Scleroderma Renal Crisis

 Q: Which of the following is NOT a part of the classic scleroderma renal crisis (SRC)? (select one)? 

A) Abrupt malignant hypertension 

B) Acute oliguric renal failure 

C) Severe proteinuria 

D) Microangiopathic hemolysis anemia 

E) Thrombocytopenia 

 Answer: C 

SRC is one of the most dreaded complications of scleroderma Although some level of kidney involvement is a norm in scleroderma, it rarely progresses to chronic renal failure. SRC may occur in 10-15 percent of cases. What makes it scarier is that it usually occurs early in the course and can catch physicians as well as patients off guard. One clinical predictor is the patient with diffuse cutaneous Systemic Sclerosis (dcSSc). Patients with limited cutaneous systemic sclerosis (lcSSc) are less inclined to develop this. It has the following classic clinical characteristics:

• Abrupt onset of high/malignant hypertension. (some reports of normal blood pressure)
• Acute oliguric renal failure 
• Urinalysis with only mild proteinuria with few cells or casts 
• Microangiopathic hemolytic anemia 
• Thrombocytopenia 

The above pathologies lead to the possible symptoms of shortness of breath from pulmonary edema, headache, blurred vision, retinal microhemorrhages, hypertensive encephalopathy, and seizures. 

Please see reference #3 for recent updates on SRC's management.

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References:

1. Traub YM, Shapiro AP, Rodnan GP, et al. Hypertension and renal failure (scleroderma renal crisis) in progressive systemic sclerosis. Review of a 25-year experience with 68 cases. Medicine (Baltimore) 1983; 62:335. 

2. Shanmugam VK, Steen VD. Renal disease in scleroderma: an update on evaluation, risk stratification, pathogenesis and management. Curr Opin Rheumatol 2012; 24:669.

3. Nagaraja V. (2019). Management of scleroderma renal crisis. Current opinion in rheumatology, 31(3), 223–230. https://doi.org/10.1097/BOR.0000000000000604