Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group
| Autor: | Guadalupe Fraile-Rodriguez, Anne Labirua-Iturburu, Luis Sáez-Comet, Josep Maria Grau-Junyent, Francisco J. García-Hernández, Iñigo Les-Bujanda, Ernesto Trallero-Araguás, Albert Selva-O'Callaghan, Mónica Rodríguez-Carballeira, Manuel Monteagudo-Jiménez |
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| Rok vydání: | 2016 |
| Předmět: |
Adult
Male medicine.medical_specialty Pathology medicine.medical_treatment Antisynthetase syndrome Asymptomatic Pulmonary function testing 03 medical and health sciences FEV1/FVC ratio 0302 clinical medicine Rheumatology Internal medicine medicine Humans Lung transplantation Registries 030212 general & internal medicine Myositis Aged Retrospective Studies 030203 arthritis & rheumatology business.industry Arthritis Interstitial lung disease Retrospective cohort study Middle Aged respiratory system medicine.disease respiratory tract diseases Anesthesiology and Pain Medicine Antibodies Antinuclear Female medicine.symptom Lung Diseases Interstitial business |
| Zdroj: | Seminars in Arthritis and Rheumatism. 46:225-231 |
| ISSN: | 0049-0172 |
| Popis: | To evaluate the clinical manifestations, long-term clinical outcome and longitudinal pulmonary function in a large cohort of Spanish patients with anti-Jo1 antibodies.We retrospectively analyzed 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.Median follow-up was 78.3 months. Clinical presentation patterns at onset are as follows: 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 dyspnea 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 (p0.0001) were related to poorer survival. Mortality was significantly higher than in the general Spanish population, with a standardised mortality ratio (95% CI) of 4.03 (2.79-5.64).Anti-Jo1 ASS is a heterogeneous syndrome. ILD in ASS under immunosuppressive therapy is mainly a non-progressive disease. Dyspnea at ILD onset and a steady FVC decrease over time were related to a poorer respiratory prognosis. |
| Databáze: | OpenAIRE |
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