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Respiratory Medicine

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Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome - 29/08/16

Doi : 10.1016/j.rmed.2016.07.009 
Ana C. Zamora a, 1, Sumedh S. Hoskote a, 1, Beatriz Abascal-Bolado b, Darin White c, Christian W. Cox c, Jay H. Ryu a, Teng Moua a, ⁎
a Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA 
b Division of Pulmonary Medicine, Instituto de Investigacion Sanitaria Valdecilla (IDIVAL), Santander, Spain 
c Department of Radiology, Mayo Clinic, Rochester, MN, USA 

∗Corresponding author. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.Division of Pulmonary and Critical Care MedicineMayo Clinic200 First Street SWRochesterMN55905USA

Abstract

Background

Interstitial lung disease (ILD) is a common extra-muscular manifestation of antisynthetase (AS) syndrome. ILD prevalence is higher with anti-Jo-1 antibody positivity. Data on long-term outcomes in these patients are lacking.

Methods

Over 15 years, we identified subjects with anti-Jo-1 positive AS syndrome and ILD. Demographics, pulmonary function testing (PFT), high-resolution computed tomography (HRCT), histopathology, and long-term survival were analyzed.

Results

We identified 103 subjects (mean age 49.2 years, female predominance [70%]). The predominant myopathy was polymyositis (64%) followed by dermatomyositis (24%). In approximately half of studied subjects, AS syndrome and ILD were diagnosed within 6 months of each other. The majority had restriction on PFTs (98%). Non-specific interstitial pneumonia (NSIP) was the most common HRCT pattern (52%), followed by NSIP overlapping with organizing pneumonia (OP) (22%). Thirty-nine subjects had biopsy data. Ten-year survival was 68%. Multivariable analysis adjusted for age at ILD diagnosis, gender, FVC and DLCO, revealed that male gender (HR = 2.60, p = 0.04) and DLCO at presentation (HR = 0.94, p = 0.05) significantly predicted mortality.

Conclusions

We present a large cohort of anti-Jo-1 positive AS syndrome with ILD and note good overall survival.

El texto completo de este artículo está disponible en PDF.

Highlights

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Anti-jo-1 antisynthetase syndrome is commonly associated with interstitial lung disease.
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In this large single-center cohort, the most common presenting radiologic pattern was NSIP.
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Usual interstitial pattern on CT or pathology was not associated with worse survival.
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Male gender and low DLCO were independent predictors of poorer outcomes.

El texto completo de este artículo está disponible en PDF.

Keywords : Antisynthetase syndrome, Jo-1 antibody, Antinuclear antibody, Interstitial lung disease, Dermatomyositis, Polymyositis


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© 2016  Elsevier Ltd. Reservados todos los derechos.
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Vol 118

P. 39-45 - septembre 2016 Regresar al número
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