Familial mediterranean fever natural history, complications and prognosis: Difference between revisions
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*If left untreated, 75% of patients with familial Mediterranean fever may progress to develop amyloidosis.<ref name="pmid5715490">{{cite journal |vauthors=Gafni J, Ravid M, Sohar E |title=The role of amyloidosis in familial mediterranean fever. A population study |journal=Isr. J. Med. Sci. |volume=4 |issue=5 |pages=995–9 |date=1968 |pmid=5715490 |doi= |url=}}</ref> | *If left untreated, 75% of patients with familial Mediterranean fever may progress to develop amyloidosis.<ref name="pmid5715490">{{cite journal |vauthors=Gafni J, Ravid M, Sohar E |title=The role of amyloidosis in familial mediterranean fever. A population study |journal=Isr. J. Med. Sci. |volume=4 |issue=5 |pages=995–9 |date=1968 |pmid=5715490 |doi= |url=}}</ref> | ||
==Complications== | ==Complications== | ||
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> | |||
*The development of amyloidosis has been associated with the following factors: | *The development of amyloidosis has been associated with the following factors: | ||
**Particular MEFV mutations (M694V, M694I, or M680I) | **Particular MEFV mutations (M694V, M694I, or M680I) | ||
**Family history of amyloidosis | **Family history of amyloidosis | ||
**Male sex | **Male sex | ||
FMF may also be complicated with non-amyloid kidney disease such as:<ref name="pmid24128782">{{cite journal |vauthors=Kukuy O, Livneh A, Ben-David A, Kopolovic J, Volkov A, Shinar Y, Holtzman E, Dinour D, Ben-Zvi I |title=Familial Mediterranean fever (FMF) with proteinuria: clinical features, histology, predictors, and prognosis in a cohort of 25 patients |journal=J. Rheumatol. |volume=40 |issue=12 |pages=2083–7 |date=December 2013 |pmid=24128782 |doi=10.3899/jrheum.130520 |url=}}</ref> | |||
*Nephrotic syndrome | |||
*Focal segmental glomerular sclerosis | |||
*IgA nephropathy | |||
*Minimal change disease | |||
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> | ||
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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, 75% of patients with familial Mediterranean fever may progress to develop amyloidosis.[2]
Complications
The most devastating complication of FMF is the development of AA-amyloidosis which may lead to end-stage renal disease.[3][4]
- The development of amyloidosis has been associated with the following factors:
- Particular MEFV mutations (M694V, M694I, or M680I)
- Family history of amyloidosis
- Male sex
FMF may also be complicated with non-amyloid kidney disease such as:[5]
- Nephrotic syndrome
- Focal segmental glomerular sclerosis
- IgA nephropathy
- Minimal change disease
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.[6]
Prognosis
- 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.
- ↑ Gafni J, Ravid M, Sohar E (1968). "The role of amyloidosis in familial mediterranean fever. A population study". Isr. J. Med. Sci. 4 (5): 995–9. PMID 5715490.
- ↑ 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.
- ↑ Kukuy O, Livneh A, Ben-David A, Kopolovic J, Volkov A, Shinar Y, Holtzman E, Dinour D, Ben-Zvi I (December 2013). "Familial Mediterranean fever (FMF) with proteinuria: clinical features, histology, predictors, and prognosis in a cohort of 25 patients". J. Rheumatol. 40 (12): 2083–7. doi:10.3899/jrheum.130520. PMID 24128782.
- ↑ 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.