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

BackgroundThe aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS).MethodsThe medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection.ResultsAnti-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).ConclusionOur 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.