The natural course of SSc-ILD is highly variable. Baseline serum KL-6 is a biomarker potentially useful for predicting FVC decline.

The major PAH-CTD population in Japan suffers from MCTD or SLE with anti-U1RNP antibody, in contrast to PAH-CTD patients in the USA and Europe. Modern PAH treatment has improved survival rates, but long-term outcomes are still unsatisfactory. Independent predictors of mortality indicate that early diagnosis and the prompt use of PAH drugs should improve survival.

No abstract