Description

Penn et al listed criteria for the diagnosis of scleroderma renal crisis. The patients are from the Royal Free Hospital in Hampstead, University College London and Royal Sussex County Hospital in Brighton.


 

Criteria for scleroderma renal crisis:

(1) presence of diffuse or limited cutaneous systemic sclerosis

(2) new onset of significant hypertension with a blood pressure > 150/85 mm Hg measured at least twice within a 24 hour period.

(3) a decreased in renal function defined as a >= 30% decrease in the calculated glomerular filtration rate (GFR)

 

Supportive evidence for the renal crisis - one or more of the following:

(1) microangiopathic hemolytic anemia

(2) retinal changes of acute hypertensive crisis

(3) new onset of microscopic hematuria with urinary RBCs not explained by other causes

(4) flash pulmonary edema

(5) oliguria or anuria

(6) characteristic changes in a renal biopsy

 

where:

• The cutoff for blood pressure is from the New York Heart Association (NYHA).

• Ideally the calculated GFR should be repeated with a creatinine from a second specimen.

• The mean blood pressure for 150/85 is 107.

 

Criteria for exclusion:

(1) identification of an alternative diagnosis on renal biopsy

(2) another diagnosis that explain the clinical and laboratory findings better

 


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