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A young female in her early 20s presented with low-grade fever for 1 month, puffiness of face and abdominal distension for 15 days. Evaluation revealed pancytopenia with normocellular marrow, hypocomplementaemia and Coomb's positive haemolytic anaemia. She had angioedema with laryngospasm and worsening facial oedema which had dramatic response to steroids. Further workup lead to a diagnosis of systemic lupus erythematosus with neuropsychiatric involvement. Treatment with pulse steroids and cyclophosphamide resulted in dramatic improvement of her symptoms with no recurrence of angioedema during her follow-up visits.

Angioedema as the presenting feature of systemic lupus erythematosus