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, 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

Prognosis

Prognosis does not differ from that of the general population. However, renal involvement is the determinant factor of patient survival rate.^[3]

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.
↑ ^3.0 ^3.1 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.
↑ 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.

[pmid5340644-1] 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.

[pmid5715490-2] 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.

[pmid22543627-3] 3.0 ^3.1 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.

[Lachmann2015-4] 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.

[pmid24128782-5] 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.

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