To describe baseline characteristics of patients with primary Sjögren's syndrome (pSS) with right heart catheterization (RHC)-confirmed pulmonary arterial hypertension (PAH) and explore risk factors for PAH in pSS.
This case-control study included consecutive patients hospitalized with pSS-PAH from 2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. All patients fulfilled the 2002 American-European Consensus Group classification criteria for pSS-PAH was defined according to RHC-based European Society of Cardiology/European Respiratory Society guidelines. Associated variables were analyzed by univariate binary logistic regression to identify possible risk factors for PAH.
Twenty-nine patients with RHC-confirmed pSS-PAH were included (mean age at onset, 38.4 ± 8.3 years; mean pSS duration, 54.6 months). PAH was the initial manifestation of pSS in 12 patients (41.4%), and shortness of breath was the most common symptom (29/29, 100%). Mean pulmonary arterial pressure was 51.8 ± 10.0 mmHg, mean cardiac index was 2.3 ± 0.8 L/min/m
PAH can be the first manifestation of pSS. The pSS patients with Raynaud's phenomenon, high-titer rheumatoid factor, hepatic injury or pericardial effusion should be screened for PAH.