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Abstract: SA-PO385

Systemic Sclerosis Medications and the Prevention of Scleroderma Renal Crisis

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials


  • Gordon, Sarah M., TAMC, Kaneohe, Hawaii, United States
  • Nee, Robert, Walter Reed National Military Medical Center, Vienna, Virginia, United States
  • Olson, Stephen W., Walter Reed National Military Medical Center, Bethesda, Maryland, United States

Scleroderma Renal Crisis (SRC) develops abruptly in systemic sclerosis (SSc) and is associated with significant morbidity and mortality. High risk SSc subjects may benefit from treatment to prevent SRC. Though angiotensin converting enzyme inhibitors (ACEi) are the standard of care for SRC at diagnosis, previous case series found no benefit for initiation at SSc diagnosis to prevent SRC and a potential paradoxical association with development of SRC. There are no previous cohort studies of the potential prophylactic benefit for calcium channel blockers (CCB), angiotensin receptor blockers (ARB), endothelin receptor blockers (ERB), non-steroidal anti-inflammatory drugs (NSAID), or mycophenolate mofetil (MMF) to prevent SRC.


: In this retrospective cohort study of the entire military electronic medical record between 2005 and 2016, we compared the use of ACEi, ARB, CCB, NSAID, ERB and MMF after SSc diagnosis for 31 cases who subsequently developed SRC to 322 SSc without SRC disease controls. We conducted logistic regression analyses to evaluate for medications independently associated with SRC.


Baseline ACEi use was more common in SSc patients that subsequently developed SRC [77% (24/31) vs. 33% (108/322), p<0.001]. ACEi was associated with an increased risk for SRC adjusted for age, race, gender, and prednisone use [odds ratio (OR) 4.0, 95% confidence interval (CI) 1.6-10.3, P=0.003]. On stratified analyses, there was no association between ACEi and SRC in the absence of proteinuria, while in the presence of proteinuria, ACEi was significantly associated with SRC [OR 5.28, 95% CI 1.1-29.2, p=0.03]. In addition, a doubling of ACEi dose [61% (19/31) vs. 12% (38/322), p<0.001) and achieving maximum ACEi dose [45% (14/31) vs. 4% (14/322), p<0.001] after SSc diagnosis was associated with future SRC. CCB, ARB, NSAIDs, ERB, and MMF use were not significantly associated with SRC.


Medications commonly used in SSC were not associated with a reduced risk of SRC. Paradoxically, ACEi use at SSC diagnosis was associated with an increased risk of SRC. This relationship was observed only in those with a heightened risk for SRC from proteinuria, suggesting that it may be a passive marker of evolving subclinical disease. SSC patients that require ACEi should be more closely monitored for SRC.


  • Other U.S. Government Support