Chronic recurrent urticaria, joint and bone pain, fever of unknown origin and monoclonal IgM = Schnitzler’s syndrome
Authors:
Z. Adam 1; R. Koukalová 2; M. Krejčí 1; Z. Čermáková 3; E. Vetešníková 1; L. Pour 1; V. Sandecká 1; M. Štork 1; Z. Řehák 2; E. Pourová 4; A. Čermák 5; Z. Král 1; J. Mayer 1
Authors‘ workplace:
Interní hematologická a onkologická klinika LF MU a FN Brno
1; Oddělení nukleární medicíny, centrum PET, RECAMO. Masarykův onkologický ústav v Brně
2; Oddělení klinické biochemie FN Brno a Katedra laboratorních metod LF MU Brno
3; Ambulance praktického lékaře pro dospělé, Pustiměř u Vyškova
4; Urologická klinika LF MU a FN Brno
5
Published in:
Transfuze Hematol. dnes,24, 2018, No. 2, p. 88-103.
Category:
Overview
Schnitzler’s syndrome is an acquired auto-inflammatory disease of as yet unclear origin. The Strasbourg criteria (non-infectious fever, chronic urticaria, changes in bone structure, leucocytosis, elevated inflammatory markers – CRP and the presence of monoclonal immunoglobulin mostly IgM, very rarely of IgG) were adopted to establish this diagnosis. First-line therapy for this disease involves blocking interleukin-1 effects. In practice, the interleukin-1 receptor antagonist, anakinra, is most commonly used. Currently, there have also been reports regarding the use of other interleukin-1 blockers, namely canakinumab and rilonacept. We are currently treating 5 men, median age at diagnosis 53 (42–60) years, with anakinra. Median duration of treatment is 56 (19–122) months. In all the patients, we began administration of anakinra at a dose of 100 mg once a day. Administration of 100 mg once a day, led to the complete resolution of symptoms in 4 patients, and a reduction by about 75 % in 1 patient. This patient required an increased dose of 2 ampoules per day when his symptoms intensified. After one year of treatment, one of the four patients whose symptoms had completely disappeared using the 100 mg daily dose required this same dose at an interval 48-hour hours. However, this patient does not tolerate further extension of the intervals between doses. We have not recorded any adverse effects of anakinra in the course of the treatment and no decline in the efficiency of anakinra has been observed: it acts as effectively now as it did at the beginning of treatment. The text discusses the differential diagnosis of Schnitzler syndrome.>
Key words:
Schnitzler’s syndrome – Adult Still’s disease – auto-inflammatory diseases – monoclonal gammopathy – interleukin 1 – anakinra – fever of unknown origin – FUO
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