Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome : introducing a vaccination provocation model

• Published in: Netherlands journal of medicine Vol. 63; no. 7; pp. 260 - 264
• Main Authors: BODAR, E. J, VAN DER HILST, J. C. H, DRENTH, J. P. H, VAN DER MEER, J. W. M, SIMON, A
• Format: Journal Article
• Language: English
• Published: Alphen aan den Rijn Van zuiden 01.07.2005
• Subjects: Adult; Anti-Inflammatory Agents, Non-Steroidal - administration & dosage; Anti-Inflammatory Agents, Non-Steroidal - therapeutic use; Biological and medical sciences; C-Reactive Protein - metabolism; Diseases of the osteoarticular system; Etanercept; Female; Follow-Up Studies; General aspects; Humans; Hypergammaglobulinemia - blood; Hypergammaglobulinemia - chemically induced; Hypergammaglobulinemia - drug therapy; Immunoglobulin D; Immunoglobulin G - administration & dosage; Immunoglobulin G - therapeutic use; Inflammatory joint diseases; Injections, Subcutaneous; Interleukin 1 Receptor Antagonist Protein; Medical sciences; Receptors, Tumor Necrosis Factor - administration & dosage; Receptors, Tumor Necrosis Factor - therapeutic use; Sialoglycoproteins - administration & dosage; Sialoglycoproteins - therapeutic use; Vaccines - adverse effects; Vaccination; Diseases of the osteoarticular system; IgD; Monoclonal antibody; TNF; Prevention; hyper-IgD syndrome; Etanercept; Anticytokine; Interleukin 1; Familial recurrent polyseritis; Antagonist; Recombinant protein; Fusion protein; Tumor necrosis factor α; Inflammation; Syndrome; Genetic disease; Immunomodulator; Medicine; Immunoprophylaxis; Tumor necrosis factor; Models; interleukin-I; periodic fever; Anakinra
• ISSN: 0300-2977; 1872-9061

Hyper-IgD and periodic fever syndrome (HIDS) is an hereditary autoinflammatory syndrome, characterised by recurrent inflammatory attacks. Treatment of HIDS is difficult, although simvastatin is beneficial and etanercept might be effective. Studying the treatment of a rare periodic syndrome is complicated by the varying frequency and severity of symptoms and low prevalence. Our aim was to develop a system of clinical observations to evaluate effectiveness of treatment-on-demand. Seven fever episodes in three HIDS patients were monitored, with and without administration of etanercept or anakinra. We developed a clinical score, which includes 12 symptoms. In one patient, inflammatory attacks were provoked by vaccination. At the onset of an attack, all patients reported a clinical score between 20 and 25. The score was used to quantify severity and define the end of an attack. Reproducible monitoring of inflammatory episodes was difficult, even in this pilot study. The effect of early administration of etanercept was variable. In one patient, a fever episode could be readily provoked within 12 to 24 hours by vaccination. In this patient, the IL-1ra analogue anakinra was more successful in aborting the inflammatory attack than etanercept. We propose that this vaccination model will allow evaluation of treatment-on-demand in a controlled setting.