Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group

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• 作者：Ernesto Trallero-Araguá ; s ; MD ; PhD^a ; ^{etrallero@vhebron.net" class="auth_mail" title="E-mail the corresponding author} ; Josep Marí ; a Grau-Junyent ; MD ; PhD^b ; Anne Labirua-Iturburu ; MD ; PhD^a ; Francisco José ; Garcí ; a-Herná ; ndez ; MD^c ; Manuel Monteagudo-Jimé ; nez ; MD ; PhD^d ; Guadalupe Fraile-Rodriguez ; MD^e ; Iñ ; igo Les-Bujanda ; MD ; PhD^f ; Mó ; nica Rodriguez-Carballeira ; MD ; PhD^g ; Luis Sá ; ez-Comet ; MD ; PhD^h ; Albert Selva-O&rsquo ; Callaghan ; MD ; PhD^a ; on behalf of the IIM Study Group ; Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI)
• 关键词：Anti-Jo1 antibodies ; Antisynthetase syndrome ; Idiopathic Inflammatory myopathies ; Myositis specific autoantibody
• 刊名：Seminars in Arthritis and Rheumatism
• 出版年：2016
• 出版时间：October 2016
• 年：2016
• 卷：46
• 期：2
• 页码：225-231
• 全文大小：1071 K

文摘

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 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.

Results

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 (p < 0.0001) 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).

Conclusions

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.