Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

The Role of Montelukast in the Management of Hyperimmunoglobulinemia D with Periodic Fever Syndrome (HIDS).

Goldsmith2,  Donald P., Barron1,  Karyl S., Jones1,  Anne, Chapelle1,  Dawn, Barham1,  Beverly, Lembo1,  Robert, Hanson1,  Eric

National Institutes of Health, Bethesda, MD
National Institutes of Health, Drexel University College of Medicine, Philadelphia, PA
NIAMS, NIH, Bethesda, MD


In 2001 Frenkel et al reported elevated urinary levels of leukotriene E4 in 7 patients with HIDS and suggested that cysteinyl leukotrienes might play a role in its pathogenesis (Arch Dis Child 85:158). There is anecdotal evidence of benefits from treatment with the leukotriene receptor antagonist, montelukast.


To assess the therapeutic response of a pediatric HIDS cohort to montelukast.


This study is a retrospective chart review of 21 children with HIDS followed at the NIH Periodic Fever Program. Seventeen of 21 patients, ages 2–16 (mean 7.2y) were treated with montelukast. All were started on once daily standard doses of montelukast based on age (2–5y 4mg; 6–14y 5mg; >15y 10mg). Daily dosage was increased up to 8–12mg in 3 children; 6 patients were also treated with one additional similar dose on the first day of each febrile episode. Clinical response was assessed at each outpatient visit by the same attending physician by interview, clinical examination, and patient score cards.


In 7 children there was no clinical response. In 2 the frequency of febrile episodes decreased slightly but each episode was more severe (not considered to be clinically beneficial). In 3 children, febrile episodes were judged to be less frequent and of reduced severity but montelukast was stopped because of behavioral side effects (one of these being parental fear [but not actual] of suicidal ideation). Similar improvement was noted in 4 additional children, but in all of these patients, benefit was judged to have dissipated after 6–8 months and montelukast was then stopped. Only 1 child still remains on montelukast. One child was lost to follow-up after starting montelukast.


Therapeutic benefit from montelukast is seen only in 7 (41%) of children and is not enduring. Significant side effects are not uncommon. In our experience montelukast is not likely to favorably alter the long term clinical course of pediatric HIDS. It seems improbable that leukotriene activation is primarily involved in the pathogenesis of HIDS but is more likely a secondary phenomenon associated with generalized immune stimulation.

Supported by the Division of Intramural Research, NIAMS

To cite this abstract, please use the following information:
Goldsmith, Donald P., Barron, Karyl S., Jones, Anne, Chapelle, Dawn, Barham, Beverly, Lembo, Robert, et al; The Role of Montelukast in the Management of Hyperimmunoglobulinemia D with Periodic Fever Syndrome (HIDS). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1698
DOI: 10.1002/art.29463

Abstract Supplement

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