Accelerated hypertension in systemic sclerosis and scleroderma renal crisis

Accelerated hypertension in systemic sclerosis is defined as an increase in blood pressure (BP) of 30 mmHg or more above a person’s baseline BP. If a person with systemic sclerosis develops accelerated hypertension, it can be associated with acute kidney failure, and together this is known as scleroderma renal crisis. Scleroderma renal crisis is a medical emergency. Refer any patient with accelerated hypertension or suspected scleroderma renal crisis to a specialist centre for urgent management.

Note: Scleroderma renal crisis is a medical emergency. Refer any patient with accelerated hypertension or suspected scleroderma renal crisis to a specialist centre for urgent management.

Commence treatment for accelerated hypertension or suspected scleroderma renal crisis immediately; see Management for scleroderma renal crisis. In contrast to managing essential hypertension in people with systemic sclerosis, angiotensin converting enzyme inhibitors (ACEIs) are the preferred antihypertensives for treating accelerated hypertension or scleroderma renal crisisDenton, 2017Kowal-Bielecka, 2017, especially captopril for its short duration and ease of titration.

Note: Angiotensin converting enzyme inhibitors (ACEIs) are the preferred antihypertensives for treating accelerated hypertension or scleroderma renal crisis in people with systemic sclerosis.

Scleroderma renal crisis can develop over a few days and previously was the leading cause of death in patients with diffuse systemic sclerosis. Acute management for accelerated hypertension using angiotensin converting enzyme inhibitors (ACEIs) has reduced mortality.

Scleroderma renal crisis usually occurs in the first 2 years of diagnosis of systemic sclerosis and may be the presenting symptom of the disease. People with diffuse systemic sclerosis, especially with positive RNA polymerase III autoantibodies (see Investigations for systemic sclerosis) and use of high-dose systemic corticosteroids, are at high risk for the development of scleroderma renal crisis; avoid prednisolone (or prednisone) doses of more than 10 mg daily in patients with systemic sclerosis.

Note: Avoid prednisolone (or prednisone) doses of more than 10 mg daily in patients with systemic sclerosis because it can precipitate scleroderma renal crisis.