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 abrupt onset of hypertension, acute renal failure 4.
For a general discussion of scleroderma, please refer to the parent article: scleroderma.
The pathophysiology is very similar to malignant nephrosclerosis and is characterised by pronounced constriction of arcuate and interlobular arterioles resulting in focal cortical ischaemia. 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
- spotted nephrogram: manifested as diffuse, spotty lucencies throughout renal parenchyma due to small patchy areas of focal ischaemia secondary to arteriolar severe narrowing and thrombosis without abnormalities of the large calibre renal arteries whereas, in malignant nephrosclerosis, major arteries are shown to be dilated due to chronicity of process
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