Autosplenectomy: rare syndrome in autoimmunopathy

Treatment was initiated with glucocorticoids and plasmapheresis, with good clinical response. Because of unresponsiveness to or unbearable toxicity of subsequently started treatment with azathioprine, cyclophosphamide and ciclosporin, a regimen of repeated plasmapheresis during the following years was used to control pleural and pericardial effusions efficiently. Laboratory studies revealed an increased C reactive protein serum level (3,2 mg/dl), increased levels of immune complexes (IgG, IgM, IgA), decreased serum complement C3 and C4 and raised liver function tests.