Ana C Zamora1, Sumedh S Hoskote1, Beatriz Abascal-Bolado2, Darin White3, Christian W Cox3, Jay H Ryu1, Teng Moua4. 1. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA. 2. Division of Pulmonary Medicine, Instituto de Investigacion Sanitaria Valdecilla (IDIVAL), Santander, Spain. 3. Department of Radiology, Mayo Clinic, Rochester, MN, USA. 4. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA. Electronic address: moua.teng@mayo.edu.
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.
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.