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The major renal manifestation of systemic sclerosis is scleroderma renal crisis (SRC). This condition is characterized by accelerated phase hypertension and acute kidney injury (AKI). The management of renal crisis was revolutionized by the introduction of angiotensin-converting enzyme (ACE) inhibitors more than 30 years ago, although in the current era, there is still a significant proportion of patients who have poor outcomes. Recognizing patients at high risk is a key part of the management of SRC. In particular, patients with early diffuse skin involvement from scleroderma and those who have the anti-RNA polymerase III autoantibody are at significantly increased risk for renal crisis. In these patient groups, we recommend avoidance of significant corticosteroid doses and regular home blood pressure monitoring. These measures should reduce the incidence of SRC and ensure its early diagnosis when it does appear. There is no role for ACE inhibitor prophylaxis of SRC. Patients with SRC should be hospitalized and receive careful supportive care. The condition presents with renal, hematological, and cardiac complications in the context of a complex multisystem rheumatological disease, so good inter-disciplinary care is a key goal to improve outcomes. The specific treatment for SRC remains ACE inhibitors, which should be titrated to the highest tolerated dose and continued indefinitely in all patients, regardless of renal recovery or establishment on dialysis. Other antihypertensives can be used in addition to achieve optimal blood pressure control. Patients with SRC can recover renal function after more than 2 years on dialysis, so renal transplantation should not be undertaken during this early period. |
