Periodic fever syndrome: Difference between revisions
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== Background == |
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* Most of the periodic fever syndromes result in caspase-1 activation and the release of IL-1β |
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{| class="wikitable" |
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!Age at Onset |
!Age at Onset |
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!Duration |
!Duration |
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!LNs |
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!Lymphadenopathy |
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!AP |
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!Abdo Pain |
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!MSK |
!MSK |
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!CP |
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!Chest Pain |
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!Rash |
!Rash |
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!Other |
!Other |
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|cervical |
|cervical |
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|very common |
|very common |
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|arthralgias, rare arthritis |
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|unusual |
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|very common, maculopapular |
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|[[hepatosplenomegaly]], [[Aphthous ulcer|aphthous ulcers]] |
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|[[etanercept]], [[IL-1 inhibitor|IL-1 inhibitors]] |
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|[[NAPS12]] |
|[[NAPS12]] |
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|yes |
|yes |
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|urticaria/erythema |
|urticaria/erythema |
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|cold sensitivity, headache |
|cold sensitivity, [[headache]] |
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|— |
|— |
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|[[TRAPS]] |
|[[TRAPS]] |
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|mostly northern European, but all ethnic groups |
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|variable |
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|more than 5 days, up to weeks to months |
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|common |
|common |
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|common |
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|arthralgias, myalgias |
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|yes |
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|common erysipelas-like including arms |
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|periorbital edema, [[headache]] |
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|[[IL-1 inhibitor|IL-1 inhibitors]], [[TNF-α inhibitor|TNF-α inhibitors]], [[IL6R inhibitor|anti-IL6R mAb]] |
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|[[CAPS]] |
|[[CAPS]] |
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|all ethnic groups |
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|childhood/neonatal |
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|variable, depending on subtype |
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|rarerare |
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|arthralgias, arthritis, bony overgrowth (depending on subtype) |
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|no |
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|[[urticaria]], erythema |
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|cold sensitivity, [[deafness]], [[aseptic meningitis]], [[papilledema]], dysmorphism (depending on subtype) |
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|[[IL-1 inhibitor|IL-1 inhibitors]] |
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|[[PFAPA]] |
|[[PFAPA]] |
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|all ethnic groups |
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|<5 years |
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|3 to 6 days |
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|cervical |
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|common |
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|arthralgias |
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|rare |
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|common |
|common |
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|[[aphthous stomatitis]], [[pharyngitis]], [[headache]] |
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|[[corticosteroids]], [[cimetidine]], [[tonsillectomy]] |
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* LNs=lymph nodes; AP=abdominal pain; MSK=musculoskeletal symptoms; CP=chest pain |
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*From [[CiteRef::savic2012fa]] |
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[[Category:Fever syndromes]] |
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[[Category:Rheumatology]] |
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Latest revision as of 21:55, 24 April 2022
Background
- Most of the periodic fever syndromes result in caspase-1 activation and the release of IL-1β
| Ethnicity | Age at Onset | Duration | LNs | AP | MSK | CP | Rash | Other | Treatment | |
|---|---|---|---|---|---|---|---|---|---|---|
| FMF | Armenian, Arab, Jewish, Turkish | <20 years | 1-4 days | rare | very common sterile peritonitis | monoarthritis of knee, ankle, wrist | pleuritis, often unilateral | rare erysipelas-like on legs | pericarditis, scrotal attacks, splenomegaly | colchicine |
| HIDS | Dutch, French | childhood | 3-7 days | cervical | very common | arthralgias, rare arthritis | unusual | very common, maculopapular | hepatosplenomegaly, aphthous ulcers | etanercept, IL-1 inhibitors |
| NAPS12 | Guadeloupe, Italy | neonatal | 2-10 days | yes | yes | arthralgias, myalgias | yes | urticaria/erythema | cold sensitivity, headache | — |
| TRAPS | mostly northern European, but all ethnic groups | variable | more than 5 days, up to weeks to months | common | common | arthralgias, myalgias | yes | common erysipelas-like including arms | periorbital edema, headache | IL-1 inhibitors, TNF-α inhibitors, anti-IL6R mAb |
| CAPS | all ethnic groups | childhood/neonatal | variable, depending on subtype | very rare | rarerare | arthralgias, arthritis, bony overgrowth (depending on subtype) | no | urticaria, erythema | cold sensitivity, deafness, aseptic meningitis, papilledema, dysmorphism (depending on subtype) | IL-1 inhibitors |
| PFAPA | all ethnic groups | <5 years | 3 to 6 days | cervical | common | arthralgias | rare | common | aphthous stomatitis, pharyngitis, headache | corticosteroids, cimetidine, tonsillectomy |
- LNs=lymph nodes; AP=abdominal pain; MSK=musculoskeletal symptoms; CP=chest pain
- From 1
References
- ^ Sinisa Savic, Laura J. Dickie, Michele Battellino, Michael F. McDermott. Familial Mediterranean fever and related periodic fever syndromes/autoinflammatory diseases. Current Opinion in Rheumatology. 2012;24(1):103-112. doi:10.1097/bor.0b013e32834dd2d5.
