Familial mediterranean fever natural history, complications and prognosis: Difference between revisions
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===Natural History=== | ===Natural History=== | ||
*The [[symptoms]] of [[Familial mediterranean fever|familial Mediterranean fever]] usually develop in the first two decades of life and start with [[symptoms]] such as [[fever]] and [[serositis]].<ref name="pmid5340644">{{cite journal |vauthors=Sohar E, Gafni J, Pras M, Heller H |title=Familial Mediterranean fever. A survey of 470 cases and review of the literature |journal=Am. J. Med. |volume=43 |issue=2 |pages=227–53 |date=August 1967 |pmid=5340644 |doi= |url=}}</ref> | *The [[symptoms]] of [[Familial mediterranean fever|familial Mediterranean fever]] usually develop in the first two decades of life and start with [[symptoms]] such as [[fever]] and [[serositis]].<ref name="pmid5340644">{{cite journal |vauthors=Sohar E, Gafni J, Pras M, Heller H |title=Familial Mediterranean fever. A survey of 470 cases and review of the literature |journal=Am. J. Med. |volume=43 |issue=2 |pages=227–53 |date=August 1967 |pmid=5340644 |doi= |url=}}</ref> | ||
[[Serositis]] may | *[[Serositis]] manifests with [[abdominal pain]], and/or [[chest pain]], [[arthralgia]] and/or [[arthritis]] of the hip, knee or ankle, and erysiplas-like erythema, frequently on the lower limbs.<ref name="pmid16755655">{{cite journal |vauthors=Lidar M, Yaqubov M, Zaks N, Ben-Horin S, Langevitz P, Livneh A |title=The prodrome: a prominent yet overlooked pre-attack manifestation of familial Mediterranean fever |journal=J. Rheumatol. |volume=33 |issue=6 |pages=1089–92 |date=June 2006 |pmid=16755655 |doi= |url=}}</ref> | ||
*[[Fever]] episodes may last 1 to 3 days. | |||
*Patients usually manifest minimal [[symptoms]] in between the attacks. | |||
*Even i the same patient, clinical presentation may vary over time. | |||
*If left untreated, 75% of patients with [[Familial mediterranean fever|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|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 [[Familial mediterranean fever|FMF]] is the development of [[AA amyloidosis|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 most devastating [[complication]] of [[Familial mediterranean fever|FMF]] is the development of [[AA amyloidosis|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> | ||
Revision as of 19:44, 3 June 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 manifests with abdominal pain, and/or chest pain, arthralgia and/or arthritis of the hip, knee or ankle, and erysiplas-like erythema, frequently on the lower limbs.[2]
- Fever episodes may last 1 to 3 days.
- Patients usually manifest minimal symptoms in between the attacks.
- Even i the same patient, clinical presentation may vary over time.
- If left untreated, 75% of patients with familial Mediterranean fever may progress to develop amyloidosis.[3]
Complications
The most devastating complication of FMF is the development of AA-amyloidosis which may lead to end-stage renal disease.[4][5]
- 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:[6][7]
- Nephrotic syndrome
- Focal segmental glomerular sclerosis
- IgA nephropathy[8]
- Minimal change disease
- mesangial proliferative glomerulonephritis[9]
Prognosis
- Prognosis is generally excellent. However, amyloid renal involvement is the determinant factor of patient survival rate.[4]
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.
- ↑ Lidar M, Yaqubov M, Zaks N, Ben-Horin S, Langevitz P, Livneh A (June 2006). "The prodrome: a prominent yet overlooked pre-attack manifestation of familial Mediterranean fever". J. Rheumatol. 33 (6): 1089–92. PMID 16755655.
- ↑ 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.
- ↑ 4.0 4.1 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.
- ↑ 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.
- ↑ Rigante, Donato; Federico, Gilda; Ferrara, Pietro; Maggiano, Nicola; Avallone, Laura; Pugliese, Anna Lisa; Stabile, Achille (2005). "IgA nephropathy in an Italian child with familial Mediterranean fever". Pediatric Nephrology. 20 (11): 1642–1644. doi:10.1007/s00467-005-2023-5. ISSN 0931-041X.
- ↑ 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.