Scleroderma Renal Crisis

Posted by • September 30th, 2011

In the latest Case Record of the Massachusetts General Hospital, a 62-year-old woman with autoimmune overlap syndrome and polymyositis presented with anemia, thrombocytopenia, and acute renal failure. Four days before admission, she was found on the floor of her home, confused and minimally conversant.                               

Approximately 70% of patients with diffuse cutaneous systemic sclerosis have kidney involvement, even in the absence of renal crisis.    

Clinical Pearls

 How does systemic sclerosis typically present?               

Systemic sclerosis or scleroderma has a female preponderance and typically presents in patients who are between 30 and 60 years of age. It is associated with sclerodactyly, Raynaud’s phenomenon, telangiectasia, xerostomia, sicca syndrome, and dysphagia.        

 How does scleroderma renal crisis typically present?   

Scleroderma renal crisis typically presents with acute kidney injury in combination with worsening symptoms of scleroderma, and new-onset hypertension. Investigations often reveal a bland urine sediment, and evidence of microangiopathic hemolytic anemia.             

Morning Report Questions

Q: What are the early warning signs for scleroderma renal crisis?              

A: Early warning signs include the onset of the diffuse-skin-disease subtype of scleroderma within the past 4 years, rapidly progressive skin disease, and the presence of RNA-polymerase antibodies. Recent intensification of glucocorticoid therapy (to doses of prednisone >15 mg daily) can also predispose to the onset of scleroderma renal crisis.    

Q: What is the outcome of patients with scleroderma renal crisis?           

A: Renal crisis is a dangerous development in a patient with scleroderma. In the era before inhibitors of the renin-angiotensin-aldosterone system, the 1-year mortality rate approached 100%. More recent series show early survival rates of 70 to 80%.

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