Clinical Manifestations and Long-Term Outcome of Anti-Jo1 Antisynthetase Patients in a Large Cohort of Spanish Patients from the GEAS-IIM Group

Objective To evaluate the clinical manifestations, long-term clinical outcome and longitudinal pulmonary function in a large cohort of Spanish patients with anti-Jo1 antibodies. Methods We retrospectively analysed the clinical data and lung function parameters of 148 anti-Jo1 patients recruited from...

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Published in:Seminars in arthritis and rheumatism 2016-10, Vol.46 (2), p.225-231
Main Authors: Trallero-Araguás, Ernesto, MD, PhD, Grau-Junyent, Josep María, MD, PhD, Labirua-Iturburu, Anne, MD, PhD, García-Hernández, Francisco José, MD, Monteagudo-Jiménez, Manuel, MD, PhD, Fraile-Rodriguez, Guadalupe, MD, Les-Bujanda, Iñigo, MD, PhD, Rodriguez-Carballeira, Mónica, MD, PhD, Saez-Comet, Luis, MD, PhD, Selva-O’callaghan, Albert, MD, PhD
Format: Article
Language:English
Subjects:
Adult
Aged
Anti-Jo1 antibodies
Antibodies, Antinuclear - immunology
Antisynthetase syndrome
Arthritis - complications
Arthritis - immunology
Female
Humans
Idiopathic Inflammatory myopathies
Lung Diseases, Interstitial - complications
Lung Diseases, Interstitial - immunology
Male
Middle Aged
Myositis - complications
Myositis - immunology
Myositis specific autoantibody
Registries
Retrospective Studies
Rheumatology
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Summary:Objective To evaluate the clinical manifestations, long-term clinical outcome and longitudinal pulmonary function in a large cohort of Spanish patients with anti-Jo1 antibodies. Methods We retrospectively analysed the clinical data and lung function parameters of 148 anti-Jo1 patients recruited from a multicentre registry including 18 Spanish hospitals. A composite endpoint was defined, comprising death due to respiratory failure directly related to antisynthetase syndrome (ASS), the need for long-term oxygen therapy or lung transplantation. Results Median follow-up was 78.3 months. Clinical presentation patterns at onset: isolated interstitial lung disease (ILD) (32.4%), isolated myositis (26.9%), concomitant myositis and ILD (22.8%), and isolated polyarthritis (17.9%). Myositis with ILD was the most frequent final clinical phenotype (67.6%). In most ASS patients, ILD was a non-progressive disease, tending to stabilize with therapy. The endpoint was reached in a significantly larger number of ILD patients with dyspnoea at onset than those with paucisymptomatic or asymptomatic forms (p=0.01). A steady FVC decrease was the hallmark of patients with end-stage lung disease. Estimated survival rates were 87.7% and 75.4% at 5 and 10 years, respectively. Cancer (p=0.02) and advanced age at ASS diagnosis (p
ISSN:0049-0172
1532-866X
DOI:10.1016/j.semarthrit.2016.03.011