title: [Hypergammaglobulinemia D syndrome].
abstract: The hyperimmunoglobulinemia D syndrome is characterized by early onset of attacks of periodic fever and an elevated serum polyclonal Ig D (> 100 U/ml). Symptoms during attacks include joint involvements (arthralgia/arthritis), skin lesions, swollen lymph nodes, headache and abdominal complaints (vomiting, diarrhea and pain). The ethiopathogenia is unknown. It is transmitted by autosomal recessive inheritance. The hyperimmunoglobulinemia D syndrome should be distinguished from other periodic febrile syndroms such as systemic-onset juvenile rheumatoid arthritis, CINCA syndrome, FADA syndrome, familial mediterranean fever and adult-onset Still disease. There is no therapy for the syndrome but the prognosis is good because the frequency and severity of the attacks tends to diaeminish with age.
authors.affiliation: Servicio de Reumatología, Nueva Clínica Quirón, Zaragoza.
authors.forename: M
authors.initials: M
authors.lastname: Medrano San Ildefonso
authors.forename: C
authors.initials: C
authors.lastname: Bruscas Izu
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications / Q000175:diagnosis / Q000209:etiology; D007072:Immunoglobulin D
chemical_list: D007072:Immunoglobulin D