[Autoinflammatory diseases in childhood]. / Klinik der autoinflammatorischen Erkrankungen im Kindesalter.
Z Rheumatol
; 68(9): 726-32, 2009 Nov.
Article
em De
| MEDLINE
| ID: mdl-19841924
ABSTRACT
Autoinflammatory diseases are a group of monogenic inflammatory diseases with an early onset in childhood. Previously these diseases were summarized as"periodic fever syndromes." Included in this spectrum are familial Mediterranean fever, mevalonate kinase deficiency, and tumor necrosis factor receptor-associated disease. They are characterized by periodic or recurrent episodes of systemic inflammation causing fever, accompanied by rash, serositis, lymphadenopathy, arthritis, and other clinical manifestations. The other large group of autoinflammatory diseases consists of the cryopyrin-associated periodic syndromes, which include the cryopyrinopathies. The mildest form is familial cold-associated syndrome, a more severe form is Muckle-Wells syndrome, and the most severe is neonatal-onset multisystem inflammatory disease/chronic infantile neurological cutaneous and articular syndrome. These are characterized by chronic or recurrent systemic inflammation associated with various clinical presentations, including urticaria-like rash, arthritis, sensorineural deafness, and central nervous system and bone involvement. In our review we focus on the clinical presentation of these diseases.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doenças Hereditárias Autoinflamatórias
/
Febre
Tipo de estudo:
Diagnostic_studies
Limite:
Child
/
Humans
Idioma:
De
Revista:
Z Rheumatol
Ano de publicação:
2009
Tipo de documento:
Article