Familial mediterranean fever 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: Sahar Memar Montazerin, M.D.[2]
Overview
Common complications of familial Mediterranean fever include amyloidosis and increased risk of vasculitis associated disorder Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of familial Mediterranean fever usually develop in the first two decades of life and start with symptoms such as fever and serositis.[1]
Serositis may include, but not limited to:
- If left untreated, [#]% of patients with familial Mediterranean fever may progress to develop amyloidosis, vasculatic disorder, and [manifestation 3].
Complications
- The most devastating complication of FMF is the development of AA-amyloidosis which may lead to end-stage renal disease.[2][3]
- Before the introduction of Colchicine, AA-amyloidosis had been reported in 75% of FMF patients aged 43 years or older.
- The development of amyloidosis has been associated with the following factors:
- Particular MEFV mutations (M694V, M694I, or M680I)
- Family history of amyloidosis
- Male sex
There appears to be an increase in the risk for developing particular vasculitis related diseases (e.g. Henoch-Schönlein purpura), spondylarthropathy, prolonged arthritis of certain joints and protracted myalgia.[4]
Prognosis
There is no known cure for familial Mediterranean fever. Most people continue to have attacks, but the number and severity of attacks is different from person to person.
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
- The presence of amyloidosis is associated with a particularly [good/poor] prognosis among patients with familial Mediterranean fever.
References
- ↑ Sohar E, Gafni J, Pras M, Heller H (August 1967). "Familial Mediterranean fever. A survey of 470 cases and review of the literature". Am. J. Med. 43 (2): 227–53. PMID 5340644.
- ↑ Akar S, Yuksel F, Tunca M, Soysal O, Solmaz D, Gerdan V, Celik A, Sen G, Onen F, Akkoc N (May 2012). "Familial Mediterranean fever: risk factors, causes of death, and prognosis in the colchicine era". Medicine (Baltimore). 91 (3): 131–6. doi:10.1097/MD.0b013e3182561a45. PMID 22543627.
- ↑ Lachmann, Helen J. (2015). "Long-Term Complications of Familial Mediterranean Fever". 3: 91–105. doi:10.1007/978-3-319-14615-7_6. ISSN 2282-6505.
- ↑ Livneh A, Langevitz P. Diagnostic and treatment concerns in familial Mediterranean fever. Baillieres Best Pract Res Clin Rheumatol 2000;14(3):477-98. PMID 10985982.