Systemic lupus erythematosus: Case

This girl has childhood-onset systemic lupus erythematosus. The diagnosis rests on the multisystem clinical picture (malar rash sparing the nasolabial folds, painless oral ulcers, photosensitivity, non-scarring alopecia, symmetrical non-erosive arthritis) combined with autoimmune haemolytic anaemia, thrombocytopenia, lymphopenia, a strongly positive antinuclear antibody, high-titre anti-dsDNA, and low complement C3 and C4. The active urinary sediment with blood and protein mandates renal biopsy because the histological class of lupus nephritis determines the induction strategy. Childhood-onset disease is more aggressive than adult-onset, with a higher burden of organ involvement. ^[1]

Clinical findings

The pattern is unequivocally that of active multisystem lupus. The mucocutaneous features (malar rash sparing the nasolabial folds, oral ulcers, photosensitivity, non-scarring alopecia) and the symmetrical non-erosive arthritis are the visible entry points to the diagnosis. The autoimmune haemolytic anaemia with a positive direct antiglobulin test, the thrombocytopenia, and the lymphopenia are haematological criteria, and the low complement with high-titre anti-dsDNA is the serological signature of active disease. The active urinary sediment reflects probable nephritis, which is the strongest predictor of long-term morbidity and mortality and the reason for urgent renal biopsy. ^[2]