[Anti-EJ antibody positive antisynthetase syndrome with pulmonary hypertention:a case report].

Antisynthetase syndrome(ASS) is an entity among the immune inflammatory myopathies(IIM), which always affects lungs. Interstitial lung disease(ILD) is common in ASS, while pulmonary hypertention(PH)is rarely observed. In this paper, we reported a case of ASS with ILD and PH. Initially, we thought PH maybe associated with ILD. After we finished pulmonary function test and right heart catheterization, rheumatism immunity,and myositis antibody spectrum,we finally considered that anti EJ antibody positive ASS related ILD (EJ-ASS-FILD) had secondary type 4 PH (chronic pulmonary disease-related pulmonary arterial hypertension). This article summarizes the clinical characteristics, diagnostic methods, and treatment options of ASS related ILD combined with PH, hoping that clinical doctors can strengthen their understanding of this disease, avoid misclassification and treatment of this disease.