Table 1 Provisional classification criteria for the classification of Juvenile Systemic Sclerosis [8].
Major criterion (Required) | |
|---|---|
Proximal (to MCP/MTP joints) skin sclerosis/induration of the skin | |
Minor criteria (At least two required) | |
Cutaneous | Sclerodactyly |
Peripheral vascular | Raynaud's phenomenon |
Nailfold capillary abnormalities | |
Digital tip ulcers | |
Gastrointestinal | Dysphagia |
Gastro-esophageal reflux | |
Cardiac | Arrhythmias |
Heart failure | |
Renal | Renal crisis |
New onset arterial hypertension | |
Respiratory | Pulmonary fibrosis (HRCT/radiograph) |
Decreased DLCO | |
Pulmonary arterial hypertension | |
Neurologic | Neuropathy |
Carpal tunnel syndrome | |
Musculoskeletal | Tendon friction rubs |
Arthritis | |
Myositis | |
Serologic | Antinuclear antibodies |
SSc selective autoantibodies | |
(anticentromere, anti-topoisomerase I (Scl 70), anti-fibrillarin, anti-PM-Scl, anti-fibrillin or anti-RNA polymerase I or III) | |