Primary Sjogren's syndrome associated pulmonary arterial hypertension: 20 new cases

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Objective: To describe baseline characteristics of patients with primary Sjogren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH). Methods: This retrospective study included consecutive patients hospitalized with pSS-PAH from Jan.2013 to June.2019 in Tianjin Medical University General Hospital. All patients fulfilled the 2002 revised criteria for pSS proposed by the American-European Consensus Group. PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines. Associated variables were described and analyzed. Predictive factors for achieving treatment goals were explored. Sub-groups(based on Anti-SSB antibody positive/negative) were analyzed. Results: Twenty patients with RHC-confirmed pSS-PAH were included (median age at onset, 54.5 years; median PAH duration, 12 months).PAH was the initial manifestation of pSS in 5 patients(26.3%), and shortness of breath was the most common symptom (17/20,85%). Mean pulmonary arterialpressure (mPAP) was 48(38, 55.75)mmHg, pulmonary vascularresistance (PVR) was 11.23 (6.17,14.69)IU, and mean cardiac index was 2.59(2.04,2.98)L·min(-1)·m(-2) in this group. In addition, right ventricular diameter≤40 mm[P=0.009, HR=5.92, 95%CI(1.55, 22.59)] was predictive factor for achieving the treatment goal. Furthermore, compared with Anti-SSB antibody negative patients, patients with Anti-SSB antibody positive had a higher PVR(IU) [14.1(11.23, 22.63)vs 6.4(6.15, 12.36),P=0.025], a higher mPAP(mmHg)[5(48.5,59)vs 39(32.5,50.75),P=0.013], and a lower cardiac index (L·min(-1)·m(-2)) [2.1(1.6,2.5)vs 2.9(2.56,3.41),P=0.002]. Conclusions: pSS-PAH patients are mainly middle-aged women with the most common symptom shortness of breath. And PAH can be the first manifestation of pSS. More attention should be paid to patients with Anti-La/SSB antibody positive and right ventricular diameter ≤40 mm because of higher mPAP, PVR, worse cardiac function and prognosis.