Disease Features | Pediatric SLE Patientsa | NCF1 Variant Patient |
---|---|---|
Age of disease onset | Average 12 years | 18 months |
Fever | 35–100% | + |
Pattern | With active disease | Recurrent episodes |
Cutaneous Involvement | 60–90% | + |
Manifestations | Malar rash; photosensitivity; discoid rash; mucosal ulceration | Inflamed linear lesion with atrophic scar |
Alopecia | 10–30% | + |
Arthritis | 60–90% | – |
Neuropsychiatric involvement | 15–95% | + |
Manifestations | Headaches; cognitive dysfunction; seizures; psychosis | Severe headache with fever |
Pericarditis | 20–30% | – |
Pleuritis | 20–30% | – |
Renal Disease | 48–100% | – |
Gastrointestinal Disease | 24–40% | + |
Manifestations | Peritonitis (sterile); abnormal liver function; pancreatitis; colitis | Focal minimal triaditis |
Hematological disorders | 33–75% | + |
Manifestations | Anemia; lymphopenia > neutropenia; thrombocytopenia | Chronic anemia; intermittent thrombocytopenia, neutropenia and lymphopenia |
Inflammatory Markers | ESR correlates with active disease; CRP often normal | ESR elevated disproportionate to CRP with fever |
Autoantibodies | ||
 ANA | > 99% | + |
 Anti-ds DNA | 84–100% | – |
 Anti-Sm | 23–48% | – |
 Anti-Ro | 38–54% | + |
 Anti-La | 16–32% | + |