Avcin, T

Glavnik, V

Kuhar, M

Toplak, N

English

PubMed

N Toplak

V Glavnik

M Kuhar

T Avcin

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Periodic fever syndrome with aphthous stomatitis, pharyngitis and cervical adenopathy treated with ketotifen � a case report

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Pediatric Rheumatology
Open AccessPoster presentation
Periodic fever syndrome with aphthous stomatitis, pharyngitis and 
cervical adenopathy treated with ketotifen – a case report
N Toplak*, V Glavnik, M Kuhar and T Avcin
Address: Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre, 
Ljubljana, Slovenia
* Corresponding author    
Background
The pathogenesis of PFAPA syndrome is unknown.
According to Stojanov et al. cytokine profile in PFAPA syn-
drome suggests Th1 mediated inflammatory process
resulting in continuous inflammation and reduced Th2
anti-inflammatory response. Interferon γ might be
responsible for suppressing the production of IL-4 and IL-
10. Hung et al. showed suppressive effects of ketotifen on
the expression of Th1 and Th2 related chemokines of
human monocytes.
Case report
We report a case of a four year old girl with PFAPA syn-
drome. Attacks of fever started in the first year of life and
recur every 2–4 weeks. The genetic testing for FMF, HIDS
and TRAPS was negative. No immunodeficiency or
autoimmune disease was proven. She responded well to
oral corticosteroid treatment but intervals between attacks
became shorter. Given the recent finding of possible sup-
pressive effect of ketotifen on Th1 and Th2- related chem-
okines of monocytes and favourable safety profile of this
medication, the patient was prescribed ketotifen 1 mg
twice per day for 8 months. During this period we
observed significant prolongation of interval between
fever attacks. She had only 3 attacks in 8 months. During
the period of treatment with ketotifen she was without
febrile attacks for 3 months, the longest period ever. After
cessation of therapy with ketotifen attacks recur with pre-
vious frequency.
Conclusion
Our case report suggests possible beneficial effect of keto-
tifen on the frequency of fever attacks in patients with
PFAPA syndrome. Ketotifen may be considered as an
alternative medication in patients with PFAPA syndrome
and recurrent fever attacks.
from 15th Paediatric Rheumatology European Society (PreS) Congress
London, UK. 14–17 September 2008
Published: 15 September 2008
Pediatric Rheumatology 2008, 6(Suppl 1):P204 doi:10.1186/1546-0096-6-S1-P204
<supplement> <title> <p>15<sup>th </sup>Paediatric Rheumatology European Society (PreS) Congress</p> </title> <editor>Wietse Kuis, Patricia Woo, Angelo Ravelli, Hermann Girschick, Michaël Hofer, Johannes Roth, Rotraud K Saurenmann, Alberto Martini, Pavla Dolezova, Janjaap van der Net, Pierre Quartier, Lucy Wedderburn and Jan Scott</editor> <note>Meeting abstracts – A single PDF containing all abstracts in this Supplement is available <a href="http://www.biomedcentral.com/content/files/PDF/1546-0096-6-S1-full.pdf">here</a>.</note> </supplement>
This abstract is available from: http://www.ped-rheum.com/content/6/S1/P204
© 2008 Toplak et al; licensee BioMed Central Ltd. 


Periodic fever syndrome with aphthous stomatitis, pharyngitis and cervical adenopathy treated with ketotifen – a case report

Periodic fever syndrome with aphthous stomatitis, pharyngitis and cervical adenopathy treated with ketotifen – a case report

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