[Fatal interstitial lung disease and pneumocystis during dermatomyositis associated with anti-MDA5 antibodies].
Dermatomyosite à anticorps anti-MDA-5 compliquée d’une pneumopathie interstitielle et d’une pneumocystose d’évolution fatale.
Anti-MDA5 antibody
Anticorps anti-MDA5
Dermatomyosite
Dermatomyositis
Interstitial lung disease
Pneumocystose
Pneumocystosis
Pneumopathie interstitielle diffuse
Journal
Annales de dermatologie et de venereologie
ISSN: 0151-9638
Titre abrégé: Ann Dermatol Venereol
Pays: France
ID NLM: 7702013
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
13
01
2020
revised:
24
06
2020
accepted:
05
08
2020
pubmed:
3
11
2020
medline:
16
10
2021
entrez:
2
11
2020
Statut:
ppublish
Résumé
Dermatomyositis (DM) in an auto-immune inflammatory myopathy with skin lesions, and, occasionally, organ involvement. Herein, we report a case of DM during anti-MDA5 antibody therapy associated with interstitial lung disease (ILD) and pneumocystosis. A 64-year-old woman was hospitalized for impairment of her general health and skin lesions. Dermatological examination revealed classic signs of DM associated with hyperkeratotic papules on the palm creases. This led us to suspect DM with anti-MDA5 antibodies, which was subsequently confirmed by immunologic tests. We also noted dysphonia, exertional dyspnea and proximal muscles weakness. Despite early corticosteroid therapy, combined later with azathioprine, the patient's dyspnoea worsened; one month later, sudden pulmonary decompensation resulted in her admission to intensive care. A chest scan showed evidence of ILD and infectious signs, and the bronchoalveolar lavage was positive for Pneumocystisjiroveci. Despite treatment of this opportunist infection with cotrimoxazole and intensified immunosuppression, the patient died in intensive care. Anti-MDA5 antibodies are associated with a specific clinical phenotype and a high degree of risk that should alert the dermatologist to the high likelihood of ILD having a poor prognosis. Associated clinical signs are erythematous, hyperkeratotic or ulcerated papules on the palm creases, as well as fingertip or periungual ulcerations or digital necrosis. This situation is associated with a high risk of pneumocystosis. However, no recommendations concerning prophylaxis are currently available.
Sections du résumé
BACKGROUND
BACKGROUND
Dermatomyositis (DM) in an auto-immune inflammatory myopathy with skin lesions, and, occasionally, organ involvement. Herein, we report a case of DM during anti-MDA5 antibody therapy associated with interstitial lung disease (ILD) and pneumocystosis.
PATIENTS AND METHODS
METHODS
A 64-year-old woman was hospitalized for impairment of her general health and skin lesions. Dermatological examination revealed classic signs of DM associated with hyperkeratotic papules on the palm creases. This led us to suspect DM with anti-MDA5 antibodies, which was subsequently confirmed by immunologic tests. We also noted dysphonia, exertional dyspnea and proximal muscles weakness. Despite early corticosteroid therapy, combined later with azathioprine, the patient's dyspnoea worsened; one month later, sudden pulmonary decompensation resulted in her admission to intensive care. A chest scan showed evidence of ILD and infectious signs, and the bronchoalveolar lavage was positive for Pneumocystisjiroveci. Despite treatment of this opportunist infection with cotrimoxazole and intensified immunosuppression, the patient died in intensive care.
DISCUSSION
CONCLUSIONS
Anti-MDA5 antibodies are associated with a specific clinical phenotype and a high degree of risk that should alert the dermatologist to the high likelihood of ILD having a poor prognosis. Associated clinical signs are erythematous, hyperkeratotic or ulcerated papules on the palm creases, as well as fingertip or periungual ulcerations or digital necrosis. This situation is associated with a high risk of pneumocystosis. However, no recommendations concerning prophylaxis are currently available.
Identifiants
pubmed: 33131899
pii: S0151-9638(20)31039-5
doi: 10.1016/j.annder.2020.08.046
pii:
doi:
Substances chimiques
Autoantibodies
0
Interferon-Induced Helicase, IFIH1
EC 3.6.4.13
Types de publication
Case Reports
Langues
fre
Sous-ensembles de citation
IM
Pagination
862-867Informations de copyright
Copyright © 2020. Published by Elsevier Masson SAS.