A 47 year-old man with selective IgA deficiency (SIgAD) consulted us in November 1981, with complaints of leg edema and common cold-like symptoms and was diagnosed as SIgAD based on data of his serum protein (IgG 2,160 mg/dl, IgM 65 mg/dl, no detectable IgA). Later in July 1989, he was admitted with edema and ascites. Laboratory examinations showed; total protein 4.6 g/dl, albumin 1.26 g/dl, IgG 2,375 mg/dl, IgM 38 mg/dl, no detectable IgA. C3 22 mg/dl, C4 6 mg/dl, antinuclear antibody 80X, anti dsDNA antibody 4.5 U/ml, anti IgA antibody 258%, and lymphocytopenia. Co-culture of lymphocytes from the patient and normal subject revealed deficiency of IgA synthesis in his B cell populations. Systemic lupus erythematosus was suggested based on the findings of skin biopsy, renal damage, oral ulcer, decreased complements, autoantibody and lymphocytopenia. We could not give him conventional products of albumin and frozen plasma because he had anti IgA antibody. Instead, we administered concentrated autogenous ascitic fluid and prednisolone. His ascitic fluid disappeared and complements and albumin in his serum normalized. He has continued in good condition and is being treated as an outpatient.