A severe anti MDA-5 antibodies associated amyopathic dermatomyositis
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Abstract
Background: Lung damage during amyopathic dermatomyositis (ADM) associated with auto antibodies anti MDA-5 is serious. We report a rare observation of a severe ADM associated to anti MDA-5 antibodies complicated with diffuse interstitial lung disease (ILD) rapidly extensive and fatal.
Observation: We report the observation of a Tunisian adult who was admitted for ADM. The anti MDA-5 antibodies were detected. He developed an ILD rapidly extensive to
fibrosis. Corticosteroids were unefficient and he deceded of pneumomediastin and refractory respiratory distress.
Conclusion: This observation illustrates the severity of ADM with anti MDA-5 antibodies. Every clinician should detect any ILD to avoid the progression to fibrosis.
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References
- Gerfaud-Valentina M, Ahmada K, Piegaya F, et al. Pneumopathie infiltrante diffuse associée à une dermatomyosite amyopathique avec auto-anticorps anti-MDA5. Revue des Maladies Respiratoires 2014;31:849-853
- Sato S, Hirakata M, Kuwana M et al. Autoantibodies to a 140- kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis and Rheumatism 2005;52:1571-6.
- Chen Z, Cao M, Plana MN, et al. Utility of anti-melanoma differentiation-associated gene 5antibody measurement in identifying patients with dermatomyositis and a high risk for developing rapidly progressive interstitial lung disease: a review of the literature and a meta-analysis. Arthritis Care Res 2013;65:1316-24.