Mortality and associated factors in patients with systemic sclerosis-associated pulmonary hypertension with and without interstitial lung disease: A long-term follow-up study.

OBJECTIVES

We aimed to investigate mortality and prognostic factors in systemic sclerosis (SSc) patients with pulmonary hypertension (PH) with or without interstitial lung disease (ILD).

METHODS

The associations between mortality and demographics, transthoracic echocardiography, right heart catheterization (RHC), pulmonary functional parameters at baseline, and treatment modalities were evaluated.

RESULTS

Survival rates for PH-SSc patients (42 female, mean age 56.6 ± 13.5, median follow-up 45 months) were 91% at the first year, 75% at 2 years, and 43.1% at 5 years. The majority of the deceased patients had PH + ILD (P = .007). The PH + ILD group had more diffuse skin involvement, anti-Scl-70, high C-reactive protein, low FVC, and lower DLCO. The deceased patients had higher estimated pulmonary arterial systolic pressure (PASP), low cardiac output, and FVC values. Median survival time was significantly better in patients on combined therapy. Mortality-related factors in the PH + ILD group were decreased initial FVC, high estimated PASP, low cardiac output, deteriorated functional class, and monotherapy.

CONCLUSION

This is the first reported SSc-PH cohort from Turkey by a multidisciplinary team. PH is a severe complication of SSc with high mortality especially in patients with accompanying severe ILD.