Amyloidosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
Overview
Natural History
- In amyloidosis, insoluble fibrils of amyloid are deposited in body organs, causing organ dysfunction and eventually death.[1]
- In AL amyloidosis untreated individuals have the worst prognosis. In this group of patients median survival is one to two years.[2]
Complications
In patients with amyloidosis the most frequent complications include:[3]
- Heart failure
- Nephrotic syndrome
- Hepatomegaly
- Peripheral neuropathy
Prognosis
- In primary (AL) amyloidosis survival rate depends on:[4]
- Type of organ involvement (amyloid heart disease is the main prognostic factor)
- The severity of different organs involvement
- Haematological response to treatment
- The median survival of patients with AL amyloidosis is aproximately 3.8 years.[5]
- In primary (AL) amyloidosis 27% of patients dying within 1 year from diagnosis.
- The major determinant of outcome in amyloidosis is the extent of cardiac involvement.
- Cardiac amyloidosis is the cause of death in 75% of the patients who died, including sudden death in 25%.
References
- ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
- ↑ Khan MF, Falk RH (November 2001). "Amyloidosis". Postgrad Med J. 77 (913): 686–93. PMC 1742163. PMID 11677276.
- ↑ Jerzykowska S, Cymerys M, Gil LA, Balcerzak A, Pupek-Musialik D, Komarnicki MA (2014). "Primary systemic amyloidosis as a real diagnostic challenge - case study". Cent Eur J Immunol. 39 (1): 61–6. doi:10.5114/ceji.2014.42126. PMC 4439975. PMID 26155101.
- ↑ Desport E, Bridoux F, Sirac C, Delbes S, Bender S, Fernandez B, Quellard N, Lacombe C, Goujon JM, Lavergne D, Abraham J, Touchard G, Fermand JP, Jaccard A (August 2012). "Al amyloidosis". Orphanet J Rare Dis. 7: 54. doi:10.1186/1750-1172-7-54. PMC 3495844. PMID 22909024.
- ↑ Merlini G, Seldin DC, Gertz MA (May 2011). "Amyloidosis: pathogenesis and new therapeutic options". J. Clin. Oncol. 29 (14): 1924–33. doi:10.1200/JCO.2010.32.2271. PMC 3138545. PMID 21483018.