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]

Management plan

The management ladder begins with hydroxychloroquine at 5 mg per kg per day (the lesser of weight-based or 400 mg per day) for every patient, because it reduces flares, improves survival, protects against renal and cutaneous relapse, and reduces thrombosis. Retinopathy screening is mandatory, with a baseline ophthalmology assessment including colour vision, visual fields, and optical coherence tomography within the first year of treatment, followed by annual screening after five years, as codified by the 2016 American Academy of Ophthalmology recommendations. Strict sun protection, vaccination, and cardiovascular and bone protection complete the foundation. [3]

For the active organ-threatening disease, the next steps are glucocorticoids and steroid-sparing immunosuppression. The autoimmune haemolysis and probable nephritis warrant intravenous methylprednisolone pulses (30 mg per kg per dose, maximum 1 g, daily for 3 days) followed by oral prednisolone (0.5 to 1 mg per kg per day, maximum 60 mg per day) tapered as quickly as the disease allows, with bone protection. The renal biopsy class will refine the induction choice: proliferative class III or IV disease is treated with mycophenolate mofetil (target 2 to 3 g per day) or low-dose Euro-Lupus cyclophosphamide (500 mg every 2 weeks for 6 doses) plus glucocorticoids, with mycophenolate preferred over cyclophosphamide first-line in children because cyclophosphamide is gonadotoxic. [4]

Reproductive counselling and transition

Because this patient is an adolescent girl, reproductive considerations are central. Mycophenolate, cyclophosphamide, methotrexate, and the biologics are teratogenic or have unknown fetal effects, so reliable contraception and pregnancy planning are essential, with a switch to pregnancy-compatible agents (hydroxychloroquine, azathioprine, low-dose glucocorticoids) before any conception. Cyclophosphamide threatens future ovarian reserve, reinforcing the preference for mycophenolate first-line. If the disease proves refractory, belimumab (a B-cell-activating factor inhibitor) or anifrolumab (a type I interferon receptor antagonist) are added. The candidate should close by emphasising the need for structured transition to adult rheumatology and nephrology, with serial monitoring of complement, anti-dsDNA, full blood count, urinalysis, renal function, and blood pressure, because loss to follow-up in the transition period is a major cause of relapse and damage accrual. [2]