Document Detail

Treatment with anakinra in the hyperimmunoglobulinemia D/periodic fever syndrome.
MedLine Citation:
PMID:  16871408     Owner:  NLM     Status:  MEDLINE    
Hyperimmunoglobulinemia D/periodic fever syndrome is caused by recessively inherited mutations in the mevalonate kinase gene and is characterized by persistently high polyclonal serum IgD titre and recurrent febrile attacks. No conventional therapy exists for preventing the typical recurrent inflammatory picture of patients. A host of studies have evidenced that elevated levels of various cytokines, such as interleukin-1 (IL-1), mark febrile attacks in this disease and that IL-1 might represent a suitable therapeutic target. We describe the case of a 7-year-old female-child with an established diagnosis of hyperimmunoglobulinemia D/periodic fever syndrome in whom anakinra, IL-1 receptor antagonist, was daily administered at the dosage of 1 mg/kg/day by subcutaneous injection for 18 months after numerous disappointing attempts with non-steroidal anti-inflammatory drugs, steroids, colchicine and etanercept through the years. The clinical response under anakinra treatment was recorded through a standardized diary, whilst inflammation parameters were serially measured in comparison with the half-year before starting anakinra. Frequency and severity of fever attacks were totally reduced by anakinra and this is the first child demonstrating that symptoms of hyperimmunoglobulinemia D/periodic fever syndrome might be at least extenuated by anakinra, though not abolished.
Donato Rigante; Valentina Ansuini; Barbara Bertoni; Anna Lisa Pugliese; Laura Avallone; Gilda Federico; Achille Stabile
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2006-07-27
Journal Detail:
Title:  Rheumatology international     Volume:  27     ISSN:  0172-8172     ISO Abbreviation:  Rheumatol. Int.     Publication Date:  2006 Nov 
Date Detail:
Created Date:  2006-10-26     Completed Date:  2009-05-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8206885     Medline TA:  Rheumatol Int     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  97-100     Citation Subset:  IM    
Department of Pediatric Sciences, Center of Periodic Fevers, Università Cattolica Sacro Cuore, Rome, Italy.
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MeSH Terms
Antirheumatic Agents / therapeutic use*
Dose-Response Relationship, Drug
Familial Mediterranean Fever / drug therapy*
Interleukin 1 Receptor Antagonist Protein / therapeutic use*
Mevalonate Kinase Deficiency / drug therapy*
Receptors, Interleukin-1 / antagonists & inhibitors
Treatment Outcome
Reg. No./Substance:
0/Antirheumatic Agents; 0/Interleukin 1 Receptor Antagonist Protein; 0/Receptors, Interleukin-1

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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