"Schnitzler syndrome"

Schnitzler syndrome refractory to anakinra: successful treatment with canakinumab. Schnitzler syndrome (SchS) is a rare autoimmune and inflammatory disease mediated by interleukin-1 beta (IL-1β). Recurrent monoclonal gammopathy and chronic urticarial rash are the symptoms required for diagnosis according to the Strasbourg criteria. The low prevalence of this syndrome (around 300 cases have been

Schnitzler Syndrome Schnitzler Syndrome: Practice Essentials, Background, Pathophysiology For YouNews & PerspectiveDrugs & DiseasesCME & EducationAcademyVideoDecision PointEdition:EnglishMedscapeEnglishDeutschEspañolFrançaisPortuguêsUKNewUnivadisLog In Sign Up It's Free!English EditionMedscape * English * Deutsch * Español * Français * Português * UKNewUnivadisXUnivadis from MedscapeRegisterLog =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA1MDc2MS1vdmVydmlldw==processing....Drugs & Diseases > Dermatology Schnitzler SyndromeUpdated: Mar 12, 2019 * Author: Brian J Thomas, MD; Chief Editor: Dirk M Elston, MD more... * * Share * Email * Print * FeedbackClose * Facebook * Twitter * LinkedIn * WhatsAppSections Schnitzler Syndrome * * Sections Schnitzler Syndrome * Overview * * * Practice Essentials * Background * Pathophysiology

Association of CCL2 with systemic inflammation in Schnitzler's syndrome. Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by urticarial exanthema, bone and joint alterations, fever and monoclonal gammopathy, which manifest mostly in the second half of life. It involves overactivation of the interleukin (IL)-1 system, but the exact pathophysiological pathways remain

Schnitzler Syndrome: A Case Report and Review of Literature

Schnitzler syndrome associated with hairy cell leukemia presenting with chronic urticaria and arthralgias

Increasing the Interval of Canakinumab Administration Effectively Supports the Remission of Schnitzler's Syndrome Schnitzler's syndrome (SchS) is a rare, disabling, autoinflammatory disorder characterized by recurrent urticarial rash and monoclonal IgM gammopathy. Interleukin-1 beta (IL-1) plays an important role in the pathophysiology of SchS. Only anecdotal reports demonstrate the efficiency

A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra. To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria

Molecular genetic investigation, clinical features and response to treatment in 21 patients with Schnitzler's syndrome. To date, the pathogenic mechanisms underlying Schnitzler syndrome remain obscure, in particular, the interplay between the monoclonal protein and increased interleukin-1β (IL-1β) production, although interest in the contribution of genetic factors has been fueled by detection of somatic mosaicism in 2 patients with the variant-type Schnitzler syndrome. At 2 specialist UK centers, we have identified 21 patients who fulfilled diagnostic criteria for Schnitzler syndrome with urticarial rash, fever, arthralgia, and bone pain; 47% reported weight loss, 40% fatigue, and 21% lymphadenopathy. An immunoglobulin M (IgM) κ paraprotein was detected in 86%; the remainder had IgM λ or IgG κ

The use of skin biomarker profiles to distinguish Schnitzler's syndrome from chronic spontaneous urticaria: results of a pilot study.

A Case of Schnitzler's Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 suggests that the correct diagnosis of this case is Schnitzler's syndrome without monoclonal gammopathy. For an adult patient with refractory chronic urticaria and systemic inflammation, Schnitzler's syndrome could be considered as a possible differential diagnosis. Although the typical form of Schnitzler's syndrome exhibits the presence of monoclonal gammopathy as a diagnostic criterion, monoclonal

Kidney involvement in the Schnitzler syndrome, a rare disease The Schnitzler syndrome (SS) is a rare and underdiagnosed entity that associates a chronic urticarial rash, monoclonal IgM (or sometimes IgG) gammopathy and signs and symptoms of systemic inflammation. During the past 45 years, the SS has evolved from an elusive little-known disorder to the paradigm of a late-onset acquired auto

Schnitzler syndrome, a rare autoinflammatory disease. Complete response to IL-1 blockade

Beyond Urticaria: Schnitzler Syndrome

Aortitis: A new feature of Schnitzler syndrome

Radiotherapy for a breast cancer patient with Schnitzler syndrome: Report of acute toxicity and early follow-up This article provides description about acute toxicity and early follow-up of one patient treated for breast cancer and Schnitzler syndrome. There are no previously reported cases exploring this interaction on medical literature. The expected radiodermitis to occur in the region treated

Schnitzler syndrome with delayed or without monoclonal gammopathy: A Systematic Review PROSPERO International prospective register of systematic reviews Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. The registrant confirms that the information supplied

Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome. Schnitzler syndrome is a rare autoinflammatory disease, which is defined by the presence of two major criteria: chronic urticaria and monoclonal immunoglobulin M (IgM) or immunoglobulin G gammopathy, in combination with at least two additional minor criteria: recurrent fever, leukocytosis and/or elevated C-reactive protein (CRP , already after the initiation of therapy with anakinra, the suspected diagnosis of Schnitzler syndrome could be confirmed by detection of IgM-gammopathy that was initially absent.

Dysregulation of proinflammatory versus anti-inflammatory human T_H17 cell functionalities in the autoinflammatory Schnitzler syndrome. T17 cells have so far been considered to be crucial mediators of autoimmune inflammation. Two distinct types of T17 cells have been described recently, which differed in their polarization requirement for IL-1β and in their cytokine repertoire. Whether hypothesized that IL-1β mediates the loss of anti-inflammatory T17 cell functionalities in patients with Schnitzler syndrome, an autoinflammatory disease. To assess proinflammatory versus anti-inflammatory T17 cell functions, we performed suppression assays and tested the effects of IL-1β dependent and independent T17 subsets on modulating proinflammatory cytokine secretion by monocytes. Patients

Schnitzler syndrome: Validation and applicability of diagnostic criteria in real life patients. Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophil-mediated inflammation. The aim of this study was to assess the applicability and validity of the existing diagnostic criteria in real-life patients . This multicentric study was conducted between 2009 and 2014 in 14 hospitals in which patients with Schnitzler syndrome or controls with related disorders were followed up. We compared the sensitivities and specificities and calculated the positive and negative predictive values of the Lipsker and of the Strasbourg criteria for the patients with Schnitzler syndrome and for the controls. We included 42 patients

Efficacy and safety of canakinumab in Schnitzler's syndrome: a multi-center randomized placebo-controlled study. Schnitzler syndrome is an adult-onset autoinflammatory disease characterized by urticarial exanthema and monoclonal gammopathy accompanied by systemic symptoms such as fever, bone, and muscle pain. Up to now, approved treatment options are not available. We assessed effects of the anti-IL-1β mAb canakinumab on the clinical signs and symptoms of Schnitzler syndrome. In this phase II, randomized placebo-controlled multicenter study, 20 patients with active disease enrolled in 4 German study centers. Patients were randomly assigned to receive single subcutaneous canakinumab 150 mg or placebo injections for 7 days, followed by a 16-week open-label phase with canakinumab injections