REVERSAL OF NEPHROTIC SYNDROME DUE TO REACTIVE AMYLOIDOSIS (AA-TYPE) AFTER EXCISION OF LOCALIZED CASTLEMANS DISEASE

The patient (TAL), a chronic asymptomatic HBV carrier with HBsAg-anti- HBsAg circulating immune complexes, was admitted to our hospital becau se of a nephrotic syndrome due to renal amyloidosis. There was no fami ly history of hereditary amyloidosis. Recurrent arthralgias, asthenia, and weight loss were the prominent clinical features. Laboratory test results showing that severe chronic inflammatory activity had been pr esent for 6 years. Interleukin-6 (IL-6) serum concentration was 10 tim es normal and C-reactive protein was 1.9 mg/ml. A complex immunologica l picture was also present (immune complex formation, exuberant B cell reactivity, and decrease in the number of CD4 T cells). A localized f orm of Castleman's disease (CD) (plasma-cell type) was diagnosed by su rgical excision of a giant axillary lymph node. AA amyloid was present in the blood vessels. Within 60 days after excision of the mass, the systemic symptoms subsided, laboratory signs of inflammatory activity disappeared and IL-6 serum concentration returned to normal, thus esta blishing a causal relationship between the localized Castleman's disea se, elevated IL-6 concentration and the chronic inflammation responsib le for AA amyloidosis. At 10 months of follow-up, the nephrotic syndro me has reversed, kidney function has slowly ameliorated, and the patie nt has gained 12 kg. Abdominal fat aspirates drawn to search for amylo id, positive before surgery, were subsequently negative. The latter fi nding, and the remission of the nephrotic syndrome, provided strong ev idence for regression of the amyloid deposits. However, the HBsAg-anti -HBsAg immune complexes and depression of T-helper cell activity persi st. This immunological derangement is therefore not a consequence of C D. Chronic stimulation of the immune system due to the patient's inabi lity to eliminate HBV, in the contest of perturbed immunity, may have favored the genesis of the lymphadenopathy. (C) 1994 Wiley-Liss, Inc.