"Amyopathic" MDA5-positive dermatomyositis with severe lung involvement presenting with net myositic morphological features - insights from an autopsy study.

Autor: Englert B; Center for Neuropathology and Prion Research, Faculty of Medicine, LMU Munich, Feodor-Lynen-Strasse 23, 81377 Munich, Germany. Electronic address: benjamin.englert@med.uni-muenchen.de., Dittmayer C; Department of Neuropathology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany., Goebel HH; Department of Neuropathology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Department of Neuropathology, Universitätsmedizin Mainz, Langenbeckstrasse 1, 55131 Mainz, Germany., Schneider U; Department of Rheumatology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany., Holzer MT; Division of Rheumatology and Systemic Inflammatory Diseases, III. Department of Medicine, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Germany., Uruha A; Department of Neuropathology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany., Stenzel W; Department of Neuropathology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany.
Jazyk: angličtina
Zdroj: Neuromuscular disorders : NMD [Neuromuscul Disord] 2024 Mar; Vol. 36, pp. 42-47. Date of Electronic Publication: 2024 Feb 04.
DOI: 10.1016/j.nmd.2024.01.009
Abstrakt: Anti-MDA5-positive dermatomyositis (MDA5-DM) often presents with extramuscular, especially pulmonary and skin manifestations, and apparent clinical signs of frank myositis can be missing (so called amyopathic DM). We hereby present two male patients who died from respiratory failure during the course of MDA5-DM. While overt signs of myositis or any skin involvement were absent at admission to hospital we noticed conspicuous inflammatory alterations in various skeletal muscles morphologically, showing different degrees of affection. Furthermore, pathological changes of the lungs compatible with rapid progressive interstitial lung disease and characteristic cutaneous vasculoocclusive features were identified at autopsy. This observation shows that muscles and skin are subclinically affected in a widespread fashion, hence subtle signs of muscle involvement should be sought after in anti-MDA5-positive patients with predominant lung affection to ensure adequate treatment.
Competing Interests: Declaration of competing interest Benjamin Englert does not disclose any conflict of interest Carsten Dittmayer does not disclose any conflict of interest Hans-Hilmar Goebel does not disclose any conflict of interest Udo Schneider does not disclose any conflict of interest Marie-Therese Holzer does not disclose any conflict of interest Akinori Uruha does not disclose any conflict of interest Werner Stenzel does not disclose any conflict of interest
(Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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