Late-Onset Scleroderma Renal Crisis Does Occur-A Multicenter Study

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

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

A total of 223 patients with SRC were identified, with 169 (75.8%) classified as early onset and 54 (24.2%) as late onset. The late-onset group had a mean SRC onset of 12.2 years after SSc diagnosis. Male predominance was observed in the late-onset compared with the early-onset 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.

Late-onset SRC was seen in up to 25% of patients with SRC, 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.