Clinical characteristics, predictors and prognosis of systemic sclerosis with scleroderma renal crisis and pulmonary arterial hypertension.

OBJECTIVES

To characterise the clinical features, predictors, and prognosis of patients with systemic sclerosis (SSc) who had both scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH).

METHODS

In this retrospective cohort study, we reviewed 234 patients diagnosed with SSc at our institute between January 2013 and April 2024. Clinical characteristics and prognosis were compared among patients with both SRC and PAH, PAH alone, SRC alone, and neither PAH nor SRC. Predictors of the coexistence of PAH and SRC were identified using multivariate logistic regression analysis.

RESULTS

Among the 234 patients with SSc, 17 (7.3%) had both PAH and SRC. PAH preceded SRC in five patients, followed SRC in three, and occurred simultaneously in nine. Compared with patients with SRC alone, those with both SRC and PAH had a higher prevalence of interstitial lung disease, a higher rate of gastrointestinal bleeding (GIB), and lower serum albumin levels. GIB (OR=9.545, 95%CI: 1.679, 54.264; p=0.011) was independently associated with the coexistence of PAH and SRC compared with SRC alone, whereas older age at disease onset (OR=1.200, 95%CI: 1.014, 1.420; p=0.033) and elevated NT-proBNP (OR=1.001, 95%CI: 1.000, 1.001; p=0.030) were independently associated with the coexistence of PAH and SRC compared with PAH alone.

CONCLUSIONS

PAH may precede, follow, or coincide with SRC. Patients with both SRC and PAH had a significantly higher risk of GIB. Older age and elevated NT-proBNP were independent factors associated with the coexistence of SRC and PAH.