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

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Summary
This summary is machine-generated.

Late onset scleroderma renal crisis (SRC), occurring over 5 years after diagnosis, affects up to 25% of patients. This late-onset SRC shares similar clinical features and outcomes with early-onset SRC, emphasizing the need for vigilance regardless of disease duration.

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Area of Science:

  • Rheumatology
  • Nephrology
  • Systemic Sclerosis

Background:

  • Scleroderma renal crisis (SRC) is traditionally associated with early systemic sclerosis (SSc) diagnosis.
  • However, SRC can manifest years after the initial SSc diagnosis, necessitating further investigation.

Purpose of the Study:

  • To determine the prevalence, clinical characteristics, and outcomes of late-onset SRC.
  • To compare these findings with those of early-onset SRC.

Main Methods:

  • Retrospective observational study of 223 SRC patients diagnosed between 1995-2018.
  • Late onset SRC defined as occurring >5 years post-SSc diagnosis.
  • Data extracted on demographics, onset, clinical features, labs, and outcomes; analyzed using Pearson's chi-square.

Main Results:

  • 24.2% of SRC patients (54/223) had late-onset SRC, with a mean onset 12.2 years after SSc diagnosis.
  • Late-onset SRC showed male predominance and higher rates of prior steroid exposure compared to early-onset SRC.
  • No significant differences were observed in autoantibody profiles or patient outcomes between early and late-onset groups.

Conclusions:

  • Late-onset SRC represents a significant proportion of cases, occurring a mean of 12 years post-SSc diagnosis.
  • Clinical characteristics and outcomes are comparable between early and late-onset SRC.
  • Recognizing SRC irrespective of disease duration is crucial for optimizing patient outcomes.