325 results match your criteria Schnitzler Syndrome


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
2 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
1 Read

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
6 Reads

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

Br J Dermatol 2019 Apr;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
1 Read

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
14 Reads

Monoclonal gammopathy, arthralgias, and recurrent fever syndrome: a new autoinflammatory syndrome?

J Rheumatol 2019 03 15. Epub 2019 Mar 15.

Hôpital Tenon (AP-HP) - Service de Médecine Interne, Paris, France

Objective: The objective of this work was 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 included in the description. 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
March 2019
3 Reads

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

Ocul Immunol Inflamm 2019 Feb 22:1-3. Epub 2019 Feb 22.

c 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
1 Read

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

Scand J Rheumatol 2019 Jan 22:1-4. 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
January 2019
3 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
11 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
2 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
February 2019
30 Reads

Association of CCL2 with systemic inflammation in Schnitzler syndrome.

Br J Dermatol 2019 Apr 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
30 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
7 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
5 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 Sep 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
5 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
13 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
2 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
June 2018
9 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
3 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
9 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
4 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
8 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
38 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
4 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
11 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
21 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
11 Reads

[Refractory urticaria: Schnitzler syndrome].

Emergencias 2017 Abr;29(2):137

Unidad de Gestión Clínica Dermatología y Venereología, Hospital Universitario Puerta del Mar, Servicio Andaluz de Salud, Cádiz, España.

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December 2018
9 Reads

Schnitzler syndrome: A rare cause of chronic recalcitrant urticaria successfully treated with Anakinra.

Australas J Dermatol 2018 Aug 12;59(3):e222-e224. Epub 2017 Oct 12.

Department of Dermatology, Fiona Stanley Hospital, Perth, Western Australia, Australia.

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http://dx.doi.org/10.1111/ajd.12731DOI Listing
August 2018
16 Reads
0.980 Impact Factor

Aortitis: A new feature of Schnitzler syndrome.

JAAD Case Rep 2017 Sep 8;3(5):454-456. Epub 2017 Sep 8.

Department of Dermatology, University Hospital of Strasbourg, Strasbourg, France.

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http://dx.doi.org/10.1016/j.jdcr.2017.06.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5602823PMC
September 2017
13 Reads

Radiotherapy for a breast cancer patient with Schnitzler syndrome: Report of acute toxicity and early follow-up.

Rep Pract Oncol Radiother 2017 Nov-Dec;22(6):463-469. Epub 2017 Sep 8.

Radiation Oncologist at Hospital Sírio-Libanês, São Paulo, Brazil.

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 with radiotherapy along with urticarial-like lesions might be challenging in view of the interaction between symptoms and therapeutic measures. Read More

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http://dx.doi.org/10.1016/j.rpor.2017.08.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5596303PMC
September 2017
19 Reads

Deregulation of levels of angiopoietin-1 and angiopoietin-2 is associated with severe courses of hantavirus infection.

J Clin Virol 2017 09 11;94:33-36. Epub 2017 Jul 11.

Department of Nephrology, University of Heidelberg, Heidelberg, Germany. Electronic address:

Background: Hantavirus disease is characterized by endothelial dysfunction. Angiopoietin-1 (Ang-1) and its antagonist angiopoietin-2 (Ang-2) play a key role in the control of capillary permeability. Ang-1 is responsible for maintenance of cell-to-cell contacts whereas Ang-2 destabilizes monolayers. Read More

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http://dx.doi.org/10.1016/j.jcv.2017.07.007DOI Listing
September 2017
21 Reads

Schnitzler Syndrome With Response to Anakinra Monotherapy: 7 Years of Follow-up.

Actas Dermosifiliogr 2017 Dec 17;108(10):956-958. Epub 2017 Jul 17.

Servicio de Anatomía Patológica, Hospital de Cabueñes, Gijón, España.

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http://dx.doi.org/10.1016/j.ad.2017.03.015DOI Listing
December 2017
6 Reads

Schnitzler Syndrome: a Review.

Authors:
L Gusdorf D Lipsker

Curr Rheumatol Rep 2017 Aug;19(8):46

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

Purpose Of Review: We focus on recent advances in diagnosis and therapeutic strategies, as well as on pathogenesis of Schnitzler syndrome.

Recent Findings: New diagnostic criteria were established, and their external validity was assessed in a retrospective cohort study. The cytokine interleukin-1 (IL-1) plays a crucial role in the pathogenesis of the Schnitzler syndrome, and this explains the spectacular efficiency of IL-1 blocking therapies. Read More

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http://dx.doi.org/10.1007/s11926-017-0673-5DOI Listing
August 2017
22 Reads

Schnitzler Syndrome With Delirium and Vertigo: The Utility of Neurologic Manifestations in Diagnosis.

J Drugs Dermatol 2017 Jun;16(6):625-627

Schnitzler syndrome (SS) is an autoinflammatory dermatosis that often goes undiagnosed for 5-6 years. Patients typically carry a diagnosis of urticaria; however, their cutaneous symptoms fail to respond to typical urticaria therapies and lack symptoms such as pruritus. Additionally, patients with SS may see multiple providers for nonspecific complaints of fever, lymphadenopathy, arthralgias, and bone pain. Read More

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June 2017
9 Reads

Use of skin biomarker profiles to distinguish Schnitzler syndrome from chronic spontaneous urticaria: results of a pilot study.

Br J Dermatol 2018 02 28;178(2):561-562. Epub 2017 Nov 28.

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

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http://doi.wiley.com/10.1111/bjd.15705
Publisher Site
http://dx.doi.org/10.1111/bjd.15705DOI Listing
February 2018
10 Reads

Beyond Urticaria: Schnitzler Syndrome.

Balkan Med J 2017 09 29;34(5):478-479. Epub 2017 May 29.

Clinic of Internal Medicine, Granada Hospital, Granada, Spain.

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http://dx.doi.org/10.4274/balkanmedj.2017.0259DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5635639PMC
September 2017
14 Reads

Interleukin-1 Blockade: An Update on Emerging Indications.

BioDrugs 2017 Jun;31(3):207-221

Department of Infectious Diseases and Immunity, Jessa Hospital, Stadsomvaat 11, 3500, Hasselt, Belgium.

Interleukin (IL)-1 is a pro-inflammatory cytokine that induces local and systemic inflammation aimed to eliminate microorganisms and tissue damage. However, an increasing number of clinical conditions have been identified in which IL-1 production is considered inappropriate and IL-1 is part of the disease etiology. In autoinflammatory diseases, gout, Schnitzler's syndrome, and adult-onset Still's disease, high levels of inappropriate IL-1 production have been shown to be a key process in the etiology of the disease. Read More

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http://dx.doi.org/10.1007/s40259-017-0224-7DOI Listing
June 2017
40 Reads

Biologic Therapy in the Treatment of Chronic Skin Disorders.

Immunol Allergy Clin North Am 2017 05 1;37(2):315-327. Epub 2017 Mar 1.

Department of Allergy and Clinical Immunology, Respiratory Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA. Electronic address:

Understanding of the immunologic pathways involved in the pathogenesis of skin-related diseases is constantly advancing. Several biologic agents play important therapeutic roles for management of patients with chronic urticaria, atopic dermatitis, and psoriasis, particularly omalizumab for antihistamine-resistant chronic urticaria, interleukin (IL)-1 inhibitors for cryopyrin-associated periodic syndrome and Schnitzler syndrome, dupilumab for recalcitrant atopic dermatitis, and IL-17 inhibitors for psoriasis. The therapeutic utility of biologic agents for patients with immune-related dermatologic disorders is likely to expand in the future. Read More

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http://dx.doi.org/10.1016/j.iac.2017.01.006DOI Listing
May 2017
27 Reads

Disorders characterized by predominant or exclusive dermal inflammation.

Authors:
Mark R Wick

Semin Diagn Pathol 2017 May 14;34(3):273-284. Epub 2016 Dec 14.

Section of Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Room 3020 University of Virginia Hospital, 1215 Lee Street, Charlottesville, VA 22908-0214, USA. Electronic address:

Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07402570163010
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http://dx.doi.org/10.1053/j.semdp.2016.12.002DOI Listing
May 2017
37 Reads

Glutamine triggers long-lasting increase in striatal network activity in vitro.

Exp Neurol 2017 04 6;290:41-52. Epub 2017 Jan 6.

Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine-University, Duesseldorf, Germany; Institute of Neuro- und Sensory Physiology, Medical Faculty, Heinrich-Heine-University, Duesseldorf, Germany.

Accumulation of ammonium and glutamine in blood and brain is a key factor in hepatic encephalopathy (HE) - a neuropsychiatric syndrome characterized by various cognitive and motor deficits. MRI imaging identified abnormalities notably in the basal ganglia of HE patients, including its major input station, the striatum. While neurotoxic effects of ammonia have been extensively studied, glutamine is primarily perceived as "detoxified" form of ammonia. Read More

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http://dx.doi.org/10.1016/j.expneurol.2017.01.003DOI Listing
April 2017
7 Reads

Clinical characterization of two severe cases of hemorrhagic fever with renal syndrome (HFRS) caused by hantaviruses Puumala and Dobrava-Belgrade genotype Sochi.

BMC Infect Dis 2016 Nov 14;16(1):675. Epub 2016 Nov 14.

Department of Nephrology, University of Heidelberg, Im Neuenheimer Feld 162, Heidelberg, 69120, Germany.

Background: Hantavirus disease belongs to the emerging infections. The clinical picture and severity of infections differ between hantavirus species and may even vary between hantavirus genotypes. The mechanisms that lead to the broad variance of severity in infected patients are not completely understood. Read More

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http://dx.doi.org/10.1186/s12879-016-2012-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5109704PMC
November 2016
17 Reads

Inflammasomes and dermatology.

An Bras Dermatol 2016 Sep-Oct;91(5):566-578

Universidade de São Paulo (USP) - São Paulo (SP), Brazil.

Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases. Autoinflammatory diseases refer to disorders in which local factors lead to the activation of innate immune cells, causing tissue damage when in the absence of autoantigens and autoantibodies. Read More

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http://dx.doi.org/10.1590/abd1806-4841.20165577DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087212PMC
May 2017
20 Reads

Efficacy and safety of canakinumab in Schnitzler syndrome: A multicenter randomized placebo-controlled study.

J Allergy Clin Immunol 2017 Apr 19;139(4):1311-1320. Epub 2016 Sep 19.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Berlin, Germany; Autoinflammation Reference Center Charité, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Background: 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.

Objective: We assessed effects of the anti-IL-1β mAb canakinumab on the clinical signs and symptoms of Schnitzler syndrome. Read More

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http://dx.doi.org/10.1016/j.jaci.2016.07.041DOI Listing
April 2017
26 Reads

[Schnitzlers SyndromeDifferential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra].

Vnitr Lek Fall 2016;62(9):713-727

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. Read More

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June 2017
58 Reads

The Schnitzler syndrome: chronic urticaria in disguise: a single-centre report of 11 cases and a critical reappraisal of the literature.

Clin Exp Rheumatol 2017 Jan-Feb;35(1):69-73. Epub 2016 Aug 31.

Department of General Internal Medicine, University Hospitals Leuven, Belgium.

Objectives: The Schnitzler syndrome is a rare inflammatory disorder, with a chronic urticaria-like rash and an IgM (rarely IgG) monoclonal gammopathy as cardinal features. Interleukin-1 β is regarded as the key mediator and the interleukin-1 receptor antagonist anakinra has been proposed as first-line treatment. This case series of eleven patients is intended to enhance disease awareness and to compare our centre's experience with that of literature. Read More

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June 2017
17 Reads

Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients.

Allergy 2017 Feb 12;72(2):177-182. Epub 2016 Sep 12.

Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.

Background: 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.

Methods: 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. Read More

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http://dx.doi.org/10.1111/all.13035DOI Listing
February 2017
5 Reads