SECONDARY AMYLOIDOSIS IN LEPROSY

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Abstract
Secondary amyloidosis is the leading cause of death in lepromatous leprosy in the United States. Mortality is directly caused by amyloid involvement of the kidneys. There is a poor correlation between the duration and degree of lepromatous leprosy and the development of amyloidosis. Amyloid is considered to be a protein polysaccharide complex which tends to vary in composition. It is related to hyperglobulinemia or to some specific fraction of serum globulins and perhaps it is related to altered connective tissue, structure and disturbed antibody antigen relationship. The diagnosis of secondary amyloidosis largely depends on the following findings in order of importance; persistent proteinuria, Congo red absorption of at least 60% and preferably 80% in 1 hour, hepatosplenomegaly and hyperglobulinemia and with no consistent alterations in the globulin fractions. Serum protein and lipid changes occur in lepromatous .leprosy per se and no additional consistent alterations occurred in these cases complicated by amyloidosis. Serial Congo red tests in cases with initial negative tests will often show results approaching 100% absorption prior to death. The average duration of life after the onset of proteinuria in amyloidosis was approximately 38 months with a fairly wide range above and below this figure. Development of anemia and a progressive elevation of non-protein nitrogen are unfavorable prognostic signs. In view of the possible relationship of hyaluronic acid to amyloid, a patient with advanced amyloidosis was given 2 courses of hyaluronidase intravenously, each course consisting of approximately 250 mg of hyaluronidase given in divided doses over a 5-day period. After this therapy, there was a tendency for the globulin fractions to improve and an associated increase in polysaccharide during the hyaluronidase therapy. Further studies are necessary to determine the significance of these changes. Cases with suspected amyloidosis, but negative Congo red tests, had liver biopsies performed, with the Vim Silverman needle. This procedure is of value in differentiating amyloidosis from severe lepromatous leprosy involving the liver.