Familial mediterranean fever natural history, complications and prognosis: Difference between revisions

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*If left untreated, [#]% of patients with [disease name] may progress to develop amyloidosis, [manifestation 2], and [manifestation 3].
*If left untreated, [#]% of patients with [disease name] may progress to develop amyloidosis, [manifestation 2], and [manifestation 3].
==Complications==
==Complications==
[[amyloidosis|AA-amyloidosis]] with [[renal failure]] is a complication and may develop without overt crises. AA (amyloid protein) is produced in very large quantities during attacks and at a low rate between them, and accumulates  mainly in the [[kidney]], as well as the [[heart]], [[spleen]], [[gastrointestinal tract]] and the [[thyroid]].<ref name=Livneh/>
*The most devastating complication of FMF is the development of AA-amyloidosis which may lead to end-stage renal disease.<ref name="pmid22543627">{{cite journal |vauthors=Akar S, Yuksel F, Tunca M, Soysal O, Solmaz D, Gerdan V, Celik A, Sen G, Onen F, Akkoc N |title=Familial Mediterranean fever: risk factors, causes of death, and prognosis in the colchicine era |journal=Medicine (Baltimore) |volume=91 |issue=3 |pages=131–6 |date=May 2012 |pmid=22543627 |doi=10.1097/MD.0b013e3182561a45 |url=}}</ref><ref name="Lachmann2015">{{cite journal|last1=Lachmann|first1=Helen J.|title=Long-Term Complications of Familial Mediterranean Fever|volume=3|year=2015|pages=91–105|issn=2282-6505|doi=10.1007/978-3-319-14615-7_6}}</ref>
*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]].<ref name=Livneh>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.</ref>
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]].<ref name=Livneh>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.</ref>
==Prognosis==
==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.
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.

Revision as of 19:52, 21 May 2019

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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

  1. 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.
  2. 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.
  3. 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.