Secondary 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] Sahar Memar Montazerin, M.D.[3]
Overview
In amyloidosis, insoluble fibrils of amyloid are deposited in organs, causing organ dysfunction and eventually death. Patients with amyloidosis may eventually suffer from heart failure, nephrotic syndrome, hepatomegaly and peripheral neuropathy. Prognosis is poor and 5-years survival rate is 51.3%.Infection and renal failure are the most common cause of death.
Natural History, Complications, and Prognosis
Natural History
- In amyloidosis, insoluble fibrils of amyloid are deposited in the organs, causing organ dysfunction.[1]
- Patients with amyloidosis may eventually suffer from nephrotic syndrome, renal failure, and death.
Complications
- In patients with amyloidosis, the most frequent complications include:[2]
- Nephrotic syndrome, the most common
- Hepatomegaly
- Peripheral neuropathy
- Heart failure
Prognosis
- Prognosis is poor and 5-years survival rate is 51.3%.[3]
- Following factors are associated with higher rates of mortality:
- It has been reported that, survival rate is not affected by the underlying etiology.[4]
- Infection and renal failure are the most common cause of death.
- Median survival has been reported between 6 to 9 years.[1]
- The most important predictable factor for development of renal failure and death is higher production of SAA.
References
- ↑ 1.0 1.1 Lachmann, Helen J.; Goodman, Hugh J.B.; Gilbertson, Janet A.; Gallimore, J. Ruth; Sabin, Caroline A.; Gillmore, Julian D.; Hawkins, Philip N. (2007). "Natural History and Outcome in Systemic AA Amyloidosis". New England Journal of Medicine. 356 (23): 2361–2371. doi:10.1056/NEJMoa070265. ISSN 0028-4793.
- ↑ 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.
- ↑ Ahbap E, Kara E, Sahutoglu T, Basturk T, Koc Y, Sakaci T, Sevinc M, Akgol C, Ucar ZA, Kayalar AO, Bayraktar F, Ozagari AA, Unsal A (July 2014). "Outcome of 121 patients with renal amyloid a amyloidosis". J Res Med Sci. 19 (7): 644–9. PMC 4214024. PMID 25364365.
- ↑ Ayar Y, Ersoy A, Oksuz MF, Ocakoglu G, Vuruskan BA, Yildiz A, Isiktas E, Oruc A, Celikci S, Arslan I, Sahin AB, Güllülü M (2017). "Clinical outcomes and survival in AA amyloidosis patients". Rev Bras Reumatol Engl Ed. 57 (6): 535–544. doi:10.1016/j.rbre.2017.02.002. PMID 29173691.