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Semin Arthritis Rheum 1994 Oct;24(2):124-38

Amyloidosis: prognosis and treatment.
Gertz MA, Kyle RA

Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN

The objective of this study was to review (1) the factors that have been
linked to prediction of clinical outcome and survival in amyloidosis and
(2) the available studies on the therapy for localized and systemic
forms of amyloidosis. We made a retrospective review of the relevant
literature on treatment and prognosis in localized and systemic
amyloidosis dating back to 1975. The most important prognostic factors
in amyloidosis are the presence of congestive heart failure, beta
2-microglobulin, and whether peripheral neuropathy dominates the
presentation. The presence of a monoclonal light chain in serum or
urine, multiple myeloma, and hepatic involvement are also important
adverse factors. Colchicine is beneficial in treating familial
Mediterranean fever and may play a role in managing secondary
amyloidosis in inflammatory bowel disease. Chlorambucil is particularly
useful in juvenile rheumatoid arthritis with amyloidosis. Dimethyl
sulfoxide provides benefit in bladder and lichen amyloidosis. A trial of
alkylating agent-based chemotherapy is reasonable in symptomatic primary
systemic amyloidosis. Advances have been made in the treatment of
amyloidosis and include chemotherapy, dialysis, transplantation, and
improved supportive care. Definite disease regressions with long-term
survival (> 10 years) are seen. Unfortunately, alternatives still need
to be developed: Of 859 patients with primary systemic amyloidosis seen
at the Mayo Clinic from 1982 to 1992, the median survival was 2.1 years.

Publication Types:
Review, academic

PMID: 7839154, UI: 95141083


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