How is a scleroderma renal crisis treated?

Updated: Jun 24, 2020
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Patients with diffuse, rapidly progressive skin involvement have the highest risk of developing scleroderma renal crisis. Renal crisis occurs in about 10% of all patients with systemic sclerosis.

Renal crisis is observed within 4 years of diagnosis in about 75% of patients but may develop as late as 20 years after diagnosis. Renal crises are slightly more common in blacks than in whites, and men have a greater risk than women. The presence of RNA polymerase III antibodies increases the risk for renal crisis.

Scleroderma renal crisis that is not treated promptly and aggressively invariably leads to renal failure requiring dialysis or renal transplantation, or even death. Consequently, it is critical to check blood pressure, monitor serum creatinine, and start angiotensin-converting enzyme (ACE) inhibitors at the earliest signs of hypertension in at-risk patients.

High doses of corticosteroids should be avoided in patients with systemic sclerosis owing to an increased risk of developing renal crisis.

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