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Real de Asúa D, Costa R, Galván JM, et al. Systemic AA amyloidosis: epidemiology, diagnosis, and management. Clin Epidemiol. 2014;6:369-77doi: 10.2147/CLEP.S39981.
Real de Asúa, D., Costa, R., Galván, J. M., Filigheddu, M. T., Trujillo, D., & Cadiñanos, J. (2014). Systemic AA amyloidosis: epidemiology, diagnosis, and management. Clinical epidemiology, 6369-77. https://doi.org/10.2147/CLEP.S39981
Real de Asúa, Diego, et al. "Systemic AA amyloidosis: epidemiology, diagnosis, and management." Clinical epidemiology vol. 6 (2014): 369-77. doi: https://doi.org/10.2147/CLEP.S39981
Real de Asúa D, Costa R, Galván JM, Filigheddu MT, Trujillo D, Cadiñanos J. Systemic AA amyloidosis: epidemiology, diagnosis, and management. Clin Epidemiol. 2014 Oct 29;6:369-77. doi: 10.2147/CLEP.S39981. eCollection 2014. PMID: 25378951; PMCID: PMC4218891.
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Clin Epidemiol. 2014 Oct 29;6:369-77. doi: 10.2147/CLEP.S39981. eCollection 2014.

Systemic AA amyloidosis: epidemiology, diagnosis, and management.

Clinical epidemiology

Diego Real de Asúa, Ramón Costa, Jose María Galván, María Teresa Filigheddu, Davinia Trujillo, Julen Cadiñanos

Affiliations

  1. Department of Internal Medicine, Fundación de Investigación Biomédica, Hospital Universitario de La Princesa, Madrid, Spain.

PMID: 25378951 PMCID: PMC4218891 DOI: 10.2147/CLEP.S39981

Abstract

The term "amyloidosis" encompasses the heterogeneous group of diseases caused by the extracellular deposition of autologous fibrillar proteins. The global incidence of amyloidosis is estimated at five to nine cases per million patient-years. While amyloid light-chain (AL) amyloidosis is more frequent in developed countries, amyloid A (AA) amyloidosis is more common in some European regions and in developing countries. The spectrum of AA amyloidosis has changed in recent decades owing to: an increase in the median age at diagnosis; a percent increase in the frequency of primary AL amyloidosis with respect to the AA type; and a substantial change in the epidemiology of the underlying diseases. Diagnosis of amyloidosis is based on clinical organ involvement and histological evidence of amyloid deposits. Among the many tinctorial characteristics of amyloid deposits, avidity for Congo red and metachromatic birefringence under unidirectional polarized light remain the gold standard. Once the initial diagnosis has been made, the amyloid subtype must be identified and systemic organ involvement evaluated. In this sense, the (123)I-labeled serum amyloid P component scintigraphy is a safe and noninvasive technique that has revolutionized the diagnosis and monitoring of treatment in systemic amyloidosis. It can successfully identify anatomical patterns of amyloid deposition throughout the body and enables not only an initial estimation of prognosis, but also the monitoring of the course of the disease and the response to treatment. Given the etiologic diversity of AA amyloidosis, common therapeutic strategies are scarce. All treatment options should be based upon a greater control of the underlying disease, adequate organ support, and treatment of symptoms. Nevertheless, novel therapeutic strategies targeting the formation of amyloid fibrils and amyloid deposition may generate new expectations for patients with AA amyloidosis.

Keywords: Congo red; amyloidosis; epidemiology; eprodisate; nephrotic syndrome; rheumatoid arthritis

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