Systemic vasculitis complicating infantile autoimmune enteropathy.

ABSTRACT

An infant with unexplained sideroblastic anaemia and severe protracted diarrhoea due to autoimmune gut disease is reported. Despite treatment with an exclusion diet and immunosuppressive agents she developed a severe systemic vasculitic illness. Intensive treatment with plasmapheresis resulted in resolution of the vasculitic process but the child subsequently died after overwhelming septicaemia.