Late-Onset Scleroderma Renal Crisis Does Occur - A Multi-Center Study.

Abstract

Objective: Scleroderma renal crisis (SRC) is historically described to occur within first 5 years of SSc diagnosis. However, it has been observed to occur beyond 5 years. In this analysis, we aim to describe the prevalence, clinical features and outcomes of late onset SRC and compare them to early onset SRC.

Methods: This retrospective observational study included patients diagnosed with SRC between 1995 to 2018 at three university-affiliated hospitals. Late onset SRC was defined as SRC occurring 5 years after SSc diagnosis. After obtaining IRB approval, data including demographics, SRC onset, clinical characteristics, laboratory data, and outcomes were extracted. Continuous data were expressed as mean/median, and categorical as frequencies/percentages. Differences were analyzed via Pearson's chi-square.

Results: A total of 223 SRC patients were identified, with 169 (75.8%) classified as early onset and 54 (24.2%) as late onset. Late onset group had a mean SRC onset at 12.2 years after SSc diagnosis. Male predominance was observed in late compared to early group (59% vs 9%). Steroid exposure was more common in late vs early group (56% vs 29%). There was no evidence of difference in autoantibodies profile and outcomes between early and late onset groups.

Conclusions: Late onset SRC was seen in up to 25% of SRC patients, with a mean of 12 years after SSc diagnosis. Our findings reveal comparable clinical characteristics and outcomes between early and late onset SRC, underlying the importance of recognizing SRC regardless of disease duration to optimize outcomes.