Characteristics and risk factors for pulmonary arterial hypertension associated with primary Sjögren’s syndrome: 15 new cases from a single center

Abstract

Aim This study aimed to retrospectively describe 15 new primary Sjögren's syndrome‐pulmonary arterial hypertension (pSS‐PAH) cases confirmed by right heart catheterization (RHC). Demographic and clinical characteristics were analyzed and risk factors for PAH in pSS were explored. Method We retrospectively described 15 new pSS‐PAH cases confirmed by RHC referred to our institution between January 2013 and March 2018. We present PAH and pSS characteristics, hemodynamic evaluations, medical management, and outcomes. A matched case control study was carried out to determine the risk factors of PAH in pSS compared with pSS‐non‐PAH patients. Results All patients were female with a mean age at PAH diagnosis of 52.9 ± 14.6 years. The delay between the first symptom and PAH diagnosis was 18.7 ± 19.7 months. The most common primary manifestation at PAH onset was exertional dyspnea (13/15). At diagnosis of PAH, PAH was severe with a mean pulmonary artery pressure of 48.8 ± 13.7 mm Hg (range, 27‐72 mm Hg) and a mean cardiac index of 2.3 ± 0.6 L/min/m2 (range, 1.47‐3.41 L/min/m2). Compared with the pSS‐PAH without pericardial effusion, pSS‐PAH with pericardial effusion had larger right arterial (53 [45‐56.75] vs 38 [35.5‐46.5], P = .018) and right ventricular sizes (47 [42.75‐51.25] vs 36 [32.5‐41], P = .007). Compared with the pSS non‐PAH group, we identified 2 risk factors for PAH in pSS: pericardial effusion (odds ratio [OR] [95% CI], 14.29 [1.14‐166.67], P = .039) and liver involvement (OR [95% CI], 14.71 [1.14‐166.67], P = .035). Conclusion For pSS patients, PAH can be the first manifestation. We believe that systemic evaluation, especially in patients with pericardial effusion and liver involvement, is important to identify high‐risk patients for PAH, improving their prognosis.

Clinical characteristics and prognosis of connective tissue disease-associated pulmonary arterial hypertension

“…CTD-PAH individuals also had signi cantly higher RV and RA values than CTD-non-PAH patients, and they were more likely to develop secondary pericardial effusions (P < 0.001). Furthermore, we found that pericardial effusion was a risk factor for CTD-PAH, and there have been similar reports in the past [38][39][40].…”

Association of red cell distribution width with pulmonary arterial hypertension in patients with mixed connective tissue disease

“…In addition, in the present study, 17 of 46 patients (37.0%) had pericardial effusion by echocardiography. The literature suggests that pericardial effusion relates to RV failure and immunologically mediated inflammatory conditions [ 15 ]. Pericardial effusion is an increasingly recognized risk factor of PAH related to CTD [ 16 ].…”

2020 Chinese expert-based consensus on the diagnosis and treatment of connective tissue disease associated pulmonary arterial hypertension

“…The results of a prospective cohort study of SS-associated PAH in China suggested that PAH screening should be conducted for SS patients with pericardial effusion, liver involvement, Raynaud’s phenomenon, and high-titer rheumatoid factor. [ 24 , 32 ] If the screening does not indicate PAH but the primary disease activity persists in a high level or deteriorates, the screening should be repeated within 3–6 months. If the screening does not indicate PAH and the disease activity remains at a low level or the patient does not show new symptoms, the screening should be repeated once a year.…”