Granulomatosis-associated myositis

Dieudonné, Yannick; Allenbach, Yves; Benveniste, Olivier; Léonard-Louis, Sarah; Hervier, B.; Mariampillai, K.; Nespola, Benoît; Lannes, Béatrice; Echaniz‐Laguna, Andoni; Wendling, Daniel; Frenckell, Christian Von; Poursac, Nicolas; Mortier, E.; Lavigne, Christian; Hinschberger, O.; Magnant, Julie; Gottenberg, Jacques-Éric; Gény, Bernard; Sibilia, Jean; Meyer, Alain

doi:10.1212/wnl.0000000000008863

Cited by 25 publications

(5 citation statements)

References 24 publications

(47 reference statements)

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“…Most patients with acute sarcoid myopathy improve under systemic treatment, whereas chronic sarcoid myopathy is frequently associated with severe disability and rarely improves after corticosteroid treatment, immunosuppressive or anti-tumor necrosis factor (TNF)-α [75,76]. In a recent study, including 23 patients with granulomatous myositis, Dieudonné et al reported that almost half of the patients met the criteria for sporadic inclusion body myositis which was the only factor associated with unresponsiveness to treatment in patients with granulomatous myositis [77].…”

Section: Musculoskeletal Manifestationsmentioning

confidence: 99%

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Sève

Pachéco

Durupt

et al. 2021

Cells

245

144

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Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25–50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and renal dysfunction, affect less than 10% of patients. The diagnosis is not standardized but is based on three major criteria: a compatible clinical and/or radiological presentation, the histological evidence of non-necrotizing granulomatous inflammation in one or more tissues and the exclusion of alternative causes of granulomatous disease. Certain clinical features are considered to be highly specific of the disease (e.g., Löfgren’s syndrome, lupus pernio, Heerfordt’s syndrome) and do not require histological confirmation. New diagnostic guidelines were recently published. Specific clinical criteria have been developed for the diagnosis of cardiac, neurological and ocular sarcoidosis. This article focuses on the clinical presentation and the common differentials that need to be considered when appropriate.

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Section: Musculoskeletal Manifestationsmentioning

confidence: 99%

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Sève

Pachéco

Durupt

et al. 2021

Cells

245

144

View full text Add to dashboard Cite

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“…as may be the case without muscle biopsy/biopsies). 7,11,13,14 In the index case, polymyositis was deemed (clinically) unlikely due to the evolutionary timelines of her symptoms, and on account of the poor clinical response to a trial of steroid therapy. Some other relevant differential diagnoses that were considered, but deemed unlikely in this case, as guided by clinical and/or other histological grounds were: MND, a drug-associated myopathy, myasthenia gravis, Lambert-Eaton Myasthenic syndrome (LEMS), diabetic amyotrophy, thyrotoxic myopathy, post-viral myocarditis, muscular dystrophies (e.g.…”

Section: Some Differential Diagnoses Of Ibmmentioning

confidence: 99%

“…hypokalemic periodic paralysis). 1,2,8,9,[14][15][16][17][18] General principles of management of IBM Some prospective treatment options have been explored (e.g. steroids, other immunosuppressants and/or other immunomodulators, and human monoclonal antibodies), but at this time, in the absence of a definitive cure or curative treatments, the management of IBM is often redirected towards supportive measures and symptomalleviation.…”

Section: Some Differential Diagnoses Of Ibmmentioning

confidence: 99%

Inclusion body myositis in an older patient

Ogundipe

Dherwani²

2022

Int J Basic Clin Pharmacol

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Inclusion body myositis (IBM) is a condition also referred to as sporadic IBM. It is a rare variant of a broader group of diseases described under the banner of inflammatory myositis. In general, myositis/myositides tend to present with a varying cluster of symptoms such as muscular weakness, aches and/or pain. Myositis has been linked to multiple causes or triggers, but generally share the common feature (present to a variable extent) of inflammatory changes within skeletal muscle tissue. In addition, some associations (e.g. Sjogren’s disease and systemic lupus erythematosus – SLE) have been reported for IBM. Some medication classes (e.g. statins and fibrates) may present a risk to certain individuals developing drug-associated myopathies. IBM can be challenging to diagnose, and may be mistaken for many other causes of muscle weakness e.g. polymyositis. Furthermore, IBM tends to affect muscles asymmetrically, and runs a typically progressive and chronic course. Consequently, over time IBM may result in significant functional impairment, activity limitation, and participation restriction. This case report describes an older woman with a clinical diagnosis of probable IBM complicated with oesophageal dysmotility. She presented to hospital with progressive dysphagia, breathlessness, and a productive cough for which treatment was started for aspiration pneumonia. The report considers some broader ‘principles of management’ of a chronic myositis. In the current absence of a definitive curative treatment, we also discuss some realistic and practical pharmacological treatment options that may be used for the selective care of patients presenting with an incurable chronic myositis such as IBM.

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“…Muscular sarcoidosis can have a severe course in a significant proportion of patients, leading to corticosteroid dependence and disability [11,12]. There are no clear treatment recommendations for this disease, and the therapeutic strategies developed in the literature are based on small retrospective series, given the rarity of the disease.…”

Section: Introductionmentioning

confidence: 99%

“…There are no clear treatment recommendations for this disease, and the therapeutic strategies developed in the literature are based on small retrospective series, given the rarity of the disease. Currently, the classic treatment is corticosteroid therapy alone as a first line with the addition of immunosuppressive treatments in case of relapse [9,11,12].…”

Section: Introductionmentioning

confidence: 99%

Spatial Transcriptomics Reveals Signatures of Histopathological Changes in Muscular Sarcoidosis

Lequain,

Dégletagne,

Streichenberger

et al. 2023

Cells

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Sarcoidosis is a multisystemic disease characterized by non-caseating granuloma infiltrating various organs. The form with symptomatic muscular involvement is called muscular sarcoidosis. The impact of immune cells composing the granuloma on the skeletal muscle is misunderstood. Here, we investigated the granuloma–skeletal muscle interactions through spatial transcriptomics on two patients affected by muscular sarcoidosis. Five major transcriptomic clusters corresponding to perigranuloma, granuloma, and three successive muscle tissue areas (proximal, intermediate, and distal) around the granuloma were identified. Analyses revealed upregulated pathways in the granuloma corresponding to the activation of T-lymphocytes and monocytes/macrophages cytokines, the upregulation of extracellular matrix signatures, and the induction of the TGF-β signaling in the perigranuloma. A comparison between the proximal and distal muscles to the granuloma revealed an inverse correlation between the distance to the granuloma and the upregulation of cellular response to interferon-γ/α, TNF-α, IL-1,4,6, fibroblast proliferation, epithelial to mesenchymal cell transition, and the downregulation of muscle gene expression. These data shed light on the intercommunications between granulomas and the muscle tissue and provide pathophysiological mechanisms by showing that granuloma immune cells have a direct impact on proximal muscle tissue by promoting its progressive replacement by fibrosis via the expression of pro-inflammatory and profibrosing signatures. These data could possibly explain the evolution towards a state of disability for some patients.

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Granulomatosis-associated myositis

Cited by 25 publications

References 24 publications

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Inclusion body myositis in an older patient

Spatial Transcriptomics Reveals Signatures of Histopathological Changes in Muscular Sarcoidosis

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