Background/Purpose: Scleroderma renal crisis (SRC) is an infrequent but life-threatening complication of systemic sclerosis (SSc). The outcome of SRC has improved considerably since the advent of angiotensin converting enzyme (ACE) inhibitors. The incidence of SRC has also appeared to have decreased, perhaps in part due to the more liberal use of ACE inhibitors in SSc. However, recent retrospective data suggests that patients with SRC exposed to ACE inhibitors prior to the onset of SRC may have worse outcomes. We undertook a prospective study to verify whether SSc patients with incident SRC on ACE inhibitors at the time of onset of SRC had worse outcomes compared to those who were not on these drugs at that time.
Methods: We designed a prospective, observational cohort study of incident SRC subjects identified through a web-based survey. Every second week, an e-mail was sent to 589 participating physicians from around the world to identify incident cases of SRC. Data on patient demographic and disease characteristics, as well as exposure to ACE inhibitors was collected. A one-year follow-up case report form was sent to all the physicians who identified a case. The primary outcome of interest was death or dialysis at one year after the onset of SRC, comparing patients exposed and unexposed to ACE inhibitors at the time of onset of SRC.
Results: We identified 88 incident cases of SRC, of which 12 were lost to follow up (86% follow up rate). Mean age was 52 years, 67% were women, 76% had diffuse SSc and median disease duration since the onset of the first non-Raynaud’s symptom was 1.5 years. The majority of cases had a hypertensive SRC (n=71/76) and only 5 had a normotensive SRC. Eighteen patients (24%) were on an ACE inhibitor immediately prior to the onset of the SRC. At one year follow up, 27 (36%) SRC patients had died and an additional 13 (17%) remained on dialysis.
The crude one-year cumulative incidence of death in those exposed to ACE inhibitors at the time of onset of SRC compared to the unexposed was 1.56 (95% confidence interval [CI] 0.70-3.47) and the crude one-year cumulative incidence of dialysis was 0.61 (95% CI 0.18-2.09). The crude Cox proportional hazard ratio comparing the time to death of SRC patients exposed to ACE inhibitors prior to the onset of SRC to those unexposed was 1.95 (95% CI 0.87-4.35). After controlling for differences in prednisone exposure and history of systemic hypertension in the two groups, the adjusted Cox proportional hazard ratio comparing the time to death of SRC patients exposed to ACE inhibitors prior to the onset of SRC to those unexposed was 2.52 (95% CI 1.05-6.05, p=0.0394).
Conclusion: SRC was associated with poor one-year outcomes. Exposure to an ACE inhibitor prior to the onset of SRC was associated with an increased risk of death during the first year of follow up after SRC. Clinicians caring for patients with early SSc should use ACE inhibitors cautiously.
Disclosure:
M. Hudson,
None;
M. Baron,
None;
S. Tatibouet,
None;
D. Furst,
Amgen, Janssen, Roche, and UCB,
2,
Amgen, Janssen, Roche, and UCB,
5;
D. Khanna,
Actelion, BMS, Gilead, Genentech, ISDIN, and United Therapeutics,
2,
Actelion, BMS, Gilead, Genentech, ISDIN, and United Therapeutics,
5,
Actelion, BMS, Gilead, Genentech, ISDIN, and United Therapeutics,
8;
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/does-the-use-of-angiotensin-converting-enzyme-inhibitors-prior-to-scleroderma-renal-crisis-affect-prognosis-results-of-the-international-scleroderma-renal-crisis-survey/