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Table 9 Criteria for the classification of the hypertensive form of scleroderma renal crisis

From: French recommendations for the management of systemic sclerosis

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, without other explanation than the SSc
b. Acute renal injury, without other explanation than the SSc AKI according to the KDIGO classification: more than 50% increase of serous 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
Target organ dysfunction
 Hypertensive retinopathy
 Hypertensive encephalopathy
 Pulmonary edema
 Acute pericarditis
Anomalies suggestive of renal biopsy (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