Prompt response of refractory Schnitzler syndrome to treatment with anakinra

Clinical and histopathologic review of Schnitzler syndrome: The Mayo Clinic experience (1972-2011)

“…Since the initial report in 2005, 23 multiple studies (mostly with isolated or small numbers of patients) have described dramatic improvement within minutes or hours after infusion of anakinra. [23][24][25][26][27][28][29] The inclusion of patients given a diagnosis before 2005 explains why only 5 of our patients received anakinra; of those 5, only 1 patient had dramatic improvement, the second had minimal benefit, and 3 were lost to follow-up. Although anakinra is considered the treatment of choice, it is worth noting that dramatic clinical response to anakinra is not a criterion for diagnosis, nor is an absent response a contraindication for the diagnosis.…”

Schnitzler’s Syndrome: Monoclonal Gammopathy Associated with Chronic Urticaria

“…At present, the most promising therapeutic options are novel agents, such as thalidomide and anakinra [17]. Particularly anakinra, a synthetic analog of the endogeneous IL-1 receptor antagonist, is considered the treatment of choice in patients with severe manifestations [2, 16, 18, 19]. The risk of SS evolution to LPD after a latency period was of concern in our patient and underlined the need for periodic long-term monitoring.…”

Schnitzler-Syndrom

“…Dies belegt das charakteristische prompte Ansprechen auf eine therapeutische Blockade des entsprechenden Signalweges, welche 2005 erstmals publiziert und inzwischen vielfach in Fallberichten bestätigt wurde [3, 8,9,15,16,20,21]. In vitro konnte eine verstärkte IL-1β-Sekretion aus stimulierten mononukleären Zellen des peripheren Blutes betroffener Patienten nachgewiesen werden [15,19].…”