From: French recommendations for the management of systemic sclerosis
(a) Hypertensive forms | |
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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 | |
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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) |