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

Abstract：

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.

journal_name

Eur J Intern Med

journal_title

European journal of internal medicine

authors

Marie I,Josse S,Decaux O,Diot E,Landron C,Roblot P,Jouneau S,Hatron PY,Hachulla E,Vittecoq O,Menard JF,Jouen F,Dominique S

doi

10.1016/j.ejim.2013.01.002

subject

Has Abstract

pub_date

2013-07-01 00:00:00

pages

474-9

issue

eissn

0953-6205

issn

1879-0828

pii

S0953-6205(13)00007-1

journal_volume

pub_type

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