Abstract
OBJECTIVE: To identify clinical and laboratory risk factors for Pulmonary Arterial Hypertension (PAH) in Idiopathic-Inflammatory-Myopathy (IIM) patients as well as construct a predicting model for PAH. METHODS: An IIM cohort in southeastern China was established, including 504 adult IIM patients who met the inclusion and exclusion criteria, and were hospitalized at four divisions of the First Affiliated Hospital, Zhejiang University School of Medicine (FAHZJU) from January 1st, 2018, to April 30st 2022. Serum cytokine profiles, myositis-specific antibodies as well as other factors of patients who met the inclusion and exclusion criteria were collected and analyzed. RESULTS: Of the 504 adult IIM patients, 25 IIM patients developed PAH and 48.0 % of them deceased within six months. IIM patients complicated with PAH were found to suffer from worse outcome (p < 0.001). After multivariate logistic regression analysis, age (p < 0.001), bacterial infection (p = 0.005), MYOACT score (p = 0.009), Interleukin (IL)-17A (p = 0.017), anti-SRP antibody (p = 0.011) and steroid monotherapy (p = 0.001) were identified as factors significantly associated with PAH in IIM patients. A "BAIMS" model was constructed by including the above six items to predict PAH with a cut-off value of ≥ 3 and an Area Under the Curve (AUC) of 0.877. CONCLUSION: PAH is a rare but potentially fatal complication in IIM patients. Aging, complication of bacterial infection, elevated disease activity, increased serum IL-17A level, positivity of anti-SRP antibody and steroid monotherapy were significantly correlated with development of PAH in IIM patients. In addition, the BAIMS model was found valuable in prediction and early-identification of PAH in IIM patients.