1. Ciprofloksacin, doksiciklin i anakinra u liječenju Schnitzlerovog sindroma
- Author
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Alemka Markotić and Višnja Škerk
- Subjects
Schnitzler syndrome ,anakinra ,ciprofloxacin ,doxycycline ,Schnitzlerov sindrom ,ciprofloksacin ,doksiciklin - Abstract
Schnitzlerov sindrom se manifestira istovremenom pojavom monoklonske gamapatije i kronične urtikarije kao glavnih simptoma s najmanje dva dodatna sporedna simptoma bolesti: artralgija, kostobolja, vrućica nepoznatog porijekla, hepato- ili splenomegalija, te povećana gustoća kostiju. Etiologija bolesti kao i imunopatogeneza nisu jasni, ali su pokazane povišene vrijednosti IL-1 u nekih bolesnika. Jedina uspješna terapija do sada je primjena anakinre [rekombinantni, neglikozilirani oblik humanog IL-1 antagonista receptora (IL-1RA)]. Ovdje smo prikazali bolesnicu s dijagnosticiranim Schnitzlerovim sindromom koja je inicijalno liječena tri godine kombinacijom ciprofloksacina i doksiciklina, a nakon toga anakinrom. Djelomična remisija u smislu značajne regresije osipa, smanjene kostobolje i povremenog subfebriliteta zabilježena je tijekom trogodišnjeg per os liječenja kombinacijom ciprofloksacina 2 × 500 mg i doksiciklina 2 × 100 mg uz desloratadin 1 × 5 mg. Svaki prekid ove terapije rezultirao bi promptnom pojavom jake urtikarije, febriliteta i pogoršanja nalaza (povišene vrijednosti sedimentacije i CRP-a, leukocitoza i neutrofilija). Ovu uspješnu kontrolu simptoma i nalaza pripisujemo sinergističkom djelovanju ciprofloksacina i doksiciklina i njihovoj vjerojatnoj imunomodulatornoj ulozi u sniženju razine proupalnih citokina, uključujući i IL-1. Značajno poboljšanje, međutim u bolesnice je uslijedilo nakon primjene anakinre. Tijekom dvogodišnjeg liječenja osim prolazne blage jetrene lezije nisu zabilježene nuspojave, kao ni simptomi bolesti, a laboratorijski nalazi su u granicama normale., Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria as major symptoms with at least two additional minor symptoms (arthralgia, arthritis, fever of unknown origin, hepato- or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate, leukocytosis/thrombocytosis or increased bone density). The etiology and immunopathogenesis of disease is unknown, but increased levels of interleukine (IL)-1 have been detected in some patients. The most successful therapy was recorded with anakinra, recombinant, nonglycosylated form of the human interleukin-1 receptor antagonist (IL-1RA). Here we describe a female patient with Schnitzler syndrome who was initially treated for three years with a combination of ciprofloxacin and doxycycline, followed by anakinra. Partial remission in terms of significant regression of the rash, reduced arthritis and occasional subfebrility was recorded during the three-year per os combination treatment with 2 × 500 mg ciprofloxacin, 2 × 100 mg doxycycline and 1 × 5 mg desloratadine. Any interruption of this therapy would result in a prompt appearance of severe urticaria, fever, and worsening of the findings (elevated ESR and CRP, leukocytosis and neutrophilia). This successful control of the symptoms and findings was attributed to a synergistic effect of ciprofloxacin and doxycycline and their probable immunomodulatory role in lowering the levels of proinflammatory cytokines, including IL-1. A significant improvement, however, was recorded in our patient after anakinra administration. During the two-year treatment, apart from transient, mild hepatic lesions, no side effects or symptoms have been reported, and laboratory findings were within normal range.
- Published
- 2011