Database : MEDLINE
Search on : Schnitzler and Syndrome [Words]
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[PMID]: 29214791
[Au] Autor:Ahn MJ; Yu JE; Jeong J; Sim DW; Koh YI
[Ad] Address:Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.
[Ti] Title:A Case of Schnitzler's Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra.
[So] Source:Yonsei Med J;59(1):154-157, 2018 Jan.
[Is] ISSN:1976-2437
[Cp] Country of publication:Korea (South)
[La] Language:eng
[Ab] Abstract: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. It became aggravated episodically and was accompanied by high fever, arthralgia, leukocytosis, and an elevated C-reactive protein and erythrocyte sedimentation rate. The episodes each lasted for over one week. Neutrophilic and eosinophilic inflammation was found on skin biopsy. However, serum and urine electrophoresis showed no evidence of monoclonal gammopathy. The cutaneous lesions were unresponsive to various kinds of anti-histamines, systemic glucocorticoids, colchicine, cyclosporine, dapsone, and methotrexate, which were administered over a span of 3 years immediately preceding successful treatment. A dramatic response, however, was observed after a daily administration of anakinra. This observation 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 gammopathy may be absent in an atypical form. In such a situation, an IL-1 antagonist should be effective for the management of chronic urticaria.
[Mh] MeSH terms primary: Interleukin 1 Receptor Antagonist Protein/therapeutic use
Paraproteinemias/complications
Schnitzler Syndrome/drug therapy
Urticaria/complications
[Mh] MeSH terms secundary: Aged
Blood Sedimentation
C-Reactive Protein/metabolism
Chronic Disease
Humans
Leukocytes/metabolism
Male
Schnitzler Syndrome/blood
[Pt] Publication type:CASE REPORTS
[Nm] Name of substance:0 (Interleukin 1 Receptor Antagonist Protein); 9007-41-4 (C-Reactive Protein)
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[Js] Journal subset:IM
[Da] Date of entry for processing:171208
[St] Status:MEDLINE
[do] DOI:10.3349/ymj.2018.59.1.154

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[PMID]: 29501512
[Au] Autor:Georgin-Lavialle S; Rodrigues F; Hentgen V; Fayand A; Quartier P; Bader-Meunier B; Bachmeyer C; Savey L; Louvrier C; Sarrabay G; Melki I; Belot A; Koné-Paut I; Grateau G
[Ad] Address: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
[Ti] Title:Panorama des maladies auto-inflammatoires. [Clinical overview of auto-inflammatory diseases].
[So] Source:Rev Med Interne;, 2018 Feb 28.
[Is] ISSN:1768-3122
[Cp] Country of publication:France
[La] Language:fre
[Ab] Abstract: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. The best-known auto-inflammatory diseases are mediated by interleukines that consisted in the 4 following diseases familial Mediterranean fever, cryopyrinopathies, TNFRSF1A-related intermittent fever, and mevalonate kinase deficiency. Since 10 years, many other diseases have been discovered, especially thanks to the progress in genetics. In this review, we propose the actual panorama of the main known auto-inflammatory diseases. Some of them are recurrent fevers with crisis and remission; some others evaluate more chronically; some are associated with immunodeficiency. From a physiopathological point of view, we can separate diseases mediated by interleukine-1 and diseases mediated by interferon. Then some polygenic inflammatory diseases will be shortly described: Still disease, Schnitzler syndrome, aseptic abscesses syndrome. The diagnosis of auto-inflammatory disease is largely based on anamnesis, the presence of peripheral inflammation during attacks and genetic analysis, which are more and more performant.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180304
[Lr] Last revision date:180304
[St] Status:Publisher

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[PMID]: 29496698
[Au] Autor:Palladini G; Merlini G
[Ad] Address:Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo; University of Pavia.
[Ti] Title:The elusive pathogenesis of Schnitzler syndrome.
[So] Source:Blood;131(9):944-946, 2018 Mar 01.
[Is] ISSN:1528-0020
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180302
[Lr] Last revision date:180302
[St] Status:In-Data-Review
[do] DOI:10.1182/blood-2018-01-824862

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[PMID]: 29284595
[Au] Autor:Rowczenio DM; Pathak S; Arostegui JI; Mensa-Vilaro A; Omoyinmi E; Brogan P; Lipsker D; Scambler T; Owen R; Trojer H; Baginska A; Gillmore JD; Wechalekar AD; Lane T; Williams R; Youngstein T; Hawkins PN; Savic S; Lachmann HJ
[Ad] Address:National Amyloidosis Centre, University College London (UCL), London, United Kingdom.
[Ti] Title:Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome.
[So] Source:Blood;131(9):974-981, 2018 Mar 01.
[Is] ISSN:1528-0020
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:To date, the pathogenic mechanisms underlying Schnitzler syndrome remain obscure, in particular, the interplay between the monoclonal protein and increased interleukin-1ß (IL-1ß) 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. Patients underwent searches for germ line and somatic mutations using next-generation sequencing technology. Moreover, we designed a panel consisting of 32 autoinflammatory genes to explore genetic susceptibility factor(s) to Schnitzler syndrome. Genetic analysis revealed neither germ line nor somatic , , , or mutations, apart from 1 patient with a germ line p.V198M substitution. The proinflammatory cytokines and extracellular apoptosis-associated speck-like protein with caspase recruitment domain (ASC) measured in the serum of Schnitzler syndrome patients during active disease were significantly higher than healthy controls. Ninety-five percent of our cohort achieved a complete response to recombinant IL-1 receptor antagonist (anakinra). Our findings do not support a role for somatic mosaicism in disease pathogenesis; although elevated levels of ASC, IL-6, and IL-18 in patients' serum, and the response to anakinra, suggest that Schnitzler syndrome is associated with upregulated inflammasome activation. Despite its rarity, Schnitzler syndrome is an important diagnosis as treatment with IL-1 antagonists dramatically improves quality of life for patients.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180302
[Lr] Last revision date:180302
[St] Status:In-Data-Review
[do] DOI:10.1182/blood-2017-10-810366

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[PMID]: 29260755
[Au] Autor:Salugina SO; Gorodetsky VR; Fedorov ES; Lopatina NE; Evsikova MD
[Ad] Address:V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
[Ti] Title:Sindrom Shnittslera: obzor literatury, sobstvennoe nabliudenie, opyt lecheniia ingibitorom interleikina-1 kanakinumabom. [Schnitzler syndrome: A review of literature, the authors' observation, and experience with the interleukin-1 inhibitor canakinumab].
[So] Source:Ter Arkh;89(11):111-115, 2017.
[Is] ISSN:0040-3660
[Cp] Country of publication:Russia (Federation)
[La] Language:rus
[Ab] Abstract: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.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180228
[Lr] Last revision date:180228
[St] Status:In-Process
[do] DOI:10.17116/terarkh20178911111-115

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[PMID]: 29469761
[Au] Autor:Wilmas K; Aria A; Torres-Cabala CA; Lu H; Duvic M
[Ad] Address:University of Texas Health Science Center, McGovern Medical School, Houston, Texas Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas. Kelly.M.Wilmas@uth.tmc.edu.
[Ti] Title:Schnitzler syndrome in a patient with a family history of monoclonal gammopathy.
[So] Source:Dermatol Online J;24(1), 2018 Jan 15.
[Is] ISSN:1087-2108
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract: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. The patient's disease has been well controlled with anakinra therapy. Our case may contribute to a better understanding of the etiology of Schnitzler syndrome as his history could suggest a hereditarypredisposition for the disease. Further studies are necessary to determine whether a genetic component of Schnitzler syndrome exists, as first-degree relatives of patients with monoclonal gammopathies may be at risk for the development of the disease.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180222
[Lr] Last revision date:180222
[St] Status:In-Process

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[PMID]: 29162318
[Au] Autor:Villarreal RS; VandenBoom T; Gonzalez-Gonzalez FJ; Carter RG; Peters NT; Peters AT; Chiarella SE
[Ad] Address:Northwestern University Feinberg School of Medicine,Chicago, Illinois.
[Ti] Title:Schnitzler syndrome with IgG gammopathy and elevated IL-1ß and IL-17 in skin biopsy.
[So] Source:Ann Allergy Asthma Immunol;, 2017 Nov 18.
[Is] ISSN:1534-4436
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1711
[Cu] Class update date: 171122
[Lr] Last revision date:171122
[St] Status:Publisher

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[PMID]: 29077281
[Au] Autor:Arjona-Aguilera C; Jiménez-Gallo D; Ossorio-García L; Linares-Barrios M
[Ad] Address:Unidad de Gestión Clínica Dermatología y Venereología, Hospital Universitario Puerta del Mar, Servicio Andaluz de Salud, Cádiz, España.
[Ti] Title:Exantema urticarial refractario: síndrome de Schnitzler. [Refractory urticaria: Schnitzler syndrome].
[So] Source:Emergencias;29(2):137, 2017 Abr.
[Is] ISSN:2386-5857
[Cp] Country of publication:Spain
[La] Language:spa
[Pt] Publication type:LETTER
[Em] Entry month:1710
[Cu] Class update date: 171027
[Lr] Last revision date:171027
[St] Status:In-Data-Review

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[PMID]: 29027204
[Au] Autor:Sharma S; Joske D; Watson D; Caccetta T; Baltic S; Kumarasinghe SP
[Ad] Address:Department of Dermatology, Fiona Stanley Hospital, Perth, Western Australia, Australia.
[Ti] Title:Schnitzler syndrome: A rare cause of chronic recalcitrant urticaria successfully treated with Anakinra.
[So] Source:Australas J Dermatol;, 2017 Oct 12.
[Is] ISSN:1440-0960
[Cp] Country of publication:Australia
[La] Language:eng
[Pt] Publication type:LETTER
[Em] Entry month:1710
[Cu] Class update date: 171013
[Lr] Last revision date:171013
[St] Status:Publisher
[do] DOI:10.1111/ajd.12731

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[PMID]: 28971133
[Au] Autor:Bursztejn AC; Imperiale A; Lipsker D
[Ad] Address:Department of Dermatology, University Hospital of Strasbourg, Strasbourg, France.
[Ti] Title:Aortitis: A new feature of Schnitzler syndrome.
[So] Source:JAAD Case Rep;3(5):454-456, 2017 Sep.
[Is] ISSN:2352-5126
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 171006
[Lr] Last revision date:171006
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1016/j.jdcr.2017.06.016


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