SYSTEMIC AMYLOIDOSIS IN A PATIENT WITH HYPOGAMMAGLOBULINEMIA

A 49-year-old patient with an 18-year history of hypogammaglobulinaemi a presented with nephrotic syndrome due to systemic amyloidosis. Recur rent infections as a consequence of an inadequate gammaglobulin substi tution therapy were regarded as the main reason for the development of amyloidosis, When a high-dose intravenous immunoglobulin therapy was started, the clinical symptoms declined and the patient felt moderatel y well. Later the patient developed symmetrical polyarthritis clinical ly suggestive of rheumatoid arthritis. Although the incidence of arthr itis is increased in hypogammaglobulinaemia, arthritis has not been re ported in any of the few previously described patients with hypogammag lobulinaemia-associated amyloidosis. Moreover, this case provides furt her evidence that, in these patients, the amyloid fibrils may be of th e AA type.