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.