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Oedematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis.

Published on Apr 22, 2020in Brain Pathology6.352
· DOI :10.1111/BPA.12844
Mathilde Duchesne , S. Leonard-Louis1
Estimated H-index: 1
(University of Paris)
+ 5 AuthorsYves Allenbach27
Estimated H-index: 27
(University of Paris)
Abstract
AIMS: Oedema of the limbs is uncommon in idiopathic inflammatory myopathies (IIM). The few reported cases have been associated with severe and refractory dermatomyositis (DM), sometimes in association with cancers. We aimed to determine if oedematous myositis is a homogeneous subtype based on clinical, serological and pathological features. METHODS: This is a retrospective observational study performed between 2008 and 2015 in the French national referral center for myositis. All adult patients with an inflammatory muscle biopsy and upper limbs oedema were included as well as IIM cases without limb oedema as controls. Clinical, biological and pathological features were collected. RESULTS: Seventeen oedematous myositis were included and compared to 174 IIM without oedema, including 50 DM controls. Oedema was the first manifestation in 23% of patients. Muscle weakness was severe and symmetric, 71% of patients presented dysphagia and a restrictive ventilatory pattern was found in 40%. Fifty-two percent of patients had a typical DM skin rash and 23% had cancer within 3 years of diagnosing myositis. Fifty-three percent of patients presented a myositis specific antibody and only DM-specific antibodies were detected. Classic pathological DM features (perifascicular atrophy, perifascicular/perimysial perivascular inflammation) were uncommon but capillary C5b-9 deposition and MxA expression were seen in 79% and 73% of cases, respectively. A perimysial oedema was found in 82% of cases. Seventeen percent of patients died (median follow up of 18 months). Oedematous myositis demonstrated more marked capillary C5b-9 deposition compared to IIM controls. There was no clinical, biological or pathological difference with DM controls except for limb oedema. CONCLUSION: Our study underlines that limb oedema could be a symptom of IIM and that oedematous myositis are mostly DM. The vasculopathy seems to play a key role in its pathophysiology. Limb oedema associated to muscle impairment should suggest the diagnosis of DM in clinical settings.
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References38
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#1Andrew L. Mammen (NIH: National Institutes of Health)H-Index: 37
#2Yves Allenbach (University of Paris)H-Index: 27
Last. Olivier Benveniste (University of Paris)H-Index: 39
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#1Johannes C. Nossent (UWA: University of Western Australia)H-Index: 33
#2Warren Raymond (UWA: University of Western Australia)H-Index: 5
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Summary Tubuloreticular inclusions (TRI) are distinctive cytoplasmic structures of unknown origin that typically associate with autoimmune and viral diseases. We investigated the clinical and prognostic relevance of TRI detection in patients with lupus nephritis (LN). We conducted a single centre study of patients (n=84) with biopsy evidence of LN. Clinical variables included demographics, SLEDAI score, and autoantibody profiling; while histological evaluation included TRI presence, Internationa...
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#1A. Uruha (Paris IV: Paris-Sorbonne University)H-Index: 1
#2Y. Allenbach (Paris IV: Paris-Sorbonne University)H-Index: 1
Last. O. Benveniste (Paris IV: Paris-Sorbonne University)H-Index: 2
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AIMS: To elucidate the diagnostic value of sarcoplasmic expression of myxovirus resistance protein A (MxA) for dermatomyositis (DM) specifically analysing different DM subforms, and to test the superiority of MxA to other markers. METHODS: Immunohistochemistry for MxA and retinoic acid-inducible gene I (RIG-I) was performed on skeletal muscle samples and compared with the item presence of perifascicular atrophy (PFA) in 57 DM patients with anti-Mi-2 (n = 6), -transcription intermediary factor 1 ...
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#1Beenish Fayyaz (Greater Baltimore Medical Center)H-Index: 1
#2Hafiz J. Rehman (UMD: University of Maryland, College Park)H-Index: 1
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ABSTRACTInflammatory myopathies are characterized by the skeletal muscle inflammation leading to symptoms of myopathy along with varying involvement of other organs such as lung, skin and joints. The strong association between inflammatory myopathies and malignancy has been well recognized. Recently, ‘cancer-associated myositis (CAM)’, has been proposed to be a paraneoplastic syndrome due to the anti-tumor immunity secondary to similar tumor and regenerating muscle antigens.As the prognosis ...
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#1K. Mariampillai (French Institute of Health and Medical Research)H-Index: 5
#2Benjamin Granger (UPMC: Pierre-and-Marie-Curie University)H-Index: 7
Last. Olivier Benveniste (French Institute of Health and Medical Research)H-Index: 39
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Importance Idiopathic inflammatory myopathies are heterogeneous in their pathophysiologic features and prognosis. The emergence of myositis-specific autoantibodies suggests that subgroups of patients exist. Objective To develop a new classification scheme for idiopathic inflammatory myopathies based on phenotypic, biological, and immunologic criteria. Design, Setting, and Participants An observational, retrospective cohort study was performed using a database of the French myositis network. Pati...
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Terminal complement complex deposition in endomysial capillaries detected by a C5b-9 immunostain is considered a diagnostic feature for dermatomyositis. However, we found widespread microvascular C5b-9 reactivity in a substantial subset of muscle biopsies with denervation changes, and in nerve biopsies of peripheral neuropathies, particularly in patients with diabetes. It is unclear whether the presence of C5b-9 deposition signifies active immune-mediated vascular injury that requires immune sup...
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#1Yves Allenbach (University of Paris)H-Index: 27
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#1Jemima Albayda (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 14
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Objective Dermatomyositis (DM) patients typically present with proximal weakness and autoantibodies that are associated with distinct clinical phenotypes. We observed that DM patients with autoantibodies recognizing the nuclear matrix protein NXP-2 often presented with especially severe weakness. The aim of this study was to characterize the clinical features associated with anti–NXP-2 autoantibodies. Methods There were 235 DM patients who underwent testing for anti–NXP-2 autoantibodies. Patient...
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#1Lisa G. Rider (NIH: National Institutes of Health)H-Index: 54
#2Nicolino Ruperto (Istituto Giannina Gaslini)H-Index: 64
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Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM. Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive rela...
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