Clinical severity scoring system
| Clinical manifestations . | Scoring . |
|---|---|
| Severe painful crisis defined as acute painful event requiring hospital admission and morphine administration | Less than 1 per year: 0 points 1 to 2 per year: 1 point 3 to 5 per year: 2 points More than 5 per year: 3 points |
| Central nervous system damage documented by MRI or MRA (and conventional arteriography) | No lesion: 0 point Diagnosis of overt stroke, silent stroke, or cerebral vasculopathy: 2 points |
| Proliferative retinopathy | No lesion: 0 points Current or previous lesion: 1 point |
| Leg ulceration | No lesion: 0 points Current or previous history of leg ulceration: 2 points |
| Aseptic necrosis of bones documented by x-ray photography and/or MRI | No bone lesion: 0 points Aseptic necrosis: 2 points per lesion |
| Renal hemorrhage secondary to papillary necrosis | No episode: 0 points History of renal hemorrhage: 1 point |
| ACS diagnosed on the basis of the association of thoracic pains, dyspnea, and pulmonary infiltrates on x-ray photography | No episode: 0 points History of documented ACS: 2 points per episode |
| Priapism | No history of priapism: 0 points History of stuttering or acute priapism: 1 point |
| Clinical manifestations . | Scoring . |
|---|---|
| Severe painful crisis defined as acute painful event requiring hospital admission and morphine administration | Less than 1 per year: 0 points 1 to 2 per year: 1 point 3 to 5 per year: 2 points More than 5 per year: 3 points |
| Central nervous system damage documented by MRI or MRA (and conventional arteriography) | No lesion: 0 point Diagnosis of overt stroke, silent stroke, or cerebral vasculopathy: 2 points |
| Proliferative retinopathy | No lesion: 0 points Current or previous lesion: 1 point |
| Leg ulceration | No lesion: 0 points Current or previous history of leg ulceration: 2 points |
| Aseptic necrosis of bones documented by x-ray photography and/or MRI | No bone lesion: 0 points Aseptic necrosis: 2 points per lesion |
| Renal hemorrhage secondary to papillary necrosis | No episode: 0 points History of renal hemorrhage: 1 point |
| ACS diagnosed on the basis of the association of thoracic pains, dyspnea, and pulmonary infiltrates on x-ray photography | No episode: 0 points History of documented ACS: 2 points per episode |
| Priapism | No history of priapism: 0 points History of stuttering or acute priapism: 1 point |
Table was inspired by the model of Hebbel et al.20
MRI indicates magnetic resonance imaging; MRA, magnetic resonance angiography; ACS, acute chest syndrome.