A 56‐year‐old physician with vitiligo and hypothyroidism presented to the emergency department with malaise, intermittent fevers and flulike symptoms of 1 month's duration. She had taken ibuprofen for 10 days and self‐prescribed dexamethasone for pain relief. She denied miscarriages, GI dysmotility, Raynaud's, oral ulcers, or hemoptysis. She had no family history of kidney, liver, or collagen vascular disease. On admission, she was afebrile, with a blood pressure of 160/70 mm Hg. Her exam was notable for chronic hypopigmented lesions on herfaceand neck and a new dependent petechial rash. Hercreatinine was 8.9, up from a baseline 2 weeks earlier of 1.0. Urinalysisdid not reveal dysmorphic red blood cells or casts. Her erythrocyte sedimentation rate was more than 100, and ANA was 1:640 speckled. She was positive for anti‐Scl‐70 and negative for anticentromeres. Renal biopsy revealed arcuate artery thrombosis with intimal thickening and recanalization without active vasculitis; immunofluorescence was negative, and a trichrome stain revealed interstitial fibrosis. Without sclerodermatous skin changes and with recent steroid use, an identified precipitant, our patient was diagnosed with systemic sclerosis sine scleroderma renal crisis.
Systemic sclerosis (SSc) is a connective tissue disorder characterized by excessive collagen deposition. SSc is categorized as diffuse cutaneous (dcSSc), limited cutaneous (lcSSc), based the extent of skin thickening, and systemic sclerosis sine scleroderma (ssSSc), organ fibrosis without cutaneous involvement. Aside from skin findings, ssSSc and lcSSc are similar; patients are typically 45‐ to 55‐year‐old women with Raynaud's or GI involvement, a positive ANA, and an SSc‐associated autoantibody.
Scleroderma renal crisis (SRC) is new accelerated hypertension or rapidly progressive renal failure in the setting of SSc. SRC occurs in 10%‐25% of those with SSc and is diagnosed by a biopsy that demonstrates intimal thickening of interlobular arteries, fibrosis, and few mono‐nuclear cells. Early rapidly progressive dcSSc carries the greatest risk, but rare cases with lc/ssSSc have been reported.
Corticosteroids are known precipitants because of their decreased prostacyclin and increased ACE activity. The treatment of choice for SRC is ACE inhibitors with dialysis as indicated. Without ACE inhibitors, the 1‐year survival is 10%, but with ACE inhibitors, 70% of patients survive 5 years. More than 50% of those who initially require dialysis are ultimately able to discontinue dialysis.
SRC is very rare in the setting of ssSSc but must be considered in a patient with new steroid use (prednisone > 15 mg/day) who is in acute renal failure. Ultimately, the diagnosis rests on serology and renal biopsy. Intimal proliferation of renal arteries with endothelial dysfunction results in a relentless cycle of ischemia, hyperreninemia, hypertension, and further vasoconstriction. An optimal outcome requires early recognition and early ACE inhibitor therapy.
P. Natarajan, Artery Therapeutics, receipt of royalties; L. Mazotti, None; M. Dandu, None.
To cite this abstract:Natarajan P, Mazotti L, Dandu M. A Total Crisis? Steroid Self‐Prescription and an Unusual Cause of Acute Renal Failure. Abstract published at Hospital Medicine 2007, May 23-25, Dallas, Texas Abstract 141. Journal of Hospital Medicine. 2007; 2 (suppl 2). https://www.shmabstracts.com/abstract/a-total-crisis-steroid-selfprescription-and-an-unusual-cause-of-acute-renal-failure/. Accessed September 22, 2019.