Abstract |
In 1984, Van der Meer first reported six patients with a long history of recurrent attacks of fever of unknown cause and a constantly elevated polyclonal IgD (> 100 U/mL); he suggested the acronym of " hyper-IgD syndrome" (HIDS). A recent literature review identified 60 cases (59 from Europe and 1 from Japan). The mean age was 27 years (range: 3-69 years). The family studies have shown a positive family history for periodic fever (40% out of the patients) but not for hyper- IgD. The median age at onset was 0.5 years (range from the first weeks of life to 53 years). The length of the febrile attacks, though variable, lasted from 3 to 7 days. The frequency of the attacks varied among the individual patients, but in general it was once a month or bimonthly. The fever was sustained in all 60 patients (from 38 degrees C to 41 degrees C), with a rapid rose, a plateau and a slow decline to normal values over 5 days. The associated clinical findings involve abdominal symptoms ( pain, vomiting, diarrhea), recurrent peritonitis, lymphadenopathy, splenomegaly, articular manifestations (non-destructive recurrent arthritis) and skin lesions ( vasculitis). The prognosis is benign. The aetiopathogenesis of HIDS is unknown. The role of IgD in the pathogenesis remains to be elucidated. The therapy is only supportive.
|
Authors | R Scolozzi |
Journal | Recenti progressi in medicina
(Recenti Prog Med)
Vol. 86
Issue 6
Pg. 243-7
(Jun 1995)
ISSN: 0034-1193 [Print] Italy |
Vernacular Title | La sindrome iper-IgD (HIDS: hyper-IgD syndrome). |
PMID | 7624586
(Publication Type: Editorial, English Abstract)
|
Chemical References |
|
Topics |
- Adolescent
- Adult
- Aged
- Child
- Child, Preschool
- Diagnosis, Differential
- Familial Mediterranean Fever
(diagnosis, immunology)
- Female
- Humans
- Hypergammaglobulinemia
(diagnosis, immunology)
- Immunoglobulin D
(analysis)
- Infant, Newborn
- Male
- Middle Aged
- Prognosis
- Syndrome
|