Familial Mediterranean fever - Revision history

Aidan at 22:49, 24 April 2022

2022-04-24T22:49:39Z

← Older revision		Revision as of 22:49, 24 April 2022
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	* One of the [[Periodic fever syndrome\|periodic fever syndromes]]		* One of the [[Periodic fever syndrome\|periodic fever syndromes]]
	* Caused by an autosomal recessive gain-of-function mutations in ''MEFV'' gene on 16p13 that encodes pyrin		* Caused by an autosomal recessive gain-of-function mutations in ''MEFV'' gene on 16p13 that encodes pyrin
	*May be associated with [[Vasculitis\|vasculitides]] or [[Behçet disease]]		*May be associated with [[Vasculitis\|vasculitides]] ([[HSP]] and [[PAN]]) or [[Behçet disease]]

	=== Types ===		=== Types ===
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	* Type 1: acute attacks of fever plus the classical symptoms of painful serositis and arthritis		* Type 1: acute attacks of fever plus the classical symptoms of painful serositis and arthritis
	* Type 2: kidney amyloidosis, without other symptoms of FMF and without fever attacks		* Type 2: kidney amyloidosis, without other symptoms of FMF and without fever attacks
	* Type 3: patients with two mutations of ''MEFV'' gene, without fever, other symptoms of FMF, nor amyloidosis.		* Type 3: patients with two mutations of ''MEFV'' gene, without fever, other symptoms of FMF, nor amyloidosis

			=== Epidemiology ===

			* First found among Armenians, Arabs, Turks, and non-Ashkenazi Jews in North Africa and Iraq, but now also known to be found in Ashkenazi Jews, Italians, and other Mediterranean people
			* Rarely found in people without Mediterranean ancestry

	== Clinical Manifestations ==		== Clinical Manifestations ==
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	***May have unnecessary surgery or laparoscopy		***May have unnecessary surgery or laparoscopy
	***May have diarrhea or vomiting, or constipation		***May have diarrhea or vomiting, or constipation
	**Arthritis is usually monoarticular in the large joints of the lower limbs		**Arthritis is usually monoarticular in the large joints of the lower limbs and may last longer than other attacks
	**Myalgias usually in the legs		**Myalgias usually in the legs
	**Pericarditis or pleuritis		**Pericarditis or pleuritis
	**Headache from [[aseptic meningitis]]		**Headache from [[aseptic meningitis]]
			**Unilateral acute scrotal inflammation in prepubertal boys
	**May have prodrome of myalgias, arthralgias, lumbar pain, headache, dyspnea, nausea, asthenia		**May have prodrome of myalgias, arthralgias, lumbar pain, headache, dyspnea, nausea, asthenia
	**Length of episodes 1 to 4 ~~days~~, occuring every 1 week to 1 decade		**Length of episodes 24 to 72 hours, occuring every 1 week to 1 decade
	*Elevated CRP, ESR, serum amyloid A, leukocytosis and neutrophilia		*Elevated CRP, ESR, serum amyloid A, leukocytosis and neutrophilia
	*Onset usually before age 20 years		*Onset usually before age 20 years
	*Severity of attacks is inversely proportional to the age at onset (earlier is more severe)		*Severity of attacks is inversely proportional to the age at onset (earlier is more severe)
	*Attacks may be triggered by infections, stress, menses, exposure to cold, fat-rich food, or drugs		*Attacks may be triggered by infections, stress, menses, exposure to cold, fat-rich food, or drugs
			**Pregnancy may be protective

	== Differential Diagnosis ==		== Differential Diagnosis ==

Aidan at 22:40, 24 April 2022

2022-04-24T22:40:51Z

@@ Line 2: / Line 2: @@
 * One of the [[Periodic fever syndrome|periodic fever syndromes]]
-* Caused by an autosomal recessive gain-of-function mutations in ''MEFV'' gene that encodes pyrin
+* Caused by an autosomal recessive gain-of-function mutations in ''MEFV'' gene on 16p13 that encodes pyrin
 [[Category:Fever syndromes]]

Aidan: Created page with "== Background == * One of the periodic fever syndromes * Caused by an autosomal recessive gain-of-function mutations in ''MEFV'' gene that encodes..."

2022-04-24T22:04:15Z

Created page with "== Background == * One of the periodic fever syndromes * Caused by an autosomal recessive gain-of-function mutations in ''MEFV'' gene that encodes..."

New page

== Background ==

* One of the [[Periodic fever syndrome|periodic fever syndromes]]
* Caused by an autosomal recessive gain-of-function mutations in ''MEFV'' gene that encodes pyrin
* Treated with [[colchicine]]

[[Category:Fever syndromes]]