Tumor necrosis factor receptor-associated periodic syndrome - Revision history

Aidan at 19:58, 12 July 2023

2023-07-12T19:58:41Z

← Older revision		Revision as of 19:58, 12 July 2023
Line 10:		Line 10:
	* Also:		* Also:
	** Myalgias and limb pain, sometimes migratory (59 to 85%)		** Myalgias and limb pain, sometimes migratory (59 to 85%)
	** Abdominal symptoms (36 to 74%), including pain and vomiting (18%)		** Abdominal symptoms (36 to 74%), including pain and [[vomiting]] (18%)
	** Rash (55 to 63%), which is single or multiple erythematous patches that may spread downward		** [[Rash]] (55 to 63%), which is single or multiple erythematous patches that may spread downward
	** Lymphadenopathy (25%)		** [[Lymphadenopathy]] (25%)
	** Headache (20 to 25%)		** [[Headache]] (20 to 25%)
	** Chest pain (14 to 25%)		** Chest pain (14 to 25%)
	** Conjunctivitis (18 to 20%)		** [[Conjunctivitis]] (18 to 20%)
	** Periorbital edema (9 to 20%)		** Periorbital edema (9 to 20%)
	** Pharyngitis, with or without aphthae (9 to 18%)		** [[Pharyngitis]], with or without aphthae (9 to 18%)
	** Monoarticular arthritis (13%)		** [[Monoarticular arthritis]] (13%)
	** Hepatomegaly and/or splenomegaly (5 to 10%)		** [[Hepatomegaly]] and/or [[splenomegaly]] (5 to 10%)
	** Occasionally, recurrent [[pericarditis]]		** Occasionally, recurrent [[pericarditis]]
	** Secondary AA amyloidosis (10 to 15%)		** Secondary AA [[amyloidosis]] (10 to 15%)

	==Diagnosis==		==Diagnosis==
	* Presence of a confirmatory TNFRSF1A genotype* and at least 1 among the following:		* Presence of a confirmatory TNFRSF1A genotype and at least 1 among the following:
	** Duration of episodes ≥7 days		** Duration of episodes ≥7 days
	** Myalgia		** [[Myalgia]]
	** Migratory rash		** Migratory rash
	** Periorbital ~~oedema~~		** Periorbital edema
	** Relatives affected		** Relatives affected
	* OR		* OR
	* Presence of a not confirmatory TNFRSF1A genotype¶ and at least 2 among the following:		* Presence of a not confirmatory TNFRSF1A genotype and at least 2 among the following:
	** Duration of episodes ≥7 days		** Duration of episodes ≥7 days
	** Myalgia		** [[Myalgia]]
	** Migratory rash		** Migratory rash
	** Periorbital ~~oedema~~		** Periorbital edema
	** Relatives affected		** Relatives affected
	[[Category:Periodic fever syndromes]]		[[Category:Periodic fever syndromes]]

Aidan at 17:05, 21 June 2023

2023-06-21T17:05:06Z

← Older revision		Revision as of 17:05, 21 June 2023
Line 37:		Line 37:
	** Periorbital oedema		** Periorbital oedema
	** Relatives affected		** Relatives affected
			[[Category:Periodic fever syndromes]]

Aidan: Created page with "==Background== * A periodic fever syndrome caused by autoimmunity * Autosomal dominant with incomplete penetrance * Rare, described in all ethnic groups * Usually presents in childhood, though 10% of cases are in those over age 30 years ==Clinical Manifestations== * Fever over 38 degrees over months to years in the absence of infection * Typically occur every five to six weeks, and last five days to 2 weeks or longer * Also: ** Myalgias and limb pain, sometimes migr..."

2023-06-21T17:04:44Z

Created page with "==Background== * A periodic fever syndrome caused by autoimmunity * Autosomal dominant with incomplete penetrance * Rare, described in all ethnic groups * Usually presents in childhood, though 10% of cases are in those over age 30 years ==Clinical Manifestations== * Fever over 38 degrees over months to years in the absence of infection * Typically occur every five to six weeks, and last five days to 2 weeks or longer * Also: ** Myalgias and limb pain, sometimes migr..."

New page

==Background==
* A [[periodic fever syndrome]] caused by autoimmunity
* Autosomal dominant with incomplete penetrance
* Rare, described in all ethnic groups
* Usually presents in childhood, though 10% of cases are in those over age 30 years

==Clinical Manifestations==
* Fever over 38 degrees over months to years in the absence of infection
* Typically occur every five to six weeks, and last five days to 2 weeks or longer
* Also:
** Myalgias and limb pain, sometimes migratory (59 to 85%)
** Abdominal symptoms (36 to 74%), including pain and vomiting (18%)
** Rash (55 to 63%), which is single or multiple erythematous patches that may spread downward
** Lymphadenopathy (25%)
** Headache (20 to 25%)
** Chest pain (14 to 25%)
** Conjunctivitis (18 to 20%)
** Periorbital edema (9 to 20%)
** Pharyngitis, with or without aphthae (9 to 18%)
** Monoarticular arthritis (13%)
** Hepatomegaly and/or splenomegaly (5 to 10%)
** Occasionally, recurrent [[pericarditis]]
** Secondary AA amyloidosis (10 to 15%)

==Diagnosis==
* Presence of a confirmatory TNFRSF1A genotype* and at least 1 among the following:
** Duration of episodes ≥7 days
** Myalgia
** Migratory rash
** Periorbital oedema
** Relatives affected
* OR
* Presence of a not confirmatory TNFRSF1A genotype¶ and at least 2 among the following:
** Duration of episodes ≥7 days
** Myalgia
** Migratory rash
** Periorbital oedema
** Relatives affected