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.
Serositis may include, but not limited to:
- If left untreated, [#]% of patients with [disease name] may progress to develop amyloidosis, [manifestation 2], and [manifestation 3].
Complications
- The most devastating complication of FMF is the development of AA-amyloidosis which may lead to end-stage renal disease.[1][2]
- 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.[3]
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
- ↑ 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.