Markant effekt af interleukin 1-receptor-antagonist hos to patienter med CINCA og med 13 års konstante symptomer
Engelsk titel: Significant effect of IL-1 receptor antagonist treatment in two patients who had CINCA syndrome with constant symptoms for 13 years
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Författare:
Idorn, Lars
;
Vissing, Nadja Hawwa
;
Jensen, Lise
;
Herlin, Troels
Email: lars.idorn@rh.regionh.dk
Språk: Dan
Antal referenser: 5
Dokumenttyp:
Fallbeskrivning
UI-nummer: 12067033
Sammanfattning
We report two cases of chronic infantile neurological, cutaneous, and articular (CINCA) syndrome. It is a rare congenital multisystemic inflammatory disease characterised by early onset of urticarial skin rash, central nervous system involvement and articular signs. Our two cases were first diagnosed at 13 and 14 years of age, respectively. From birth they presented with typical symptoms of CINCA syndrome, however isolated each symptom was non-specific. After the syndrome was recognised and treatment with anakinra was initiated, most symptoms disappeared.