Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients

Abstract

We sought to investigate the characteristics, survival and risk factors for mortality in Chinese patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) in modern therapy era.129 consecutive adult patients who visited one of three referral centres in China with a diagnosis of CTD-APAH confirmed by right heart catheterisation during the previous 5 years were enrolled. The end-point was all-cause death or data censoring.Systemic lupus erythematosus was the most common underlying CTD (49%) and systemic sclerosis just accounted for 6% in this cohort. The overall survival at 1 and 3 years was 92% and 80%, respectively. Pericardial effusion, a shorter 6-min walk distance, lower mixed venous oxygen saturation, higher pulmonary vascular resistance (PVR) and alkaline phosphatase (ALP), and lower total cholesterol levels were all associated with a higher risk of death among the study population. Higher PVR and ALP were independent predictors of mortality.In conclusion, unlike in western patients, systemic lupus erythematosus is the most common underlying disease in Chinese patients with CTD-APAH. The survival of Chinese patients with CTD-APAH in the modern treatment era is similar to that in western countries. Elevated PVR and ALP are independent risk factors for poor outcomes. @ERSpublications Systemic lupus erythematosus is the most common underlying disease in Chinese patients with CTD-APAH

Pulmonary Hypertension in Connective Tissue Disease is Associated with the New York Heart Association Functional Class and Forced Vital Capacity, But Not with Interstitial Lung Disease

“…In patients with SLE, the prevalence of PH was reported with variety from 0.5% to 14% [30], although PH is not common compared with other manifestations. Although SLE was recently reported the most common underline disease in Chinese patients with CTD associated PH [31], our study showed similar prevalence (9.3%) to western data. PH is known to be rare in patients with RA; however, the mean pulmonary artery pressures measured by echocardiography were reported to be elevated and the prevalence of PH was higher in the patients with RA than in healthy controls (21% vs. 4%, respectively) [32].…”

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…PAH associated with CTD is the second most prevalent type of PAH after IPAH in Western countries [10]. SSc, particularly in its limited variant, represents the main CTD associated with PAH in Europe and the USA (systemic lupus erythematosus is more common in Asia) [325,329]. The prevalence of haemodynamically proven pre-capillary PH in large cohorts of SSc patients ranges from 5 to 12% [46,325,330,331].…”

“…Recommendations for PAH associated with CHD are reported in the Table 25. 7.3 Pulmonary arterial hypertension associated with connective tissue disease PAH is a well-known complication of CTDs such as SSc, systemic lupus erythematosus, mixed CTD and, to a lesser extent, rheumatoid arthritis, dermatomyositis and Sjögren's syndrome [325][326][327][328][329]. PAH associated with CTD is the second most prevalent type of PAH after IPAH in Western countries [10].…”

Reynolds Syndrome: a rare rheumatologic disease that internists should have in mind. Case report

“…According to Stadie (25), the coincidence of progressive systemic sclerosis and primary biliary cirrhosis seems to be a favorable association for the progression of primary biliary cirrhosis; in this case, the good prognosis of RS could be affected by a pulmonary complication (26).…”

“…Observational studies have demonstrated that mortality remains high in SSc patients with PAH; more specifically, the three-year survival rate for SSc patients with PAH has been estimated in 56%, compared with 94% in those without PAH, therefore, constituting itself as a leading cause of morbidity and mortality (1) (26,29,30).…”