Background/Purpose: Scleroderma renal crisis (SRC) is the most acute and life-threatening complication of scleroderma characterized by sudden increase in blood pressure and worsening kidney function. Historically, development of SRC was almost always fatal. With the use of angiotensin-converting enzyme inhibitors (ACEI), one-year survival has improved up to 76%; however, SRC remains a poor prognostic marker for long-term survival. Dialysis dependency after an episode of SRC portends a worse long-term prognosis. Here we report on our 15 years of experience with SRC at our institution with a focus on identifying characteristics that predicted initiation of dialysis or death.

Methods: We undertook a retrospective single-center chart review of patients aged 18-80 years old that developed SRC between the years 2002-2016. Baseline characteristics such as age, gender, race, comorbidities and exposures were noted. Outcome data was collected at time of admission, 6 months and 12 months. Patients with the diagnosis of scleroderma and acute kidney injury for any other reason other than SRC were excluded.

Results: In the 15-year period, we identified 34 patients with an episode of SRC (table 1). Of these patients, 16 developed poor outcome (death or dialysis) during their hospital admission. Exposure to steroids within one month of admission was similar in both groups; however, poor outcome was more common in those with exposure to ≥40mg prednisone (p=0.007). This trend continued at the 6 and 12-month follow-up.

Conclusion: Exposure to steroids is known to be a risk factor for the development of SRC. Use of higher dosages of steroids may also be associated with worse outcomes in patients with SRC.

Table 1. Results are reported as proportions or means ± standard deviation or ranges

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/scleroderma-renal-crisis-the-association-of-high-dose-steroids-and-poor-outcome/