Background/Purpose: Scleroderma renal crisis (SRC) is a rare complication of patients with systemic sclerosis (SSc). ACE inhibitors (ACEI) favorably alter the natural history of this condition. However, clinicians are wary of initiating this therapy because of a rising serum creatinine. There is paucity of data on the association between the time of initiation of therapy and long-term outcomes. We evaluated the effect and timing of treatment on long term outcomes in patients with SRC.

Methods: We screened 393 adult patients with SSc (fulfilling 2013 ACR/EULAR criteria) in our institution from 2004 to 2/2018 of which 25 satisfied the diagnosis of SRC, as defined by Steen et al¹. Data on patient demographics, clinical presentation, serology, time to initiation of therapy, and type of therapy were evaluated. We evaluated the following outcomes: mortality, need for dialysis and partial or complete renal recovery. Follow up was censored on May 11^th, 2018. Continuous variables were expressed as mean ± SD. Discrete variables were expressed as mean (%) and compared with chi-squared tests. Kaplan-Meier curves were used to describe long term survival estimates. A two-tailed p-value of less than 0.05 was considered significant for all statistical testing.

Results: Our cohort included 25 patients with SRC of which one patient was normotensive; 63% were females and mean ages at diagnoses of SSc and SRC were 54 ± 14.1 years and 58 ± 11.2 years respectively; 96% of the patients were treated with ACEI including 16% who were already on one. Baseline characteristics, clinical features, and disease course of patients with SRC are shown in Table 1. At one year, mortality was 41.2%; 52% (13/25) required dialysis.  Of which one patient was able to discontinue dialysis after 25 days. Dialysis requirement was significantly higher in those who were started on ACEI >48 hours after onset of clinical manifestations (78.6% vs 16.7%, p= 0.018). Kaplan-Meier survival estimates at 5 years follow up are shown in Figure 1.

Conclusion: Early (≤ 48 hours) initiation of ACEI in patients with SRC significantly reduces need for dialysis. However, irrespective of the timing of initiation of therapy, the long-term mortality of patients with SRC remains high.

Reference:

1.      Steen VD, Medsger TA. Long-Term Outcomes of Scleroderma Renal Crisis. Ann Intern Med. 2000;133:600–603. 

                                   Figure 1: Kaplan-Meier survival estimates at 5 years follow up