The epidemiology of AL and AA amyloidosis

doi:10.1016/S0950-3579(05)80119-0

Baillière's Clinical Rheumatology

Volume 8, Issue 3, August 1994, Pages 627-634

https://doi.org/10.1016/S0950-3579(05)80119-0 Get rights and content

Summary

The limited available epidemiological information on AL amyloidosis suggests that there may be differences between population-based studies and case series data with respect to variables such as age and racial patterns. Much more work in this area is required before specific aetiologic hypotheses can be tested.

Most available data to approximate the epidemiology of AA amyloidosis are derived from autopsies. Most patients with AA amyloidosis die from causes other than amyloidosis, therefore mortality data based on death certificates is of limited value in AA amyloidosis. Case ascertainment in autopsy studies may be difficult due to the frequent lack of adequate histological controls. Establishment of registries for both AL and AA amyloidosis would facilitate epidemiological research in these disorders.

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Amyloidosis is a disease caused by misfolded proteins that deposit in the extracellular matrix as fibrils, resulting in the dysfunction of the involved organ. The lung is a common target of Amyloidosis, but pulmonary amyloidosis is uncommonly diagnosed since it is rarely symptomatic. Diagnosis of pulmonary amyloidosis is usually made in the setting of systemic amyloidosis, however in cases of localized pulmonary disease, surgical or transbronchial tissue biopsy might be indicated. Pulmonary amyloidosis can be present in a variety of discrete entities. Diffuse Alveolar septal amyloidosis is the most common type and is usually associated with systemic AL amyloidosis. Depending on the degree of the interstitial involvement, it may affect alveolar gas exchange and cause respiratory symptoms. Localized pulmonary Amyloidosis can present as Nodular, Cystic or Tracheobronchial Amyloidosis which may cause symptoms of airway obstruction and large airway stenosis. Pleural effusions, mediastinal lymphadenopathy and pulmonary hypertension has also been reported.
Treatment of all types of pulmonary amyloidosis depends on the type of precursor protein, organ involvement and distribution of the disease. Most of the cases are asymptomatic and require only close monitoring. Diffuse alveolar septal amyloidosis treatment follows the treatment of underlying systemic amyloidosis. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions including debulking and stenting or with external beam radiation. Long-term prognosis of pulmonary amyloidosis usually depends on the type of lung involvement and other organ function.
Ethnic/racial differences in risk factors and clinical outcomes among patients with amyloidosis
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Citation Excerpt :
Only a few epidemiological data have been published for amyloidosis. The first study of AL amyloidosis, conducted in the United States, had an all-White sample population with an incidence rate of 9 cases per million person-years.1,30,31 The median age at presentation was 55 to 60 with the mean age of AL amyloidosis patients being 63 years.17,18
Cardiac amyloidosis is caused by abnormal extracellular deposition of insoluble fibrils in cardiac tissue. It can be fatal when untreated and is often underdiagnosed. Understanding the ethnic/racial differences in risk factors is critical for early diagnosis and treatment to improve clinical outcomes.
We performed a retrospective cross-sectional study utilizing the National Inpatient Sample database from 2015 to 2018 using ICD-10-CM codes. The primary variables of interest were race/ethnicity and amyloidosis subtypes, while the primary outcomes were in-hospital mortality, gastrointestinal bleeding, renal failure, and hospital length-of-stay.
Amyloidosis was reported in 0.17% of all hospitalizations (N = 19,678,415). Of these, 0.09% were non-Hispanic whites, 0.04% were non-Hispanic blacks, and 0.02% were Hispanic. Hospitalizations with ATTR amyloidosis subtype were frequently observed in older individuals and males with coronary artery disease, whereas AL amyloidosis subtype was associated with non-Hispanic whites, congestive heart failure, and longer hospital length of stay. Renal failure was associated with non-Hispanic blacks (adjusted relative risk [RR] = 1.31, p < 0.001), Hispanics (RR = 1.08, p = 0.028) and had an increased risk of mortality. Similarly, the hospital length of stay was longer with non-Hispanic blacks (RR = 1.19, p < 0.001) and Hispanics (RR = 1.05, p = 0.03) compared to non-Hispanic whites. Hispanics had a reduced risk of mortality (RR = 0.77, p = 0.028) compared to non-Hispanic whites and non-Hispanic blacks, and no significant difference in mortality was seen between non-Hispanic whites and non-Hispanic blacks (RR = 1.00, p = 0.963).
Our findings highlight significant ethnic/racial differences in risk factors and outcomes among amyloidosis-related US hospitalizations that can possibly be used for early detection, treatment, and better clinical outcomes.
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The epidemiology of AL and AA amyloidosis

Summary

Blood

Transactions of the Royal Society of Tropical Medicine and Hygiene

Multiple myeloma in the United States, 1950–1975

Cancer

Amyloidosis associated with rheumatoid arthritis

Medical Clinics of North America

Amyloidosis in Papua, New Guinea

Medical Journal of Australia

International variations and temporal trends in mortality from multiple myeloma

International Journal of Cancer

The prevalence, importance and significance of amyloidosis with rheumatic diseases in Turkey

Mortality rate of amyloidosis in Japan: secular trends and geographical variations

American Journal of Medical Genetics