Familial mediterranean fever natural history, complications and prognosis: Difference between revisions
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**[[Family history]] of [[amyloidosis]] | **[[Family history]] of [[amyloidosis]] | ||
**Male sex | **Male sex | ||
[[Familial mediterranean fever|FMF]] may also be [[Complications|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> | [[Familial mediterranean fever|FMF]] may also be [[Complications|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>{{cite journal |vauthors=Ardalan M, Nasri H |title=Massive proteinuria and acute glomerulonephritis picture in a patient with Familial Mediterranean fever and E148Q mutation |journal=Iran J Kidney Dis |volume=8 |issue=6 |pages=486–8 |date=November 2014 |pmid=25362225 |doi= |url=}} | ||
*[[Nephrotic syndrome]] | *[[Nephrotic syndrome]] | ||
*[[Focal segmental glomerulosclerosis|Focal segmental glomerular sclerosis]] | *[[Focal segmental glomerulosclerosis|Focal segmental glomerular sclerosis]] | ||
*[[IgA nephropathy]] | *[[IgA nephropathy]] | ||
*[[Minimal change disease]] | *[[Minimal change disease]] | ||
*mesangial proliferative glomerulonephritis<ref name="CagdasGucer2005">{{cite journal|last1=Cagdas|first1=Deniz N.|last2=Gucer|first2=Safak|last3=Kale|first3=Gülsev|last4=Duzova|first4=Ali|last5=Ozen|first5=Seza|title=Familial Mediterranean fever and mesangial proliferative glomerulonephritis: report of a case and review of the literature|journal=Pediatric Nephrology|volume=20|issue=9|year=2005|pages=1352–1354|issn=0931-041X|doi=10.1007/s00467-005-1991-9}}</ref> | |||
==Prognosis== | ==Prognosis== | ||
Revision as of 02:28, 22 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 vasculitic disorders. The prognosis does not differ from that of the general population. However, renal involvement is the determinant factor of patient survival rate.
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]Ardalan M, Nasri H (November 2014). "Massive proteinuria and acute glomerulonephritis picture in a patient with Familial Mediterranean fever and E148Q mutation". Iran J Kidney Dis. 8 (6): 486–8. PMID 25362225.
- Nephrotic syndrome
- Focal segmental glomerular sclerosis
- IgA nephropathy
- Minimal change disease
- mesangial proliferative glomerulonephritis[6]
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.
- ↑ Cagdas, Deniz N.; Gucer, Safak; Kale, Gülsev; Duzova, Ali; Ozen, Seza (2005). "Familial Mediterranean fever and mesangial proliferative glomerulonephritis: report of a case and review of the literature". Pediatric Nephrology. 20 (9): 1352–1354. doi:10.1007/s00467-005-1991-9. ISSN 0931-041X.