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 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
- spotted nephrogram: manifested as diffuse, spotty lucencies throughout renal parenchyma due to small patchy areas of focal ischemia secondary to arteriolar severe narrowing and thrombosis without abnormalities of the large caliber renal arteries whereas, in malignant nephrosclerosis, major arteries are shown to be dilated due to chronicity of process
- 1. Harrison's rheumatology. McGraw-Hill Professional. ISBN:0071457437. Read it at Google Books - Find it at Amazon
- 2. Winograd J, Schimmel DH, Palubinskas AJ. The spotted nephrogram of renal scleroderma. AJR Am J Roentgenol. 1976;126 (4): 734-8. doi:10.2214/ajr.126.4.734 - Pubmed citation
- 3. Kumar V, Abbas AK, Aster JC et-al. Robbins and Cotran pathologic basis of disease. Saunders. ISBN:1416031219. Read it at Google Books - Find it at Amazon
- 4. Denton CP, Lapadula G, Mouthon L et-al. Renal complications and scleroderma renal crisis. Rheumatology (Oxford). 2009;48 Suppl 3 (suppl 3): iii32-5. doi:10.1093/rheumatology/ken483 - Pubmed citation
- 5. Steen VD, Syzd A, Johnson JP et-al. Kidney disease other than renal crisis in patients with diffuse scleroderma. J. Rheumatol. 2005;32 (4): 649-55. Pubmed citation