title: [Schnitzlers SyndromeDifferential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra].
abstract: Schnitzlers 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 - Schnitzlers Syndrome - Adult Stills disease.
authors.forename: Zdeněk
authors.initials: Z
authors.lastname: Adam
authors.forename: Anna
authors.initials: A
authors.lastname: Šedivá
authors.forename: Renata
authors.initials: R
authors.lastname: Koukalová
authors.forename: Zdeněk
authors.initials: Z
authors.lastname: Řehák
authors.forename: Hana
authors.initials: H
authors.lastname: Petrášová
authors.forename: Petr
authors.initials: P
authors.lastname: Szturz
authors.forename: Zdenka
authors.initials: Z
authors.lastname: Adamová
authors.forename: Eva
authors.initials: E
authors.lastname: Vetešníková
authors.forename: Luděk
authors.initials: L
authors.lastname: Pour
authors.forename: Marta
authors.initials: M
authors.lastname: Krejčí
authors.forename: Viera
authors.initials: V
authors.lastname: Sandecká
authors.forename: Eva
authors.initials: E
authors.lastname: Pourová
authors.forename: Zdeňka
authors.initials: Z
authors.lastname: Čermáková
authors.forename: Sabina
authors.initials: S
authors.lastname: Ševčíková
authors.forename: Zdeněk
authors.initials: Z
authors.lastname: Král
authors.forename: Jiří
authors.initials: J
authors.lastname: Mayer
mesh_terms: D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D019873:Schnitzler Syndrome / Q000188:drug therapy*
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein