Periodic fever: From Still's disease to Muckle-Wells syndrome.

Autor: Solís Marquínez MN; Servicio de Medicina Interna, Hospital Universitario de Cabueñes, Gijón, Asturias, España. Electronic address: natayasolis@gmail.com., García Fernández E; Servicio de Reumatología, Hospital Universitario de Cabueñes, Gijón, Asturias, España., Morís de la Tassa J; Servicio de Medicina Interna, Hospital Universitario de Cabueñes, Gijón, Asturias, España; Departamento de Medicina, Facultad de Medicina y Ciencias de la Salud, Universidad de Oviedo, Oviedo, Asturias, España.
Jazyk: English; Spanish; Castilian
Zdroj: Reumatologia clinica [Reumatol Clin (Engl Ed)] 2019 Sep - Oct; Vol. 15 (5), pp. e39-e40. Date of Electronic Publication: 2017 Jun 02.
DOI: 10.1016/j.reuma.2017.04.008
Abstrakt: Muckle-Wells syndrome is a systemic autoinflammatory disease included in the group of hereditary periodic febrile syndromes. We report the case of a patient with this rare disease to call the attention to the singularity of this condition, its low incidence, its atypical presentation and the subsequent delay in the diagnosis, which is reached when late and devastating consequences have taken place. In this case, the first-line therapy, anti-interleukin 1 (IL-1), failed to control the disease. Nevertheless, the IL-6 inhibitor, tocilizumab, proved effective, achieving the total remission of nephrotic syndrome associated with AA secondary amyloidosis, changing the bleak prognosis of this disease.
(Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)
Databáze: MEDLINE
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