Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies
Cavagna, Lorenzo ; Cagnotto, Giovanni LU
and Gonzalez-Gay, M. A.
(2022)
In Clinical and Experimental Rheumatology
40(2).
p.274-283
- Abstract
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... (More)
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.
(Less)
- author
- Cavagna, Lorenzo
; Cagnotto, Giovanni
LU
and Gonzalez-Gay, M. A.
- author collaboration
- publishing date
- 2022
- type
- Contribution to journal
- publication status
- published
- keywords
- Autoantibodies, Dermatomyositis/complications, Female, Humans, Interferon-Induced Helicase, IFIH1, Lung Diseases, Interstitial/drug therapy, Middle Aged, Prognosis, Retrospective Studies
- in
- Clinical and Experimental Rheumatology
- volume
- 40
- issue
- 2
- pages
- 274 - 283
- publisher
- Pacini
- external identifiers
-
- pmid:35200123
- scopus:85125551103
- ISSN
- 0392-856X
- language
- English
- LU publication?
- no
- id
- 107b18c9-5c4e-470e-8910-8e61cb224d5e
- alternative location
- https://www.clinexprheumatol.org/abstract.asp?a=17797
- date added to LUP
- 2022-03-22 23:47:16
- date last changed
- 2025-02-02 08:32:32
@article{107b18c9-5c4e-470e-8910-8e61cb224d5e,
abstract = {{<p>OBJECTIVES: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies.</p><p>METHODS: We conducted a multicentre, international, retrospective cohort study.</p><p>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.</p><p>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.</p>}},
author = {{Cavagna, Lorenzo and Cagnotto, Giovanni and Gonzalez-Gay, M. A.}},
issn = {{0392-856X}},
keywords = {{Autoantibodies; Dermatomyositis/complications; Female; Humans; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial/drug therapy; Middle Aged; Prognosis; Retrospective Studies}},
language = {{eng}},
number = {{2}},
pages = {{274--283}},
publisher = {{Pacini}},
series = {{Clinical and Experimental Rheumatology}},
title = {{Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies}},
url = {{https://www.clinexprheumatol.org/abstract.asp?a=17797}},
volume = {{40}},
year = {{2022}},
}