right shoulder and lower extremities, with marked elevation in muscle enzymes. Diagnosis of myositis was made after a muscle biopsy, which showed neutrophilic infiltrate in the deep subcutaneous tissue and skeletal muscle. Review of the literature indicates only a few cases of myositis associated with ulcerative colitis, most of them during an acute exacerbation of the disease. However, the present patient had his first episode when the ulcerative colitis was in remission. Myositis in ulcerative colitis may present atypically, in particular mimicking pyomyositis. Clinicians should be aware of this unusual steroid-sensitive complication.
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Affiliation(s)
- Jazibeh A Qureshi
- Department of Rheumatic and Immunologic Disease, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
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28
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Le Roux K, Streichenberger N, Vial C, Petiot P, Feasson L, Bouhour F, Ninet J, Lachenal F, Broussolle C, Sève P. Granulomatous myositis: a clinical study of thirteen cases. Muscle Nerve 2007; 35:171-7. [PMID: 17068767 DOI: 10.1002/mus.20683] [Citation(s) in RCA: 56] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Granulomatous myositis (GM) is a rare condition that has generally been described in association with sarcoidosis. In the absence of sarcoidosis or other underlying disease, a diagnosis of isolated GM is considered. Only one study has focused on the clinical difference between isolated GM and sarcoid myopathy (SM). We report 13 cases of symptomatic GM; 8 had sarcoidosis. All patients with sarcoidosis had predominantly proximal, symmetrical lower-limb weakness, and 3 subsequently developed upper-limb or distal involvement. Three of the five patients with isolated GM had predominantly distal muscle involvement, and two had dysphagia. Corticosteroid treatment was followed by prolonged improvement in only one patient with sarcoidosis. One patient had acute sarcoid myositis and benefited from methotrexate; other immunosuppressants and etanercept proved ineffective in chronic sarcoid myopathy. Three of the five patients with isolated GM responded to corticosteroid treatment. When last examined, three patients with sarcoidosis had severe disability, whereas patients with isolated GM showed milder weakness. Thus, SM was frequently associated with severe disability and rarely improved after corticosteroid treatment, whereas most patients with isolated GM improved.
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Affiliation(s)
- Karine Le Roux
- Hospices Civils de Lyon, Université Claude Bernard Lyon I, Lyon, France
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29
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Paoluzi OA, Crispino P, Rivera M, Iacopini F, Palladini D, Consolazio A, Paoluzi P. Skeletal muscle disorders associated with inflammatory bowel diseases: occurrence of myositis in a patient with ulcerative colitis and Hashimoto's thyroiditis--case report and review of the literature. Int J Colorectal Dis 2006; 21:473-7. [PMID: 16205931 DOI: 10.1007/s00384-005-0035-z] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/02/2005] [Indexed: 02/04/2023]
Abstract
Ulcerative colitis (UC) and Crohn's disease are inflammatory bowel diseases often associated with extra-intestinal manifestations. Of these, neutrophilic dermatoses and arthropathies are the more frequently observed, while the occurrence of striated muscle disorders, namely, myositis, has been very rarely diagnosed in these kinds of patients. The coexistence of immuno-mediated diseases in patients with inflammatory bowel diseases and myositis suggests a common aetiopathogenetic mechanism underlying these conditions. The present report refers to a rare case of a 51-year-old female with UC and Hashimoto's thyroiditis who developed myositis.
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Affiliation(s)
- Omero Alessandro Paoluzi
- U.O. di Gastroenterologia ed Endoscopia Digestiva, Poliambulatorio Don Bosco ASL RMB, Via Antistio 15, 00174, Rome, Italy.
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30
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Leibovitch I, Galanopoulos A, Selva D. Suppurative granulomatous myositis of an extra-ocular muscle in Crohn's disease. Am J Gastroenterol 2005; 100:2136-7. [PMID: 16128967 DOI: 10.1111/j.1572-0241.2005.50395_10.x] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
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31
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Affiliation(s)
- R M Hoffmann
- Ev. Krankenhaus Kalk, Akad. Lehrkrankenhaus der Universität zu Köln, Buchforststr. 2 D-51103 Köln
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32
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Abstract
Inflammatory bowel disease includes Crohn's disease and ulcerative colitis, and is characterized by chronic inflammation of the intestines. The advances in understanding of the inflammatory process have resulted in improved treatment of inflammatory bowel disease. The systemic complications of inflammatory bowel disease involve many organs, eyes included. The ophthalmic complications are usually of inflammatory origin. Some of these complications, like scleritis, may reflect overall disease activity. Treatment of intestinal inflammation-either medical or surgical-usually helps resolution of ophthalmic complications. This review describes recent developments in the diagnosis and management of the inflammatory bowel disease and its ophthalmic complications.
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Affiliation(s)
- Faruque D Ghanchi
- Bradford Teaching Hospitals, Royal Infirmary, Bradford, West Yorkshire, UK
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33
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Mastaglia FL, Garlepp MJ, Phillips BA, Zilko PJ. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve 2003; 27:407-25. [PMID: 12661042 DOI: 10.1002/mus.10313] [Citation(s) in RCA: 147] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
Abstract
The three major forms of immune-mediated inflammatory myopathy are dermatomyositis (DM), polymyositis (PM), and inclusion-body myositis (IBM). They each have distinctive clinical and histopathologic features that allow the clinician to reach a specific diagnosis in most cases. Magnetic resonance imaging is sometimes helpful, particularly if the diagnosis of IBM is suspected but has not been formally evaluated. Myositis-specific antibodies are not helpful diagnostically but may be of prognostic value; most antibodies have low sensitivity. Muscle biopsy is mandatory to confirm the diagnosis of an inflammatory myopathy and to allow unusual varieties such as eosinophilic, granulomatous, and parasitic myositis, and macrophagic myofasciitis, to be recognized. The treatment of the inflammatory myopathies remains largely empirical and relies upon the use of corticosteroids, immunosuppressive agents, and intravenous immunoglobulin, all of which have nonselective effects on the immune system. Further controlled clinical trials are required to evaluate the relative efficacy of the available therapeutic modalities particularly in combinations, and of newer immunosuppressive agents (mycophenolate mofetil and tacrolimus) and cytokine-based therapies for the treatment of resistant cases of DM, PM, and IBM. Improved understanding of the molecular mechanisms of muscle injury in the inflammatory myopathies should lead to the development of more specific forms of immunotherapy for these conditions.
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Affiliation(s)
- Frank L Mastaglia
- Centre for Neuromuscular and Neurological Disorders, University of Western Australia, Queen Elizabeth II Medical Centre, Nedlands, Australia.
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34
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Tateyama M, Fujihara K, Ishii N, Sugamura K, Onodera Y, Itoyama Y. Expression of OX40 in muscles of polymyositis and granulomatous myopathy. J Neurol Sci 2002; 194:29-34. [PMID: 11809163 DOI: 10.1016/s0022-510x(01)00668-2] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
OX40 is selectively expressed on activated autoreactive memory T cells and these OX40+ lymphocytes may play a crucial role in autoimmune diseases. To determine whether OX40+ lymphocytes are involved in the pathomechanism of human inflammatory muscle diseases, we immunohistochemically examined the distribution of OX40+ cells in muscles from patients with polymyositis and granulomatous myopathy, and compared with that of cells bearing other activation markers, such as IL-2 receptor (IL-2R) and HLA-DR. In polymyositis, OX40+ mononuclear cells were found predominantly in the perivascular sites and to a lesser degree in the endomysium. Scanty IL-2R+ mononuclear cells were located only in the endomysium and HLA-DR was expressed on half of the mononuclear cells distributed diffusely in the perivascular sites and in the endomysium. Mononuclear cell infiltration in the perivascular sites was greater in the muscles in which OX40+ cells were present in the perivascular sites than in those without OX40+ cells in the perivascular sites (p<0.05). In granulomatous myopathy, OX40+ cells were detected in the centers of the granulomas. In contrast, IL-2R+ cells were present at the periphery of the granulomas and HLA-DR was detected on mononuclear cells throughout the granulomas. OX40+ mononuclear cells with specific distributions in muscles may be involved in the pathomechanism of polymyositis and granulomatous myopathy, and can be a candidate molecule of selective immunotherapy in these diseases.
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Affiliation(s)
- Maki Tateyama
- Department of Neurology, Tohoku University School of Medicine, 1-1, Seiryo-machi, Aoba-ku, 980-8574, Sendai, Japan.
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35
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Abstract
Granulomatous myopathies are rare. Most cases are associated with sarcoidosis. We report a case of granulomatous myopathy associated with primary biliary cirrhosis, pancytopenia, and thymoma. The literature in regard to granulomatous myopathy and its pathogenesis is reviewed. Intermittent pulsed intravenous methylprednisolone may be useful as maintenance therapy for granulomatous myopathy and other neuromuscular syndromes for patients intolerant of oral corticosteroids.
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Affiliation(s)
- D N Herrmann
- Department of Neurology, University of Rochester, SMH 601 Elmwood Ave., Box 673, Rochester, New York 14642, USA.
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36
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Bailey M, Chapin W, Licht H, Reynolds JC. The effects of vasculitis on the gastrointestinal tract and liver. Gastroenterol Clin North Am 1998; 27:747-82, v-vi. [PMID: 9890113 DOI: 10.1016/s0889-8553(05)70032-7] [Citation(s) in RCA: 51] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Vasculitis can affect every organ of the digestive system. In many cases, it may first present with gastrointestinal symptoms. In several forms of vasculitis, including Churg Strauss syndrome, Henoch-Schönlein purpura, and lupus, the majority of patients have gastrointestinal involvement. The astute gastroenterologist should consider vasculitic causes of the symptoms seen in many patients. Making the correct diagnosis requires a thorough understanding of the potential role of vasculitis in causing these symptoms and the appropriate path to making a diagnosis. This article reviews the variety of manifestations of vasculitis on the digestive system, and emphasizes diagnosis and clinical manifestations.
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Affiliation(s)
- M Bailey
- Department of Medicine, Allegheny University of the Health Sciences-Medical College of Pennsylvania, USA
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37
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Affiliation(s)
- A K Akobeng
- Department of Child Health, University of Manchester, United Kingdom
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38
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Alric L, Laroche M, Faucheux JM, Bonnet E, Massip P, Duffaut M. [Systemic manifestations of hemorrhagic rectocolitis: apropos of a case of hemorrhagic rectocolitis associated with multiple sclerosis]. Rev Med Interne 1997; 18:132-7. [PMID: 9092032 DOI: 10.1016/s0248-8663(97)84679-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Extra-intestinal manifestations of inflammatory bowel diseases are frequent and numerous. We report a case of ulcerative colitis associated with multiple sclerosis, ankylosing spondylitis and pyoderma gangrenosum. This observation shows the rare association of ulcerative colitis-multiple sclerosis, and extra-intestinal manifestations of inflammatory colitis are discussed.
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Affiliation(s)
- L Alric
- Service de médecine interne, CHU Rangueil, Toulouse, France
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39
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Seibold F, Klein R, Jakob F. Polymyositis, alopecia universalis, and primary sclerosing cholangitis in a patient with Crohn's disease. J Clin Gastroenterol 1996; 23:121-4. [PMID: 8877639 DOI: 10.1097/00004836-199609000-00011] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
We report a 36-year-old man with Crohn's disease, primary sclerosing cholangitis, and alopecia universalis. Six years after the onset of intestinal disease, the patient developed severe muscular pain and weakness of the neck. Muscle biopsy revealed myositis. Immunosuppressive treatment led to a significant improvement of muscular symptoms. Myositis in inflammatory bowel disease appears to be an important differential diagnosis in corticoid myopathy. Both alopecia and polymyositis are rarely associated with inflammatory bowel disease; thus, they have to be discussed as extraintestinal manifestations.
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Affiliation(s)
- F Seibold
- Medizinische Poliklinik and Pathologisches Institut (Neuropathologie), University of Wuerzburg, Germany
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40
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Abstract
In six cases of granulomatous myopathy immunohistological analysis showed a typical pattern with macrophages and T4 cells diffusely distributed throughout the cellular exudate. T8 lymphocytes were interspersed irregularly within the granulomatous cellular infiltrate early in granuloma maturation and in later stages predominantly confined to a lymphocytic mantle surrounding the granulomas. The cellular infiltrate displayed numerous activated HLA-DR and interleukin-2 receptor positive cells including cell proliferation. Increased connective tissue showed strong immunoreactivity for fibronectin and hyaluronate. Muscle fibres were negative for MHC class I molecules. Atrophic muscle fibres expressed desmin, a marker to distinguish desmin positive myogenic giant cells from desmin negative Langhans giant cells.
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Affiliation(s)
- D S Tews
- Division of Neuropathy, University of Mainz, Germany
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41
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Abstract
An elderly woman with chronic ulcerative colitis who developed proximal muscle weakness, increased serum creatine phosphokinase activity, and histological and electromyographic abnormalities characteristic of polymyositis is described. Treatment with corticosteroids and 5-acetylsalicylic acid was followed by a remission in bowel symptoms, improvement in muscle power, and reversal of electromyographic changes. An autoimmune link between the two disorders seems likely.
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Affiliation(s)
- S Chugh
- Department of Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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42
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Affiliation(s)
- D K Podolsky
- Gastrointestinal Unit, Center for the Study of Inflammatory Bowel Disease, Massachusetts General Hospital, Boston
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43
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Weiner SR, Clarke J, Taggart NA, Utsinger PD. Rheumatic manifestations of inflammatory bowel disease. Semin Arthritis Rheum 1991. [DOI: 10.1016/0049-0172(91)90011-n] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
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44
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Abstract
A wide range of conditions can mimic polymyositis. Thus, diagnosing this condition can be a challenge. Although no single criterion is diagnostic of polymyositis, the following criteria have been proposed and widely used: (1) symmetric proximal muscle weakness; (2) characteristic violaceous rash on the hands, elbows, and knees; (3) increased muscle enzymes in the serum; (4) characteristic electromyographic findings (insertional activity, fibrillation potentials, motor unit potentials of increased frequency and decreased duration, and normal conduction velocity in nerves); and (5) muscle biopsy specimen with characteristic inflammatory and myopathic changes. Although polymyositis primarily involves muscle, up to 20% of patients may have extramuscular problems. The main treatment for polymyositis is high-dose corticosteroids. In corticosteroid-resistant patients, methotrexate is often effective. In this report, case histories are presented to highlight the usefulness and the limitations of the common diagnostic criteria for polymyositis.
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Affiliation(s)
- T W Bunch
- Division of Rheumatology, Mayo Clinic, Rochester, MN 55905
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45
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Abstract
Granulomatous muscle disease is most commonly seen in sarcoidosis, but may be seen in association with a wide variety of other disorders or in isolation. Patients with granulomatous myositis usually present with slowly progressive muscle pain and weakness affecting mainly proximal muscles. There are, however, a few reports of granulomatous muscle disease presenting with flexion contractures of the limbs. Two further patients with granulomatous muscle disease and flexion contractures of the limbs, but with no evidence of systemic granulomatous disease, is presented. It is suggested that such patients represent a separate clinical entity that is distinct from idiopathic granulomatous myositis presenting with muscle pain and weakness. The association of contracturing granulomatous myositis with a long-standing vasculitis in one patient suggests that the two conditions may be related.
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Ahrenstedt O, Knutson L, Nilsson B, Nilsson-Ekdahl K, Odlind B, Hällgren R. Enhanced local production of complement components in the small intestines of patients with Crohn's disease. N Engl J Med 1990; 322:1345-9. [PMID: 2325733 DOI: 10.1056/nejm199005103221903] [Citation(s) in RCA: 101] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
There is evidence that complement components may be formed locally in inflammatory lesions containing monocytes and macrophages. To investigate the role of complement in Crohn's disease we measured jejunal-fluid concentrations of the complement components C4, C3, and factor B by perfusion of a closed segment of the jejunum in 22 patients with Crohn's disease thought to be limited to the terminal ileum. The mean (+/- SEM) jejunal-fluid C4 concentration was 2.0 +/- 0.3 mg per liter, significantly higher than the mean level in 35 healthy controls (0.7 +/- 0.1 mg per liter; P less than 0.001). The mean C3 concentration was 1.0 +/- 0.1 mg per liter in the patients and 0.7 +/- 0.1 mg per liter in the controls (P less than 0.05). The factor B levels were similar in the two groups. Calculated rates of intestinal secretion of these components showed differences of the same magnitude. Leakage of protein from plasma was not increased. The jejunal-fluid:serum ratios of these complement proteins indicated that their appearance in the lumen of the jejunum was due to at least in part to local mucosal synthesis. The increased jejunal secretion of C4, but not C3 or factor B, paralleled the clinical activity of Crohn's disease. Values were normal in first-degree relatives of the patients (n = 13), patients with celiac disease (n = 8), and patients with ulcerative colitis (n = 4). We conclude that increased secretion of complement by clinically unaffected jejunal tissue in patients with Crohn's disease reflects the systemic nature of this disorder and may be due to the stimulated synthesis of complement by activated intestinal monocytes and macrophages.
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Affiliation(s)
- O Ahrenstedt
- Department of Surgery, University Hospital, Uppsala, Sweden
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Chiodini RJ. Crohn's disease and the mycobacterioses: a review and comparison of two disease entities. Clin Microbiol Rev 1989; 2:90-117. [PMID: 2644025 PMCID: PMC358101 DOI: 10.1128/cmr.2.1.90] [Citation(s) in RCA: 258] [Impact Index Per Article: 7.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023] Open
Abstract
Crohn's disease is a chronic granulomatous ileocolitis, of unknown etiology, which generally affects the patient during the prime of life. Medical treatment is supportive at best, and patients afflicted with this disorder generally live with chronic pain, in and out of hospitals, throughout their lives. The disease bears the name of the investigator who convincingly distinguished this disease from intestinal tuberculosis in 1932. This distinction was not universally accepted, and the notion of a mycobacterial etiology has never been fully dismissed. Nevertheless, it was 46 years after the distinction of Crohn's disease and intestinal tuberculosis before research attempting to reassociate mycobacteria and Crohn's disease was published. Recently, there has been a surge of interest in the possible association of mycobacteria and Crohn's disease due largely to the isolation of genetically identical pathogenic Mycobacterium paratuberculosis from several patients with Crohn's disease in the United States, the Netherlands, Australia, and France. These pathogenic organisms have been isolated from only a few patients, and direct evidence for their involvement in the disease process is not clear; however, M. paratuberculosis is an obligate intracellular organism and strict pathogen, which strongly suggests some etiologic role. Immunologic evidence of a mycobacterial etiology, as assessed by humoral immune determinations, has been conflicting, but evaluation of the more relevant cellular immunity has not been performed. Data from histochemical searches for mycobacteria in Crohn's disease tissues have been equally conflicting, with acid-fast bacilli detected in 0 to 35% of patients. Animal model studies have demonstrated the pathogenic potential of isolates as well as elucidated the complexity of mycobacterial-intestinal interactions. Treatment of Crohn's disease patients with antimycobacterial agent has not been fully assessed, although case reports suggest efficacy. The similarities in the pathology, epidemiology, and chemotherapy of Crohn's disease and the mycobacterioses are discussed. The issue is fraught with controversy, and the data generated on the association of mycobacteria and Crohn's disease are in their infantile stages so that a general conclusion on the legitimacy of this association cannot be made. While no firm evidence clearly implicates mycobacteria as an etiologic agent of Crohn's disease, the notion is supported by suggestive and circumstantial evidence and a remarkable similarity of Crohn's disease to known mycobacterial diseases.
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Affiliation(s)
- R J Chiodini
- Department of Medicine, Rhode Island Hospital, Providence
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Bookman AA, Gould MI, Barrowman JA, Chittal SM. Periosteal new bone formation and disseminated granulomatosis in a patient with Crohn's disease. Am J Med 1988; 84:330-3. [PMID: 3407658 DOI: 10.1016/0002-9343(88)90435-4] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Abstract
In a 20-year-old man, a proliferative periosteal new bone growth developed over the left forearm. Crohn's disease had been diagnosed the year before. Bone biopsy demonstrated granuloma formation. Biopsy specimens of skin lesions demonstrated granulomas as well. Bowel studies indicated active small intestinal inflammation with fistula formation. Despite the superficial resemblance to hypertrophic osteoarthropathy, it is believed that this case represents Crohn's disease with disseminated granulomatosis involving skin and periosteum.
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Affiliation(s)
- A A Bookman
- Division of Rheumatology, Toronto General Hospital, University of Toronto, Ontario, Canada
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