345 results match your criteria Schnitzler Syndrome


Somatosensory area 3b is selectively unaffected in corticobasal syndrome: combining MRI and histology.

Neurobiol Aging 2020 May 21;94:89-100. Epub 2020 May 21.

Institute for Anatomy I, Medical Faculty, Heinrich Heine University of Düsseldorf, Düsseldorf, Germany; Institute of Neuroscience and Medicine (INM-1), Research Centre Jülich, Jülich, Germany; JARA-BRAIN, Jülich-Aachen Research Alliance, Research Centre Jülich, Jülich, Germany. Electronic address:

An increasing number of neuroimaging studies addressing patients with corticobasal syndrome use macroscopic definitions of brain regions. As a closer link to functionally relevant units, we aimed at identifying magnetic resonance-based atrophy patterns in regions defined by probability maps of cortical microstructure. For this purpose, three analyses were conducted: (1) Whole-brain cortical thickness was compared between 36 patients with corticobasal syndrome and 24 controls. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2020.05.009DOI Listing

A Chinese case series of Schnitzler syndrome and complete remission in one tocilizumab-treated patient.

Clin Rheumatol 2020 Jun 9. Epub 2020 Jun 9.

Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Schnitzler syndrome (SchS) is a rare acquired systemic autoinflammatory disease. The major clinical features of SchS are urticarial rash and monoclonal gammopathy, accompanied by fever, joint pain, and lymphadenopathy. There were few reports about SchS in Chinese population. Read More

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http://dx.doi.org/10.1007/s10067-020-05204-2DOI Listing

Efficacy and safety of canakinumab treatment in schnitzler syndrome: A systematic literature review.

Semin Arthritis Rheum 2020 May 25;50(4):636-642. Epub 2020 May 25.

Department of general internal medicine, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Laboratory of clinical infectious and inflammatory disease, department of Microbiology, Immunology and Transplantation, KU Leuven, Herestraat 49, 3000 Leuven, Belgium.

Background: Schnitzler syndrome is a rare autoinflammatory disorder characterized by chronic urticarial rash and a monoclonal gammopathy, accompanied by intermittent fever, bone pain, and arthralgia or arthritis. Canakinumab is a fully human monoclonal anti-interleukin-1β (IL-1β) antibody proven to be effective in IL-1 driven autoinflammatory disorders.

Methods: We systematically searched PubMed and Embase to include all types of studies on canakinumab treatment in Schnitzler syndrome published until March 16, 2020. Read More

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http://dx.doi.org/10.1016/j.semarthrit.2020.05.002DOI Listing

Long-term efficacy of canakinumab in the treatment of Schnitzler syndrome.

J Allergy Clin Immunol 2020 Jun 12;145(6):1681-1686.e5. Epub 2020 Jan 12.

Department of Dermatology and Allergy, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany.

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http://dx.doi.org/10.1016/j.jaci.2019.12.909DOI Listing

Hereditary systemic autoinflammatory diseases and Schnitzler's syndrome.

Rheumatology (Oxford) 2019 11;58(Suppl 6):vi31-vi43

National Institute for Health Research-Leeds Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, UK.

The systemic autoinflammatory diseases are disorders of the innate immune system distinguished by severe inflammation resulting from dysregulation of the innate immune system. Hereditary fever syndromes, such as FMF, TNF receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes and mevalonate kinase deficiency, were the first group of systemic autoinflammatory diseases for which a genetic basis was established, between 1999 and 2001. Currently according to the latest report of the international union of immunological societies, 37 separate monogenic disorders were classified as autoinflammatory. Read More

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http://dx.doi.org/10.1093/rheumatology/kez448DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6878846PMC
November 2019

Diagnostic value of 18-F fluorodeoxyglucose PET/CT and bone scan in Schnitzler syndrome.

Autoimmunity 2019 Nov - Dec;52(7-8):264-271. Epub 2019 Oct 24.

Internal Medicine Department, Rennes University Hospital, Rennes, France.

Schnitzler syndrome is an auto-inflammatory disease defined by chronic urticarial eruption and monoclonal gammopathy. F fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) is often performed, but its utility in Schnitzler syndrome has not been specifically investigated. The aim of this study was to determine whether PET/CT is informative in the diagnosis and follow-up of Schnitzler syndrome relative to other imaging techniques, including bone scans. Read More

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http://dx.doi.org/10.1080/08916934.2019.1680649DOI Listing
July 2020
1 Read

Spontaneous lymphoblastoid cell lines from patients with Epstein-Barr virus infection show highly variable proliferation characteristics that correlate with the expression levels of viral microRNAs.

PLoS One 2019 30;14(9):e0222847. Epub 2019 Sep 30.

German Cancer Research Centre (DKFZ) Unit F100, Heidelberg, Germany.

The Epstein-Barr virus (EBV) induces B-cell proliferation with high efficiency through expression of latent proteins and microRNAs. This process takes place in vivo soon after infection, presumably to expand the virus reservoir, but can also induce pathologies, e.g. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0222847PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6768455PMC
March 2020
3 Reads
3.234 Impact Factor

Neutrophilic urticarial dermatosis: an entity bridging monogenic and polygenic autoinflammatory disorders, and beyond.

Authors:
L Gusdorf D Lipsker

J Eur Acad Dermatol Venereol 2020 Apr 28;34(4):685-690. Epub 2019 Oct 28.

Faculté de Médecine, Université de Strasbourg, and Clinique Dermatologique, Hôpitaux Universitaires, Strasbourg, France.

Neutrophilic urticarial dermatosis (NUD) has been described in 2009. Clinically, it is an eruption of rose or red macules or slightly elevated plaques, vanishing within 24 h. It occurs mostly on the trunk and the limbs. Read More

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http://dx.doi.org/10.1111/jdv.15984DOI Listing
April 2020
2 Reads

Oral vancomycin treatment does not alter markers of postprandial inflammation in lean and obese subjects.

Physiol Rep 2019 08;7(16):e14199

Department of Vascular Medicine, Amsterdam UMC, Location AMC at University of Amsterdam, Amsterdam, The Netherlands.

Intake of a high-fat meal induces a systemic inflammatory response in the postprandial which is augmented in obese subjects. However, the underlying mechanisms of this response have not been fully elucidated. We aimed to assess the effect of gut microbiota modulation on postprandial inflammatory response in lean and obese subjects. Read More

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http://dx.doi.org/10.14814/phy2.14199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6698488PMC
August 2019
4 Reads

Cerebellar Involvement in DYT-THAP1 Dystonia.

Cerebellum 2019 Oct;18(5):969-971

Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Germany.

DYT-THAP1 dystonia is known to present a variety of clinical symptoms. To the best of our knowledge, this is the first case with DYT-THAP 1 dystonia and clinical signs of cerebellar involvement studied with transcranial magnetic stimulation in vivo. We report a case of a 51-year-old male DYT-THAP1 mutation carrier with dystonia, who additionally developed ataxia 1. Read More

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http://dx.doi.org/10.1007/s12311-019-01062-0DOI Listing
October 2019
3 Reads

Exploratory Study of MYD88 L265P, Rare NLRP3 Variants, and Clonal Hematopoiesis Prevalence in Patients With Schnitzler Syndrome.

Arthritis Rheumatol 2019 12 14;71(12):2121-2125. Epub 2019 Oct 14.

NIHR, Leeds, UK.

Objective: To assess the prevalence of the MYD88 L265P mutation and variants within NLRP3 and evaluate the status of oligoclonal hematopoiesis in 30 patients with Schnitzler syndrome (SchS).

Methods: Thirty patients with SchS were recruited from 3 clinical centers. Six patients with known acquired cryopyrin-associated periodic syndromes (aCAPS) were included as controls. Read More

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http://dx.doi.org/10.1002/art.41030DOI Listing
December 2019
9 Reads

[Membranoproliferative glomerulonephritis with relapsing episodes of acute kidney injury in the Schnitzler syndrome].

G Ital Nefrol 2019 Jun 11;36(3). Epub 2019 Jun 11.

Centro di Ricerca delle Malattie Rare e delle Febbri Periodiche, Fondazione Policlinico Gemelli, Università Cattolica, Roma.

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 auto-inflammatory acquired syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin 1 (IL-1) receptor antagonist anakinra. Read More

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June 2019
14 Reads

Schnitzler's syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström's disease.

Orphanet J Rare Dis 2019 06 22;14(1):151. Epub 2019 Jun 22.

Clinical Genetics, Maastricht University Medical Centre, Maastricht, The Netherlands.

Schnitzler's syndrome is an auto-inflammatory disorder which is characterized by two mandatory features: an urticarial rash and a monoclonal gammopathy. Although the pathophysiology of this syndrome is not yet fully understood, a role for interleukin-1 seems apparent. While this presumed link between interleukin-1 and the monoclonal gammopathy is not yet elucidated, a mutual factor in pathophysiology however seems likely. Read More

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http://dx.doi.org/10.1186/s13023-019-1117-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6589170PMC
June 2019
1 Read

A peek into premonitory urges in Tourette syndrome: Temporal evolution of neurophysiological oscillatory signatures.

Parkinsonism Relat Disord 2019 08 31;65:153-158. Epub 2019 May 31.

Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Düsseldorf University, Germany.

Background: Tics are the core symptom of patients with Gilles de la Tourette syndrome, yet the spatial-temporal dynamics of neural activity causing a tic remains to be determined.

Objective: Identification of cortical events preceding tic onset.

Methods: In twelve patients with Tourette syndrome we performed magnetoencephalography to trace the time course of beta oscillations (15-30 Hz) in motor cortical areas before tic onset. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2019.05.039DOI Listing
August 2019
5 Reads

Schnitzler syndrome.

Hautarzt 2019 Jun 5. Epub 2019 Jun 5.

Department of Dermatology, University Hospital Dresden, Fetscherstr. 74, 01307, Dresden, Germany.

Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and IgM monoclonal gammopathy. Other clinical features include fever, muscle, bone, and/or joint pain, and lymphadenopathy. Read More

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http://link.springer.com/10.1007/s00105-019-4434-4
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http://dx.doi.org/10.1007/s00105-019-4434-4DOI Listing
June 2019
11 Reads

Variable Responses to Tocilizumab in Four Patients with Schnitzler Syndrome.

J Clin Immunol 2019 05 20;39(4):370-372. Epub 2019 May 20.

Clinical Department of General Internal Medicine, University Hospitals Leuven, Herestraat 49, B-3000, Leuven, Belgium.

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http://dx.doi.org/10.1007/s10875-019-00644-1DOI Listing
May 2019
6 Reads

Refractory urticaria and the importance of diagnosing Schnitzler's syndrome.

BMJ Case Rep 2019 Apr 29;12(4). Epub 2019 Apr 29.

Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

A 52-year-old man presented with chronic urticaria that was refractory to standard chronic spontaneous urticaria (CSU) treatment. Over time, he developed systemic symptoms including fatigue, weight loss, arthralgia and bone pain. His laboratory investigations also became significant for microcytic anaemia, neutrophilia and elevated C reactive protein, erythrocyte sedimentation rate and IgE levels, in addition to an IgM monoclonal protein. Read More

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http://dx.doi.org/10.1136/bcr-2018-228546DOI Listing
April 2019
33 Reads

Schnitzler syndrome associated with L265P mutation.

JAAD Case Rep 2019 Apr 1;5(4):312-316. Epub 2019 Apr 1.

Department of Medicine, Division of Hematology/Oncology, University of California San Diego, La Jolla, California.

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https://linkinghub.elsevier.com/retrieve/pii/S23525126193005
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http://dx.doi.org/10.1016/j.jdcr.2019.02.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6446129PMC
April 2019
27 Reads

Skin and Systemic Inflammation in Schnitzler's Syndrome Are Associated With Neutrophil Extracellular Trap Formation.

Front Immunol 2019 22;10:546. Epub 2019 Mar 22.

Department of Dermatology and Allergy, Allergie-Centrum-Charité, Berlin Institute of Health, Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany.

Schnitzler's syndrome is a rare autoinflammatory disorder characterized by interleukin-1ß-mediated and neutrophil-dominated inflammation. Neutrophil extracellular traps (NETs) are web-like structures of decondensed chromatin, histones, and antimicrobial peptides released by neutrophils. NETs were initially described in the context of pathogen defense but are also involved in autoimmune-mediated skin diseases. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00546DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6438918PMC
March 2019
7 Reads

State of care for patients with systemic autoinflammatory diseases - Results of a tertiary care survey.

World Allergy Organ J 2019 14;12(3):100019. Epub 2019 Mar 14.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Germany.

Background: Systemic autoinflammatory diseases (SAIDs) are rare debilitating disorders of which there is limited awareness and a significant delay in diagnosis. There is no uniform approach in the diagnosis and treatment of these disorders and the real life state of SAID patient care is poorly characterized. The aim of this study was to obtain data on the epidemiology, state of care and the perception of physicians who are involved in the care of SAID patients. Read More

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http://dx.doi.org/10.1016/j.waojou.2019.100019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439415PMC
March 2019
28 Reads

CCL2 serves as a downstream effector of interleukin-1β and tumour necrosis factor-α signalling in Schnitzler syndrome.

Br J Dermatol 2019 04;180(4):706-707

Department of Dermatology and Venereology, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh, Vietnam.

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http://dx.doi.org/10.1111/bjd.17503DOI Listing
April 2019
8 Reads

Antithrombotic Therapy after Acute Coronary Syndrome or PCI in Atrial Fibrillation.

N Engl J Med 2019 04 17;380(16):1509-1524. Epub 2019 Mar 17.

From the Duke Clinical Research Institute, Duke University School of Medicine, Durham, NC (R.D.L., G.H., A.N.V., T.M., C.B.G., J.H.A.); Bristol-Myers Squibb, Princeton, NJ (R.A., J.L.); Zena and Michael A. Wiener Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, and Cardiovascular Research Foundation, New York (R.M.); Canadian VIGOUR Centre, University of Alberta, Edmonton (S.G.G.), and the Terrence Donnelly Heart Centre, St. Michael's Hospital, University of Toronto, Toronto (S.G.G.) - both in Canada; Swiss Cardiovascular Center, Bern, Switzerland (S.W.); Vivantes Neukoelln Medical Center, Berlin (H.D.), and Heart Center Leipzig, Department of Internal Medicine-Cardiology, University of Leipzig, Leipzig (H.T.) - both in Germany; Pirogov Russian National Research Medical University, Moscow (O.A.); Instituto de Neurología Cognitiva (INECO) Neurociencias Oroño, Fundación INECO, Rosario, Argentina (M.C.B.); Hospital Israelita Albert Einstein, São Paulo (O.B.); Postgraduate Medical School, Grochowski Hospital, Warsaw, Poland (A.B.); the Department of Medical Sciences, Cardiology, and Uppsala Clinical Research Center, Uppsala University, Uppsala, Sweden (Z.H.); the National Scientific Center, Strazhesko Institute of Cardiology, Kiev, Ukraine (A.P.); University Hospitals Leuven, University of Leuven, Leuven, Belgium (P.S.); the Department of Infection, Immunity, and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom (R.F.S.); and University of Medicine and Pharmacy Carol Davila, University and Emergency Hospital, Bucharest, Romania (D.V.).

Background: Appropriate antithrombotic regimens for patients with atrial fibrillation who have an acute coronary syndrome or have undergone percutaneous coronary intervention (PCI) are unclear.

Methods: In an international trial with a two-by-two factorial design, we randomly assigned patients with atrial fibrillation who had an acute coronary syndrome or had undergone PCI and were planning to take a P2Y inhibitor to receive apixaban or a vitamin K antagonist and to receive aspirin or matching placebo for 6 months. The primary outcome was major or clinically relevant nonmajor bleeding. Read More

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http://dx.doi.org/10.1056/NEJMoa1817083DOI Listing
April 2019
46 Reads

Monoclonal Gammopathy, Arthralgias, and Recurrent Fever Syndrome: A New Autoinflammatory Syndrome?

J Rheumatol 2019 11 15;46(11):1535-1539. Epub 2019 Mar 15.

From the Sorbonne Université, AP-HP, Hôpital Tenon, Service de Médecine Interne, Centre de Référence des Maladies Auto-inflammatoires et des Amyloses d'Origine Inflammatoire (CEREMAIA); Service d'Immuno-hélatologie, Hôpital St. Louis; INSERM UMRS-933, et Laboratoire de Génétique, Hôpital Trousseau, AP-HP, Faculté de Médecine, Sorbonne Université, Paris; Service de Médecine Interne, Centre Hospitalier d'Auxerre, Auxerre; Service de Médecine Interne, Centre Hospitalier Universitaire (CHU) Henri Mondor; and Service d'Immunologie Clinique, Hôpital St. Louis, Paris, France.

Objective: To describe a new autoinflammatory syndrome with recurrent fever and monoclonal gammopathy that differs from Schnitzler syndrome.

Methods: We conducted a retrospective study of patients with monoclonal gammopathy and recurrent fever of unknown origin.

Results: Five patients were studied; median age at onset of symptoms was 44 years. Read More

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http://www.jrheum.org/lookup/doi/10.3899/jrheum.181204
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http://dx.doi.org/10.3899/jrheum.181204DOI Listing
November 2019
13 Reads

The First Case Report of Schnitzler Syndrome Presenting with Eye Pain.

Ocul Immunol Inflamm 2020 22;28(2):175-177. Epub 2019 Feb 22.

Save Sight Institute, University of Sydney, Sydney, NSW, Australia.

Schnitzler syndrome is a rare, auto inflammatory condition known to manifest with bone pain, urticarial rash, fevers, relapsing arthralgia, and fatigue. In this case report, we describe a patient who was diagnosed with Schnitzler Syndrome that had initially presented with a unilateral pressure-type headache with a sensation of a 'dagger' stabbing into the back of the eye. He also had an associated ipsilateral redness of the conjunctiva, eyelid swelling, subtle optic disc elevations bilaterally and facial flushing - but with no visual acuity, pupillary, or lacrimatory changes. Read More

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http://dx.doi.org/10.1080/09273948.2018.1563704DOI Listing
February 2019
9 Reads

Cytokines and soluble receptors of the interleukin-1 family in Schnitzler syndrome.

Scand J Rheumatol 2019 May 22;48(3):235-238. Epub 2019 Jan 22.

b Institute of Protein Biochemistry , National Research Council , Naples , Italy.

Objectives: Schnitzler syndrome (SchS) is an autoinflammatory disorder characterized by chronic urticaria, fever, and monoclonal gammopathy. The success of interleukin-1 (IL-1) blocking therapies suggests a crucial role for IL-1 in disease induction. The aim of this study is to perform a comprehensive analysis of IL-1 family cytokines and soluble receptors in a group of SchS patients. Read More

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http://dx.doi.org/10.1080/03009742.2018.1550210DOI Listing
May 2019
11 Reads

[Schnitzler syndrome].

Z Rheumatol 2019 Feb;78(1):43-54

Klinik und Poliklinik für Dermatologie, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Anstalt des öffentlichen Rechts des Freistaates Sachsen, Fetscherstr. 74, 01307, Dresden, Deutschland.

Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and a monoclonal gammopathy with IgM. Other clinical features include fever, muscle, bone and/or joint pain, and lymphadenopathy. Read More

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http://dx.doi.org/10.1007/s00393-019-0591-1DOI Listing
February 2019
21 Reads

[Diagnosis and treatment of Schnitzler syndrome].

Zhonghua Xue Ye Xue Za Zhi 2018 Dec;39(12):1052-1056

Department of Hematology, Affiliated Zhongshan Hospital of Dalian University, Dalian 116000, China.

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2018.12.018DOI Listing
December 2018
9 Reads

Schnitzler's syndrome: A female elderly case presenting intractable non-pruritic febrile urticarial rush.

Asian Pac J Allergy Immunol 2020 Mar;38(1):64-66

Graduate School of Medical Sciences, Department of Dermatology, Kyushu University, Fukuoka, Japan.

Schnitzler's syndrome is an acquired autoinflammatory disease characterized by chronic urticarial rash and monoclonal gammopathy (predominantly IgM type). A 75-year-old Japanese woman complained of high fever and non-pruritic urticarial rash appearing almost every day for 3 years. Her abnormal laboratory data included leukocytosis and neutrophilia with elevated erythrocyte sedimentation rate and C-reactive protein level. Read More

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http://dx.doi.org/10.12932/AP-150818-0394DOI Listing
March 2020
32 Reads

Low-Dose Methotrexate for the Prevention of Atherosclerotic Events.

N Engl J Med 2019 02 10;380(8):752-762. Epub 2018 Nov 10.

From the Center for Cardiovascular Disease Prevention, Division of Preventive Medicine (P.M.R., B.M.E., A.P., J.G.M., E.Z., V.M., N.P.P., R.J.G.), and the Divisions of Cardiovascular Medicine (P.M.R., B.M.E., P.L., S.Z.G.) and Rheumatology (D.H.S.), Brigham and Women's Hospital, Boston; the National Heart, Lung, and Blood Institute, Bethesda, MD (A.H., Y.R., E.I.); McMaster University, Hamilton (M.G.), the Canadian Collaborative Research Network, Brampton (M.T.), St. Michael's Hospital, Toronto (S.V.), the University of Ottawa Heart Institute, Ottawa (M.L.M.), and KMH Cardiology, Diagnostic and Research Centres, Mississauga (J.J.), ON, and Laval University, Quebec City, QB (O.B.) - all in Canada; Touro University, Vallejo, CA (M.C.); Cardiology Associates Carolina, Morganton, NC (R.S.); Wooster Community Hospital, Wooster (C.O.), and Dayton Veteran Affairs Medical Center, Dayton (M.S.) - both in Ohio; Verde Valley Medical Center, Cottonwood, AZ (S.B.); and Atlanta Heart Specialists, Atlanta (N.S.).

Background: Inflammation is causally related to atherothrombosis. Treatment with canakinumab, a monoclonal antibody that inhibits inflammation by neutralizing interleukin-1β, resulted in a lower rate of cardiovascular events than placebo in a previous randomized trial. We sought to determine whether an alternative approach to inflammation inhibition with low-dose methotrexate might provide similar benefit. Read More

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http://www.nejm.org/doi/10.1056/NEJMoa1809798
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http://dx.doi.org/10.1056/NEJMoa1809798DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6587584PMC
February 2019
60 Reads

Association of CCL2 with systemic inflammation in Schnitzler syndrome.

Br J Dermatol 2019 04 5;180(4):859-868. Epub 2018 Dec 5.

Interdisciplinary Group of Molecular Immunopathology, Dermatology/Medical Immunology and, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.

Background: 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 largely unknown.

Objectives: To identify and characterize the pathogenetic players in SchS. Read More

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http://dx.doi.org/10.1111/bjd.17334DOI Listing
April 2019
45 Reads

Neutrophilic urticarial dermatosis: A review.

Authors:
L Gusdorf D Lipsker

Ann Dermatol Venereol 2018 Dec 14;145(12):735-740. Epub 2018 Sep 14.

Dermatology Department, Hôpitaux Universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg, France. Electronic address:

Neutrophilic urticarial dermatosis (NUD) is a rare form of dermatosis. In clinical terms, it consists of a chronic or recurrent eruption comprising slightly elevated, pink to reddish plaques or macules. The elementary lesion lasts 24 to 48hours and resolves without leaving any residual pigmentation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01519638183050
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http://dx.doi.org/10.1016/j.annder.2018.06.010DOI Listing
December 2018
14 Reads

[Schnitzler syndrome].

Hautarzt 2018 Sep;69(9):761-772

Klinik und Poliklinik für Dermatologie, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Anstalt des öffentlichen Rechts des Freistaates Sachsen, Fetscherstr. 74, 01307, Dresden, Deutschland.

Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and a monoclonal gammopathy with IgM. Other clinical features include fever, muscle, bone and/or joint pain, and lymphadenopathy. Read More

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http://dx.doi.org/10.1007/s00105-018-4250-2DOI Listing
September 2018
13 Reads

Schnitzler syndrome co-occurring with idiopathic multicentric Castleman disease that responds to anti-IL-1 therapy: A case report and clue to pathophysiology.

Curr Res Transl Med 2018 09 11;66(3):83-86. Epub 2018 Aug 11.

CHU Lille, Département de Médecine Interne et Immunologie Clinique, Lille, France.

Patients with HHV-8-negative/idiopathic multicentric Castleman disease (iMCD) experience systemic inflammatory symptoms and polyclonal lymphoproliferation due to an unknown etiology. Schnitzler's syndrome (SS) is characterized by recurrent urticarial rash, monoclonal IgM gammopathy, and other clinical signs of inflammation. To our knowledge, we report the first case of iMCD associated with SS and the fourth case of anakinra inducing a complete response for an iMCD patient. Read More

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http://dx.doi.org/10.1016/j.retram.2018.06.001DOI Listing
September 2018
14 Reads

Schnitzler Syndrome: A Case Report and Review of Literature.

Ann Dermatol 2018 Aug 28;30(4):483-485. Epub 2018 Jun 28.

Department of Dermatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

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https://synapse.koreamed.org/DOIx.php?id=10.5021/ad.2018.30.
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http://dx.doi.org/10.5021/ad.2018.30.4.483DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6029961PMC
August 2018
40 Reads

[Schnitzler syndrome: a case report].

Authors:
M Wang Z L Li

Zhonghua Xue Ye Xue Za Zhi 2018 Jun;39(6):523

China-Japan Friendship Hospital, Beijing 100029, China.

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2018.06.020DOI Listing
June 2018
8 Reads

Mimickers of Urticaria: Urticarial Vasculitis and Autoinflammatory Diseases.

J Allergy Clin Immunol Pract 2018 Jul - Aug;6(4):1162-1170. Epub 2018 Jun 2.

Department of Infectious Diseases and Immunity, Jessa Hospital. BIOMED Research Institute, University of Hasselt, Hasselt, Belgium.

A wide differential diagnosis must be considered in a patient presenting with urticarial plaques. Although acute and chronic urticaria are the commonest diagnoses, other differential diagnoses include polymorphous eruption of pregnancy, mast cell disorders, hypereosinophilic syndrome, urticarial vasculitis, pemphigoid, systemic lupus erythematosus, and autoinflammatory disease. This review will specifically address urticarial vasculitis and autoinflammatory syndromes. Read More

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http://dx.doi.org/10.1016/j.jaip.2018.05.006DOI Listing
October 2019
18 Reads

A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome.

Case Rep Rheumatol 2018 8;2018:7041576. Epub 2018 Mar 8.

Division of Rheumatology, Department of Medicine, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

Background: 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. Read More

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http://dx.doi.org/10.1155/2018/7041576DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5863343PMC
March 2018
9 Reads

[Clinical overview of auto-inflammatory diseases].

Rev Med Interne 2018 Apr 28;39(4):214-232. Epub 2018 Feb 28.

Service de médecine interne, université Paris 6, Pierre-et-Marie-Curie (UPMC), hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), 20, rue de la Chine, 75020 Paris, France; Inserm UMRS_933, université Pierre-et-Marie-Curie (UPMC)-Paris 6, hôpital Trousseau, Assistance publique-Hôpitaux de Paris, 75012 Paris, France; Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire (CEREMAIA), 75020 Paris, France.

Monogenic auto-inflammatory diseases are characterized by genetic abnormalities coding for proteins involved in innate immunity. They were initially described in mirror with auto-immune diseases because of the absence of circulating autoantibodies. Their main feature is the presence of peripheral blood inflammation in crisis without infection. Read More

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http://dx.doi.org/10.1016/j.revmed.2018.01.004DOI Listing
April 2018
15 Reads

The elusive pathogenesis of Schnitzler syndrome.

Blood 2018 03;131(9):944-946

Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo; University of Pavia.

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http://dx.doi.org/10.1182/blood-2018-01-824862DOI Listing
March 2018
13 Reads

Schnitzler syndrome in a patient with a family history of monoclonal gammopathy.

Dermatol Online J 2018 Jan 15;24(1). Epub 2018 Jan 15.

University of Texas Health Science Center, McGovern Medical School, Houston, Texas Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Schnitzler syndrome is a rare disease characterized by chronic urticaria and a monoclonal gammopathy, most commonly IgM with light chains of the kappa type. There are currently no known risk factorsassociated with development of the disease. We report a case of Schnitzler syndrome in a 48-year-old man with a family history of monoclonal gammopathies. Read More

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January 2018
17 Reads

Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome.

Blood 2018 03 28;131(9):974-981. Epub 2017 Dec 28.

National Amyloidosis Centre, University College London (UCL), London, United Kingdom.

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. Read More

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http://dx.doi.org/10.1182/blood-2017-10-810366DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5877784PMC
March 2018
75 Reads

[Schnitzler syndrome: A review of literature, the authors' observation, and experience with the interleukin-1 inhibitor canakinumab].

Ter Arkh 2017;89(11):111-115

V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.

The paper reviews the publications dealing with Schnitzler syndrome, a rare autoinflammatory disease, and describes the authors' own clinical observation. It describes the first Russian experience in successfully using the interleukin-1 inhibitor canakinumab to treat this disease. Read More

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http://dx.doi.org/10.17116/terarkh20178911111-115DOI Listing
March 2018
11 Reads

Kidney involvement in the Schnitzler syndrome, a rare disease.

Clin Kidney J 2017 Dec 26;10(6):723-727. Epub 2017 Jul 26.

Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico Gemelli, Catholic University, Rome, Italy.

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-inflammatory syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin-1 receptor antagonist anakinra. Read More

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http://dx.doi.org/10.1093/ckj/sfx077DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5716098PMC
December 2017
18 Reads

A Case of Schnitzler's Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra.

Yonsei Med J 2018 Jan;59(1):154-157

Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.

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 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. Read More

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http://dx.doi.org/10.3349/ymj.2018.59.1.154DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5725354PMC
January 2018
31 Reads

Pivot and Cluster: An Exercise in Clinical Reasoning.

J Gen Intern Med 2018 02 28;33(2):226-230. Epub 2017 Nov 28.

Department of Medicine, University of California San Francisco, San Francisco, CA, USA.

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http://dx.doi.org/10.1007/s11606-017-4216-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5789108PMC
February 2018
4 Reads

Schnitzler syndrome with IgG gammopathy and elevated IL-1β and IL-17 in skin biopsy.

Ann Allergy Asthma Immunol 2018 Jan 20;120(1):99-101. Epub 2017 Nov 20.

Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address:

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http://dx.doi.org/10.1016/j.anai.2017.10.006DOI Listing
January 2018
20 Reads