Figure 4.

Survival by Kaplan-Meier analysis. Within a follow-up period of 2.9 ± 1.9 years (25 ± 13 months), two patients died due to pulmonary hypertension (PH), one died due to carcinoma, and one patient underwent lung transplantation. The dashed line indicates mortality due to connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH), the straight line indicates overall survival, and the dotted line indicates transplantation-free survival. The Kaplan-Meier PAH-survival rate was 100% after 1, and 2 years, and 80% after 3 years. The overall survival rate was 100% after 1, and 2 years, and 73% after 3 years. The transplantation-free survival rate was 95% after 1 and 2 years.