Simultaneous presentation but contrasting clinical features in 2 female siblings with juvenile systemic lupus erythematosus (JSLE)

Methods Fifteen year old sibling 1 presented with history of low grade fevers since 2 months, oral ulcers, malar rash, alopecia, pallor, arthralgia, history of loss of weight and appetite. Investigations revealed anaemia, leukopenia, high ESR, and low C3, C4 levels. She also had ANA +ve 1: 1280, ds DNA +ve, lupus anticoagulant +ve, anticardiolipin antibody +ve, normal urine protein creatinine ratio and normal BP. She was treated with oral steroids, hydroxychloroquine, azathioprine and calcium supplements. 10 year old sibling 2 presented at the same time with history of high grade fever since one week with no obvious focus. She was initially worked up for infective cause and treated symptomatically. During second week of illness she developed rashes in her lower limbs with arthritis in her ankle joints. Within next few days she developed acute abdominal pain, pedal oedema, elevated blood pressure and seizures. Her investigations revealed anaemia , leukopenia, elevated ESR, low C3 C4, ANA +ve 1:1000,dsDNA positive, elevated urine protein and high BP of 160/110 mmHg. US abdomen showed mild ascites, CT angiogram of abdomen was normal and renal biopsy showed evidence of necrotising vasculitis. She was treated with intravenous steroids followed by oral steroids, IV cyclophosphamide, hydroxychloroquine, antihypertensives and antoconvulsants.