Scleroderma (renal manifestations)

Renal manifestations of scleroderma are common, affecting up to 25% of patients. Some patients (5-10%) can present with a scleroderma renal crisis (SRC). These patients have an abrupt onset of hypertension and acute renal failure .

For a general discussion of scleroderma, please refer to the parent article: scleroderma.

Pathology

The pathophysiology is very similar to malignant nephrosclerosis and is characterized by pronounced constriction of arcuate and interlobular arterioles resulting in focal cortical ischemia. However, in scleroderma, this is a primary process that subsequently produces hypertension.

Histopathologic changes of small arcuate and interlobular arterioles are:

  • fibrinoid necrosis 
  • hyperplastic fibroblastic arteriolitis
  • glomerular sclerosis

Radiographic features

Spotted nephrogram: manifested as diffuse, spotty lucencies throughout renal parenchyma.

  • due to small patchy areas of focal ischemia secondary to severe arteriolar narrowing and thrombosis without abnormalities of the large caliber renal arteries
  • contrast with malignant nephrosclerosis in which the major arteries are dilated due to chronicity of the process

See also