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Symptomatic muscular sarcoidosis: Lessons from a nationwide multicenter study
Fleur Cohen Aubart1, Salam Abbara1, Thierry Maisonobe1
1AP-HP (F.C.A., S.A, J.H., A.M., M.P., Z.A), Service de Médecine Interne 2, Institut e3m, Hôpital de la Pitié-Salpêtrière, Centre National de Référence Maladies Systémiques Rares, Lupus, Syndrome des anticorps antiphospholipides; Université Paris VI (F.C.A., J.H., O.B.), UPMC, Sorbonnes Universités; AP-HP (T.M.), Département de neurophysiologie et de neuropathologie, Hôpital de la Pitié-Salpêtrière, Paris; Service de Pneumologie (V.C.), Centre des maladies pulmonaires rares, Lyon; AP-HP (T.P.), Service de Médecine Interne, Hôpital Bichat; AP-HP (L.G., B.H., O.B., Z.A.), Service de Médecine Interne et immunologie clinique, Hôpital de la Pitié-Salpêtrière, Paris; AP-HP (M.S.), Service de Médecine Nucléaire, Hôpital Avicenne, Bobigny; Service de Médecine Interne (P.M.), CHU Bordeaux, Bordeaux; AP-HP (H.N., D.V.), Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
This study identified four distinct patterns of granulomatous myositis in muscular sarcoidosis patients. These patterns vary in clinical presentation, severity, and treatment response, impacting the overall sarcoidosis phenotype.
Area of Science:
- Rheumatology
- Neurology
- Pathology
Background:
- Muscular sarcoidosis is a rare manifestation of sarcoidosis.
- Understanding its clinicopathologic features is crucial for diagnosis and management.
Purpose of the Study:
- To characterize the clinicopathologic features of muscular sarcoidosis.
- To identify distinct sarcoidosis phenotypes associated with muscular involvement.
Main Methods:
- Nationwide multicenter study.
- Inclusion criteria: histologically proven sarcoidosis with symptomatic muscular involvement.
- Confirmation via biological, imaging, or histologic examinations.
Main Results:
- Forty-eight patients studied; median age at onset 45 years.
- Identified four patterns: nodular (27%), smoldering (29%), myopathic (35%), and combined (10%).
- All cases showed multivisceral involvement (median 3 organs) and required prolonged treatment.
Conclusions:
- Four distinct patterns of granulomatous myositis identified in muscular sarcoidosis.
- These patterns differ in phenotypes and clinical course.
- Myopathic and neuromuscular patterns were associated with increased severity.
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