Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis
Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman wi...
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doaj-913c9402518e4e64a471eb1ca4887ca82021-04-22T23:03:04ZengMDPI AGClinics and Practice2039-72832021-04-01113523524010.3390/clinpract11020035Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic DermatomyositisHouari Aissaoui0Kinan Drak Alsibai1Naji Khayath2Department of Medicine, Pneumology Unit, Cayenne Hospital Center Andrée Rosemon, F-97306 Cayenne, FranceDepartment of Pathology and Centre of Biological Resources (CRB Amazonie), Cayenne Hospital Center Andrée Rosemon, F-97306 Cayenne, FranceChest Diseases Department, Strasbourg University Hospital, F-67000 Strasbourg, FranceAnti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management.https://www.mdpi.com/2039-7283/11/2/35anti-MDA5 antibodiesdermatomyositisrapidly progressive interstitial pneumopathypolyarthritis |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Houari Aissaoui Kinan Drak Alsibai Naji Khayath |
spellingShingle |
Houari Aissaoui Kinan Drak Alsibai Naji Khayath Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis Clinics and Practice anti-MDA5 antibodies dermatomyositis rapidly progressive interstitial pneumopathy polyarthritis |
author_facet |
Houari Aissaoui Kinan Drak Alsibai Naji Khayath |
author_sort |
Houari Aissaoui |
title |
Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis |
title_short |
Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis |
title_full |
Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis |
title_fullStr |
Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis |
title_full_unstemmed |
Fast-Onset Diffuse Interstitial Lung Disease in Anti-MDA5 Antibodies-Associated Amyopathic Dermatomyositis |
title_sort |
fast-onset diffuse interstitial lung disease in anti-mda5 antibodies-associated amyopathic dermatomyositis |
publisher |
MDPI AG |
series |
Clinics and Practice |
issn |
2039-7283 |
publishDate |
2021-04-01 |
description |
Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management. |
topic |
anti-MDA5 antibodies dermatomyositis rapidly progressive interstitial pneumopathy polyarthritis |
url |
https://www.mdpi.com/2039-7283/11/2/35 |
work_keys_str_mv |
AT houariaissaoui fastonsetdiffuseinterstitiallungdiseaseinantimda5antibodiesassociatedamyopathicdermatomyositis AT kinandrakalsibai fastonsetdiffuseinterstitiallungdiseaseinantimda5antibodiesassociatedamyopathicdermatomyositis AT najikhayath fastonsetdiffuseinterstitiallungdiseaseinantimda5antibodiesassociatedamyopathicdermatomyositis |
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1721513791938625536 |