18th January 2024

Scleroderma Renal Crisis

Scleroderma renal crisis is a life-threatening complication, presenting with sudden-onset malignant hypertension and severe progressive renal failure. Other manifestations include hypertensive encephalopathy, congestive cardiac failure and microangiopathic haemolytic anaemia.

It most commonly presents in patients with the diffuse cutaneous sub-type; it is very rare in those with limited cutaneous disease. It usually occurs early in the disease course and can occasionally be the presenting feature.

Pathophysiology

Blood vessels undergo structural changes (thickening and proliferation of the intima with fibrin deposition), which causes reduced blood flow, further exacerbated by vasospasm. When this occurs in the kidneys, renal ischaemia develops, causing activation of the renin-angiotensin-aldosterone system (RAAS) and subsequent rise in blood pressure. The hypertension causes even further damage to renal blood vessels, and this cycle of events eventually results in malignant hypertension and fulminant renal failure.

Management

Since activation of the RAAS is the basis of the pathogenesis of the scleroderma renal crisis, management is based on inhibition of this pathway using Angiotensin-Converting Enzyme (ACE) inhibitors. Patients should be admitted for blood pressure control and careful monitoring of renal function. Despite the potential to further worsen the renal function, ACE inhibitors are an essential part of the treatment of scleroderma renal crises. In some cases, dialysis may be needed, but renal recovery can occur up to 24 months after the crisis and decisions about transplantation should be delayed until this point.