Shiri Keret1, Yolanda Braun-Moscovici2,3, Anna Solomonov3,4, Doron Markovits2, Sami Giryes2, Kohava Toledano2,3, Yonit Tavor2, Katya Dolnikov2, Alexander Rozin2,3, Elite Vainer Evgrafov4, Hanna Dawood4, Luda Guralnik3,5, Alexandra Balbir-Gurman2,3*
Objective: To assess the morbidity, mortality and prognostic factors of patients with Systemic Sclerosis (SSc) and Interstitial Lung Disease (ILD).
Methods: A retrospective analysis of prospectively collected data of SSc patients between the years 2000-2020. Data collection included demographic and clinical parameters, with repeated assessments of Forced Vital Capacity (FVC), Diffusing Capacity of the Lungs for Carbon Monoxide (DLCO), and Pulmonary Artery Pressure (PAP) in SSc-ILD patients.
Results: Among 446 SSc patients [367 (82.2%) female, mean age 46.5 years, 175 (39.2%) diffuse SSc [DcSSc]], 141 (31.6%) had SSc-ILD, 121 (27.1%) deceased, of which 74 (61.6%) patients died from SSc-related complication. SSc-ILD was associated with male sex, Arab descent, DcSSc, anti-topoisomerase antibodies, myopathy, and Pulmonary Hypertension (PAH). Mortality in SSc-ILD was associated with older age, Arab descent, and PAH. Elevated PAP estimated by echocardiography at the time of SSc-ILD diagnosis, and rapid decline of FVC or DLCO, correlated with mortality. Five-year survival rates in SSc and SSc-ILD patients were constant.
Conclusion: Arab descent is an important prognostic factor for ILD and mortality. In SSc-ILD, early elevation of PAP and rapid decline in FVC or DLCO indicated a poor prognosis. SSc- related mortality is still high, and has remained stable in recent decades.