Background and Aims We describe the case of a eight year old girl who presented with severe acute renal failure, on a background of a previous presentation with bilateral orbital inflammatory lesions. Method Our patient underwent extensive immunological testing to ascertain the cause for both presentations. Results An eight year old female was referred with acute renal failure, following a six day history of vomiting, mild non-bloody diarrhoea and oliguria. Investigations showed a creatinine of 1398umol/l, urea 68umol/l and hyperkalaemia. Urine microscopy revealed granular and white cell casts. Immunological studies showed positive perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA) with a myeloperoxidase (MPO) titre of 9.3IU/ml (0.0-3.4 IU/ml). Renal biopsy(figure1) showed severe necrotising glomerulonephritis. Interestingly, this patient had presented 1 year prior with bilateral orbital inflammatory masses with orbital biopsy showing reactive lymphoid hyperplasia and immunohistochemical analysis indicating IgG4 positive plasma cells. Orbital lesions had resolved with corticosteroid therapy. Following representation with severe renal failure, she was treated with haemofiltration, plasmapheresis, methylprednisolone and subsequent immunosuppression. She had a remarkable response to treatment and measured creatinine recovered to 53umol/l. There was no evidence of other system involvement at this stage. Conclusion ANCA associated vasculitides are extremely rare in childhood. Paediatric data remains limited with most knowledge derived from larger adult studies. We describe the case of a young girl with severe acute renal failure and a previous presentation with bilateral orbital inflammatory masses associated with MPO-ANCA positivity. We postulate a link between both presentations. Fortunately she demonstrated an excellent response to treatment and continues to progress well without relapse.
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