Characteristics and risk factors of pulmonary arterial hypertension in patients with primary Sjögren's syndrome

Aim 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. Methods 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. Results 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/m2, and mean pulmonary vascular resistance was 13.0 ± 6.0 Wood units in this group. Treatments included immunosuppressive agents (93.1%) and PAH‐targeted therapies (86.2%). We identified four independent risk factors for PAH in pSS: Raynaud's phenomenon (odds ratio [OR] = 9.660, P = 0.000), rheumatoid factor ≥ 200 U/mL (OR = 6.691, P = 0.001), hepatic injury (OR = 3.284, P = 0.008) and pericardial effusion (OR = 3.279, P = 0.016). Conclusions 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.