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Table 1 Systemic Lupus International Collaborating Clinics (SLICC) criteria and the presence (√) or absence (x) for each affected sibling [25]

From: Successful use of ofatumumab in two cases of early-onset juvenile SLE with thrombocytopenia caused by a mutation in protein kinase C δ

  II-1 II-2 II-3
Acute cutaneous lupus
Chronic cutaneous lupus x x x
Oral or nasal ulcers x x
Non-scarring alopecia
Arthritis x x x
Serositis x x
Renal x x
Neurologic x x x
Haemolytic anaemia
Leukopenia x
Thrombocytopenia
ANA (above lab range)
Anti-dsDNA(above lab range)
Anti-Sm x x
Anti-phospholipid antibody x
Low complement
Direct Coombs’ test not assessed not assessed not assessed
  1. WCC White cell count, ANA Anti-nuclear antibodies, ds-DNA Double-stranded deoxyribonucleic acid, Sm Smith. To fulfil these classification criteria, 4 or more criteria must be present (including at least one clinical and one immunological feature), or biopsy proven lupus nephritis with positive ANA or anti-DNA antibodies