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Long-Term Outcomes of Scleroderma Renal Crisis

Virginia D. Steen, MD; and Thomas A. Medsger Jr., MD
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Copyright ©2004 by the American College of Physicians


Ann Intern Med. 2000;133(8):600-603. doi:10.7326/0003-4819-133-8-200010170-00010
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Background: Although scleroderma renal crisis, a complication of systemic sclerosis, can be treated with angiotensin-converting enzyme (ACE) inhibitors, its long-term outcomes are not known.

Objective: To determine outcomes, natural history, and risk factors in patients with systemic sclerosis and scleroderma renal crisis.

Design: Prospective observational cohort study.

Setting: University program specializing in scleroderma.

Patients: 145 patients with scleroderma renal crisis who received ACE inhibitors and 662 patients with scleroderma who did not have renal crisis.

Measurements: Among patients with renal crisis, the four outcomes studied were no dialysis, temporary dialysis, permanent dialysis, and early death. Demographic, clinical, and laboratory data were compared to identify risk factors for specific outcomes. Follow-up was 5 to 10 years.

Results: 61% of patients with renal crisis had good outcomes (55 received no dialysis, and 34 received temporary dialysis); only 4 of these (4%) progressed to chronic renal failure and permanent dialysis. More than half of the patients who initially required dialysis could discontinue it 3 to 18 months later. Survival of patients in the good outcome group was similar to that of patients with diffuse scleroderma who did not have renal crisis. Some patients (39%) had bad outcomes (permanent dialysis or early death).

Conclusions: Renal crisis can be effectively managed when hypertension is aggressively controlled with ACE inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hopes of discontinuing dialysis.

Figures

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Figure 1.
Relation of serum creatinine concentration to time of renal crisis in 145 patients with systemic sclerosis.nnnn

The solid line indicates those who did not receive dialysis ( = 51), the dotted line indicates those who received permanent dialysis ( = 25), the dashed line indicates those who died early ( = 23), and the dashed and dotted line indicates those who received temporary dialysis ( = 34). Patients who did not have a late follow-up measurement of creatinine were excluded. To convert mg/dL to µmol/L, multiply by 88.4.

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Figure 2.
Cumulative survival rate for patients with different outcomes after scleroderma renal crisis and patients with diffuse scleroderma who did not have renal crisis.nnnnn

The solid line indicates those who did not receive dialysis ( = 55), the dotted line indicates those who received permanent dialysis ( = 28), the small dashed line indicates those who died early ( = 28), the dashed and dotted line indicates those who received temporary dialysis ( = 34), and the large dashed line indicates those who did not have renal crisis ( = 662).

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Long-Term Outcomes of Scleroderma Renal Crisis

Copyright ©2004 by the American College of Physicians

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