Schnitzler’s Syndrome
Differential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra

Czech version

Authors: Zdeněk Adam ¹; Anna Šedivá ²; Renata Koukalová ³; Zdeněk Řehák ³; Hana Petrášová ⁴; Petr Szturz ¹; Zdenka Adamová ⁵; Eva Vetešníková ¹; Luděk Pour ¹; Marta Krejčí ¹; Viera Sandecká ¹; Eva Pourová ⁶; Zdeňka Čermáková ⁷; Sabina Ševčíková ⁸; Zdeněk Král ¹; Jiří Mayer ¹
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice ¹; Ústav imunologie 2. LF UK a FN v Motole, Praha ²; Oddělení nukleární medicíny, centrum PET, RECAMO, Masarykova onkologického ústavu Brno ³; Radiologická klinika LF MU a FN Brno, pracoviště Bohunice ⁴; Ambulance pro děti a dorost, Obilní trh 9, Brno ⁵; Ambulace praktického lékaře pro dospělé, Pustiměř ⁶; Oddělení klinické biochemie FN Brno a Katedra laboratorních metod LF MU Brno ⁷; Ústav patologické fyziologie LF MU Brno ⁸
Published in: Vnitř Lék 2016; 62(9): 713-727
Category: Reviews

Overview

Schnitzler’s syndrome is an acquired auto-inflammatory disease of still unclear origin. The Strasbourg criteria were adopted (non-infectious fever, chronic urticaria, changes in the bone structure, leukocytosis and higher values of inflammatory markers – CRP and presence of monoclonal immunoglobulin mostly of type IgM, very rarely of IgG) to establish this diagnosis. The first-choice therapy for this disease is the blocking of interleukin-1 effects. In practice, the interleukin-1 receptor antagonist, anakinra, is the most commonly used. Currently reports also appear of the use of other medicines blocking the effect of interleukin-1, namely canakinumab and rilonacept. We have been treating 5 patients with anakinra (108, 72, 33, 32 and 1 months) on a long-term basis. In all the patients, we commenced administration of anakinra in a dose of 100 mg once a day. As a result of 100 mg being administered once a day, all symptoms went away completely in 4 patients, while they receded by about 75 % in 1 patient, without disappearing completely. This patient needs an increased dose of 2 ampoules per day on the days of spontaneously intensified medical ailments. After one year of treatment it turned out for one of the four patients whose symptoms had completely disappeared when administered the 100mg daily dose, that he only needed the respective dose of anakinra at 48-hour intervals. However this patient does not tolerate further extension of the intervals between dose administrations. 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 the treatment. The text discusses the differential diagnostics of the Schnitzler syndrome.

Key words:
anakinra – auto-inflammatory diseases – canakinumab – fever of unknown origin – FUO – interleukin 1 – cryopyrin-associated autoinflammatory syndrome (CAPS) – monoclonal gammopathy – rilonacept – Schnitzler’s Syndrome – Adult Still’s disease

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