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Table 4 Classification criteria for scleroderma renal crisis

From: French recommendations for the management of systemic sclerosis

(a) Hypertensive forms
a. HBP ≥ 140/90 mmHg (or increase of systolic BP ≥ 30 mmHg or diastolic BP ≥ 20 mmHg) obtained on two different measurements with a minimum separation of 5 min, with no explanation other than SSc
b. Acute renal injury, with no explanation other than SSc AKI according to the KDIGO classification: more than 50% increase of serum creatinine from the reference value within the preceding 7 days or an absolute increase of 26.5 µmol/l (≥ 0.3 mg/dl) in 48 h
c. Thrombotic microangiopathy
 New or aggravated anemia without other explanation
 Schizocytes
 Thrombopenia ≤ 100,000/mm3 confirmed on a smear
 Signs of hemolysis: elevated LDH, low haptoglobin, increased reticulocytes
 Negative antiglobulin test
d. Target organ dysfunction
 Hypertensive retinopathy
 Hypertensive encephalopathy
 Pulmonary edema
 Acute pericarditis
e. Anomalies suggestive of renal biopsy (fibrinous thrombi, fibrinoid necrosis, glomerular collapse, onion bulb proliferation in pre-glomerular arterioles and arch arteries)
(b) Normotensive forms
1. Increase of creatinine level > 50% of baseline value
 OR creatinine level ≥ 120% of upper normal laboratory reference value
 AND
2. At least one of the following five criteria:
 a. Proteinuria ≥ 2+ by strip
 b. Hematuria ≥ 2+ by strip or ≥ 10 RBC per field
 c. Thrombopenia < 100,000/mm3
 d. Hemolysis defined by anemia not linked to another cause with:
  (1) Schizocytes or other RBC fragments found on blood smear
  (2) Increase in reticulocyte level
 e. Renal biopsy showing a typical appearance of scleroderma renal crisis (fibrinous thrombi, fibrinoid necrosis, glomerular collapse, onion bulb proliferation in pre-glomerular arterioles and arch arteries)
  1. HBP high blood pressure, BP blood pressure, SSc systemic sclerosis, AKI acute kidney injury, LDH lactate dehydrogenase