Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome

摘要

Background The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS). Methods The medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. Results Anti-PL7 patients exhibited polymyositis (n = 14) and dermatomyositis (n = 1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n = 5), was identified concomitantly with ASS (n = 8) and occurred after ASS diagnosis (n = 1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 1), progressive onset of lung signs (n = 11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n = 2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO 45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP). Conclusion Our series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO 45% at ILD diagnosis and UIP pattern on HRCT-scan.