BOLI AUTOINFLAMATORII
DOI: 10.7241/ourd.2022book.2_7 |
First Page (Prima pagina)
Bolile auto-inflamatorii (BAI) sunt determinate de anomalii monogenice sau multifactoriale ale imunită?ii înnăscute, care produc episoade recurente de tip inflama?ii multisis-temice, fără factor declan?ant specific [1]. Sistemul imun înnăscut sau nespecific este prima linie de apărare imună, rapidă, nespecifică, fără produc?ia de anticorpi.
BAI au fost descrise în 1999, prototipul fiind febra recurentă ereditară, boală monogenică caracterizată prin modificarea produc?iei de interleukină I (IL-1) [1]. Ulterior conceptul de BAI s-a extins, incluzând alte afec?iuni caracterizate de episoade inflamatorii intermitente, cu febră recurentă inexplicabilă asociată cu manifestări sistemice (în principal cutanate, musculo-scheletice, seroase, oftalmologice). Din punct de vedere genetic spectrul BAI a cuprins bolile monogenice care afectează diferite căi specifice imunită?ii înnăscute: interferon (IFN) sau calea NF-?B, explicând astfel auto-inflama?ia, autoimunitatea ?i deficitul imun [2].
BAI reprezintă boli în care imunitatea înnăscută are rol principal [3].
Manifestările dermatologice din BAI pot apare în copilărie, pot fi caracteristice ?i pot ridica suspiciunea unei afec?iuni de tip auto-inflamator sistemic.
Keywords
- Bolile auto-inflamatorii; Tumour necrosis factor; PFAPA; DIRA; MAJEED; DITRA; PAPA; SAVI; CANDLE; HA20; PLAID; APLAIDl
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