Main clinical and biological manifestations of scleroderma renal crisis from the literature.
| Steen, et al2 | Walker, et al6 | DeMarco, et al5 | Penn, et al8 | Teixeira, et al7 | Guillevin, et al72 | Hudson, et al70 | |
|---|---|---|---|---|---|---|---|
| Type of study | R | R | R | R | R | R | P |
| No. patients | 195 | 16 | 18 | 110 | 50 | 91 | 75 |
| Age, yrs | 50 | 54 | 45 | 51 | 53 | 53 | 52.5 |
| Sex, % males | 25 | 31 | 17 | 21 | 26 | 22 | 33.3 |
| Symptoms < 4 years, % | 76 | 69 | 100 | 66 (< 1 y) | 86 | 79 (< 3 y) | 32 (< 1 y) |
| Diffuse SSc, % | 83 | 100 | 100 | 78 | 86 | 85.7 | 74.7 |
| Antitopoisomerase 1 antibodies, % | 20 | 6 | ND | 17.2 | 32 | 31 | 25.3 |
| Anticentromere antibodies, % | 1 | ND | ND | 1.8 | 0 | 3.4 | 4 |
| Hypertension, % | 90 | 94 | ND | ND | 88 | 85.7 | 93.3 |
| Systolic/diastolic BP, mmHg | 184/108 | 203/113 | 130/76 | 193/114 | 189/111 | 184/107 | ND/ND |
| Pericarditis, % | 53 | ND | ND | ND | 6 | ND | 33.3 |
| Left ventricular insufficiency, % | 25 | 56 | 39 | 31 | 46 | 46 | ND |
| Arrhythmia, % | ND | ND | ND | ND | 18 | ND | ND |
| Seizures, % | 8 | 12 | ND | ND | 10 | ND | ND |
| Hypertensive encephalopathy, % | ND | ND | ND | ND | 34 | 58 | ND |
| Intracerebral hemorrhage, % | ND | ND | ND | ND | 10 | ND | ND |
| Thrombotic microangiopathy, % | 30 | 81 | ND | 59 | 46 | 56 | ND |
| Platelet count < 150,000/mm3, % | 39 | ND | ND | 50 | ND | ND | ND |
| Hematuria, % | 38 | ND | ND | ND | 42* | 42* | ND |
| Proteinuria, % | 63 (> 0.25 g/d) | ND | ND | ND | 53 (> 0.5 g/d) | 68 | ND |
↵* Hematuria documented with dipstick measurement or urinalysis. SSc: systemic sclerosis; BP: blood pressure; ND: not documented; P: prospective; R: retrospective.