Incidence and survival impact of pulmonary arterial hypertension among patients with systemic lupus erythematosus: a nationwide cohort study

Abstract

Background No population-based study has investigated the cumulative incidence of pulmonary arterial hypertension (PAH) in patients with newly diagnosed systemic lupus erythematosus (SLE) or the survival impact of PAH in this population. Method We used a nationwide database in Taiwan and enrolled incident SLE patients between January 1, 2000, and December 31, 2013. The cumulative incidence of PAH in the SLE patients and the survival of these patients were estimated by the Kaplan-Meier method. Potential predictors of the development of PAH were determined using a Cox proportional hazards regression model. Results Of 15,783 SLE patients, 336 (2.13%) developed PAH. The average interval from SLE diagnosis to PAH diagnosis was 3.66 years (standard deviation [SD] 3.36, range 0.1 to 13.0 years). Seventy percent of the patients developed PAH within 5 years after SLE onset. The 3- and 5-year cumulative incidence of PAH were 1.2% and 1.8%, respectively. Systemic hypertension was an independent predictor of PAH occurrence among the SLE patients (adjusted hazard ratio 2.27, 95% confidence interval 1.59–2.97). The 1-, 3-, and 5-year survival rates of SLE patients following the diagnosis of PAH were 87.7%, 76.8%, and 70.1%, respectively. Conclusions PAH is a rare complication of SLE and the majority of PAH cases occur within the first 5 years following SLE diagnosis. Systemic hypertension may be a risk factor for PAH development in the SLE population. The overall 5-year survival rate after PAH diagnosis was 70.1%.

Pulmonary hypertension in connective tissue diseases, new evidence and challenges

“…The prevalence of PAH in non‐SSc CTDs remains to be determined due to the lack of large, RHC based cohort studies but is estimated to be lower than in SSc patients 14 . Based on a nationwide cohort study in Taiwan and a systematic review of the literature, the prevalence of PAH in SLE is <4% 15,16 . For the other CTDs, the prevalence is estimated to be <1% 1…”

Systemic lupus erythematosus: year in review 2019

“…The overall 1-, 3-, and 5-year survival rates after PAH diagnosis were respectively 87.7%, 76.8%, and 70.1%. [ 36 ] Risk factors in identifying PAH from SLE were widely investigated last year. [ 37 , 38 ] In addition, some scholars proposed a novel category of SLE-PAH, vasculitic subtype and vasculopathic subtype according to the systemic manifestations and disease activity.…”

Pulmonary hypertension: Spectrum of disease, clinical presentation and treatment outcomes at the main respiratory pulmonary hypertension clinic in KwaZulu‑Natal Province, South Africa

“…This is in contrast with the findings from the REVEAL study. [10] However, studies in some Asian countries [11] show that SLE is more prevalent than SS in PAH. There have been no studies to determine if SS or SLE is more prevalent in the SA population.…”