"Amyopathic" MDA5-positive dermatomyositis with severe lung involvement presenting with net myositic morphological features - insights from an autopsy study
Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:36 |
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| Enthalten in: |
Neuromuscular disorders : NMD - 36(2024) vom: 05. März, Seite 42-47 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Englert, Benjamin [VerfasserIn] |
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| Links: |
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| Themen: |
Amyopathic |
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| Anmerkungen: |
Date Completed 04.03.2024 Date Revised 04.03.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.nmd.2024.01.009 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM368446883 |
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| 520 | |a Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved. | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Amyopathic | |
| 650 | 4 | |a Dermatomyositis | |
| 650 | 4 | |a Inflammatory myopathy | |
| 650 | 4 | |a MDA5-DM | |
| 650 | 7 | |a Autoantibodies |2 NLM | |
| 650 | 7 | |a Interferon-Induced Helicase, IFIH1 |2 NLM | |
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| 700 | 1 | |a Goebel, Hans-Hilmar |e verfasserin |4 aut | |
| 700 | 1 | |a Schneider, Udo |e verfasserin |4 aut | |
| 700 | 1 | |a Holzer, Marie-Therese |e verfasserin |4 aut | |
| 700 | 1 | |a Uruha, Akinori |e verfasserin |4 aut | |
| 700 | 1 | |a Stenzel, Werner |e verfasserin |4 aut | |
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