• David Kiefer, Ioana Andreica, and Jürgen Braun.
• Rheumazentrum Ruhrgebiet, Ruhr-Universität Bochum.
• Dtsch. Med. Wochenschr. 2020 Mar 1; 145 (6): 394-398.

AbstractIn this case series we present three patients with autoimmune dermatomyositis or polymyositis with rapid-progressive interstitial pulmonary involvement. Despite intensive escalation of the immunosuppressive therapy the patients developed acute respiratory distress syndrome with lethal outcome only a few months after diagnosis. All three patients had increased myositis-specific or myositis-associated antibodies - two patients were detected with anti-melanoma differentiation-associated gene (MDA5) antibodies and the third patient with anti-Ku antibodies. Dermatomyositis and polymyositis are rare autoimmune diseases with variable clinical manifestations and several different antibody constellations. In particular the presence of anti-MDA5 antibodies is associated with progressive pulmonary involvement, complicated progression and poor prognosis.© Georg Thieme Verlag KG Stuttgart · New York.

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