Observational studyBrain pathology (Zurich, Switzerland)2020
Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis.
Observational study in Brain pathology (Zurich, Switzerland), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 16 citations in OpenAlex.
- Revisiting the link between oropharyngeal dysphagia and cancer in autoimmune myositis: a descriptive study.Arthritis research & therapy · 2025Article
- Bullous periorbital oedema in anti-p155/140-positive dermatomyositis: a case series.Rheumatology (Oxford, England) · 2023Article
- Dermatomyositis Presenting with Generalized Subcutaneous Edema.Internal medicine (Tokyo, Japan) · 2023Article
- Anti-MDA5 dermatomyositis after COVID-19 vaccination: a case-based review.Rheumatology international · 2022Review
- Clinically amyopathic dermatomyositis associated with anti-nuclear matrix protein 2 antibody.Rheumatology advances in practice · 2021Article
- Pericardial effusion in idiopathic inflammatory myopathies: A cross-sectional study from Asia and review of the literature.International journal of immunopathology and pharmacologyReview
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
aimsEdema 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 edematous myositis is a homogeneous subtype based on clinical, serological and pathological features.
methodsThis 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 edema were included as well as IIM cases without limb edema as controls. Clinical, biological and pathological features were collected.
resultsSeventeen edematous myositis were included and compared to 174 IIM without edema, including 50 DM controls. Edema 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 edema was found in 82% of cases. Seventeen percent of patients died (median follow up of 18 months). Edematous 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 edema.
conclusionOur study underlines that limb edema could be a symptom of IIM and that edematous myositis are mostly DM. The vasculopathy seems to play a key role in its pathophysiology. Limb edema associated with muscle impairment should suggest the diagnosis of DM in clinical settings.
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