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Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

2022Artigo científico Acesso restrito
http://hdl.handle.net/10451/58144
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Autores

Cavagna, Lorenzo
Meloni, Federica
Meyer, Alain
Sambataro, Gianluca
Belliato, Mirko
De Langhe, Ellen
Cavazzana, Ilaria
Pipitone, Nicolò
Triantafyllias, Konstantinos
Mosca, Marta

Orientador(es)

Resumo(s)

Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Descrição

© Copyright Clinical and Experimental Rheumatology 2022.

Palavras-chave

Melanoma differentiation-associated protein 5 antibody Rapidly progressive interstitial lung diseases Idiopathic inflammatory myopathies

URI

http://hdl.handle.net/10451/58144

Contexto Educativo

Citação

Clin Exp Rheumatol. 2022 Feb;40(2):274-283

Projetos de investigação

Unidades organizacionais

Fascículo

Editora

Pacini Editore SpA

DOI

10.55563/clinexprheumatol/di1083

Coleções

IMM - Artigos em Revistas Internacionais
FM-CUR-Artigos em Revistas Internacionais

Licença CC

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