373 results match your criteria Schnitzler Syndrome


Interpretation of myocardial injury subtypes in COVID-19 disease per fourth version of Universal Definition of Myocardial Infarction.

Biomarkers 2021 Jul 24;26(5):401-409. Epub 2021 May 24.

Department of Internal Medicine III, Cardiology, University of Heidelberg, Heidelberg, Germany.

Background: Application of the 4th version of Universal Definition of Myocardial Infarction (UDMI) to characterize rates and prognostic relevance of myocardial injury in COVID-19 disease.

Methods: This retrospective, single-centre observational study enrolled 104 patients hospitalized with SARS-CoV-2 infection. Kaplan-Meier analysis and multivariate Cox regression were used to identify influence of acute or chronic myocardial injury on a composite primary (mortality, incident acute respiratory distress syndrome, incident mechanical ventilation) and secondary endpoint (mortality, incident acute myocardial injury during hospitalization, incident venous thrombosis, pulmonary embolism or stroke). Read More

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Infection Complications after Lymphodepletion and Dosing of Chimeric Antigen Receptor T (CAR-T) Cell Therapy in Patients with Relapsed/Refractory Acute Lymphoblastic Leukemia or B Cell Non-Hodgkin Lymphoma.

Cancers (Basel) 2021 Apr 2;13(7). Epub 2021 Apr 2.

Department of Internal Medicine V-Hematology, Oncology & Rheumatology, University Hospital Heidelberg, 69120 Heidelberg, Germany.

Chimeric antigen receptor T (CAR-T) cell therapy has proven to be very effective in patients with relapsed/refractory acute lymphoblastic leukemia (ALL) and non-Hodgkin lymphoma (NHL). However, infections-related either due to lymphodepletion or the CAR-T cell therapy itself-can result in severe and potentially life-threatening complications, while side effects such as cytokine release syndrome (CRS) might complicate differential diagnosis. Sixty-seven dosings of CAR-T cells in sixty adult patients with NHL (85%) and ALL (15%) receiving CAR-T cell therapy were assessed for infectious complications. Read More

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Case Report: Interferon-γ Restores Monocytic Human Leukocyte Antigen Receptor (mHLA-DR) in Severe COVID-19 With Acquired Immunosuppression Syndrome.

Front Immunol 2021 7;12:645124. Epub 2021 Apr 7.

Department of Anesthesiology and Intensive Care Medicine, University Medical, Center Goettingen, Goettingen, Germany.

Background: The major histocompatibility complex (MHC) class II characterized by monocytes CD14+ expression of human leukocyte antigen receptors (HLA-DR), is essential for the synapse between innate and adaptive immune response in infectious disease. Its reduced expression is associated with a high risk of secondary infections in septic patients and can be safely corrected by Interferon-y (IFNy) injection. Coronavirus disease (COVID-19) induces an alteration of Interferon (IFN) genes expression potentially responsible for the observed low HLA-DR expression in circulating monocytes (mHLA-DR). Read More

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Dramatic Efficacy of Ibrutinib in a Schnitzler Syndrome Case with Indolent Lymphoma.

J Clin Immunol 2021 Apr 19. Epub 2021 Apr 19.

Hematology Department, University Hospital of Grenoble, La Tronche, France.

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Dysregulated Host Response in Severe Acute Respiratory Syndrome Coronavirus 2-Induced Critical Illness.

Open Forum Infect Dis 2021 Mar 18;8(3):ofab019. Epub 2021 Jan 18.

Department of Gastroenterology and Infectious Diseases, University Hospital Heidelberg, Heidelberg, Germany.

Background: Impaired immune response has been reported to be the cause of the development of coronavirus disease 2019 (COVID-19)-related respiratory failure. Further studies are needed to understand the immunopathogenesis and to enable an improved stratification of patients who are at risk for critical illness.

Methods: Thirty-two severely ill patients hospitalized with COVID-19 were recruited in our center at the University Hospital Heidelberg. Read More

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Schnitzler Syndrome: A Recherche Entity.

Cureus 2021 Feb 14;13(2):e13338. Epub 2021 Feb 14.

Internal Medicine, All India Institute of Medical Sciences, Bhubaneswar, IND.

Schnitzler syndrome (SS) is a rare disease of unknown etiology. Literature suggests that only around 300 well-diagnosed cases have only been reported worldwide and rarely from India. This syndrome has a slight male predominance with a mean age of onset of around 50 years. Read More

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February 2021

A case of Schnitzler's syndrome without monoclonal gammopathy successfully treated with canakinumab.

BMC Musculoskelet Disord 2021 Mar 8;22(1):257. Epub 2021 Mar 8.

Department of Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, Fukushima, 960-1295, Japan.

Background: Schnitzler's syndrome (SchS) is a rare autoinflammatory syndrome with diagnostic challenge and be characterized by chronic urticaria, a monoclonal gammopath, periodic fever and bone pain. In addition to the monoclonal gammopathy, bone abnormalities are often found at the site of bone pain in patients with SchS. The remarkable efficacy of interleukin-1 (IL-1) inhibition was also demonstrated in this syndrome. Read More

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Validation of two severity scores as predictors for outcome in Coronavirus Disease 2019 (COVID-19).

PLoS One 2021 19;16(2):e0247488. Epub 2021 Feb 19.

Department of Internal Medicine III, Cardiology, University of Heidelberg, Heidelberg, Germany.

Background: An established objective and standardized reporting of clinical severity and disease progression in COVID-19 is still not established. We validated and compared the usefulness of two classification systems reported earlier-a severity grading proposed by Siddiqi and a system from the National Australian COVID-19 guideline. Both had not been validated externally and were now tested for their ability to predict complications. Read More

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Antisynthetase Syndrome-Associated Interstitial Lung Disease: Monitoring of Immunosuppressive Treatment Effects by Chest Computed Tomography.

Front Med (Lausanne) 2020 25;7:609595. Epub 2021 Jan 25.

Department of Nephrology and Rheumatology, University Medical Center Goettingen, Goettingen, Germany.

Antisynthetase syndrome (ASyS) is a rare autoimmune disease characterized by inflammatory myopathy, arthritis, fever, and interstitial lung disease (ILD). Pulmonary involvement in ASyS significantly increases morbidity and mortality and, therefore, requires prompt and effective immunosuppressive treatment. Owing to the rarity of ASyS, limited data exists on progression and prognosis of ILD under immunosuppression. Read More

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January 2021

Tocilizumab treatment in patients with Schnitzler syndrome: An open-label study.

J Allergy Clin Immunol Pract 2021 Jun 2;9(6):2486-2489.e4. Epub 2021 Feb 2.

Dermatological Allergology, Allergie-Centrum-Charité, 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; Autoinflammation Reference Center Charité (ARC2), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany. Electronic address:

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Can therapeutic response to IL-1 blockade confirm the diagnosis of Schnitzler syndrome?

Authors:
Chen Wang

Clin Rheumatol 2021 02 11;40(2):805. Epub 2021 Jan 11.

Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL, 33606, USA.

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February 2021

Evidence of B Cell Clonality and Investigation Into Properties of the IgM in Patients With Schnitzler Syndrome.

Front Immunol 2020 3;11:569006. Epub 2020 Dec 3.

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

The Schnitzler Syndrome (SchS) is an acquired, autoinflammatory condition successfully treated with IL-1 inhibition. The two main defining features of this late-onset condition are neutrophilic urticarial dermatoses (NUD) and the presence of an IgM monoclonal component. While the former aspect has been extensively studied in this disease setting, the enigmatic paraproteinaemia and its potential consequential effects within SchS, has not previously been thoroughly addressed. Read More

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Cytokine Signature in Schnitzler Syndrome: Proinflammatory Cytokine Production Associated to Th Suppression.

Front Immunol 2020 26;11:588322. Epub 2020 Nov 26.

Laboratoire Inflammation, Tissus Epithéliaux et Cytokines (LITEC), EA4331, Université de Poitiers, Poitiers, France.

Background: Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by urticarial exanthema, bone and joint alterations, fever and monoclonal IgM gammopathy. Overactivation of the interleukin(IL)-1 system is reported, even though the exact pathophysiological pathways remain unknown.

Objective: To determine v cytokine profiles of Peripheral Blood Mononuclear Cells (PBMCs) from SchS patients prior to treatment and after initiation of anti-IL-1 therapy (anakinra). Read More

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November 2020

Schnitzler Syndrome: the paradigm of an acquired adult-onset auto-inflammatory disease.

G Ital Dermatol Venereol 2020 Oct;155(5):567-573

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

Schnitzler Syndrome is a rare acquired auto-inflammatory syndrome defined by an urticarial eruption and a monoclonal gammopathy, mainly of the IgM kappa isotype. It shares many clinical and biological features with other autoinflammatory disorders such as NLRP3-auto-inflammatory disorders (NLRP3-AID, formerly cryopyrin associated periodic syndromes or CAPS) or adult-onset Still disease (AOSD). Hence, recurrent fever, urticarial rash with a neutrophilic infiltrate on skin biopsy (i. Read More

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October 2020

Prolonged Neuropsychological Deficits, Central Nervous System Involvement, and Brain Stem Affection After COVID-19-A Case Series.

Front Neurol 2020 5;11:574004. Epub 2020 Nov 5.

Department of Neurology, Medical Faculty, Heinrich-Heine University, Düsseldorf, Germany.

The affection of both the peripheral (PNS) and central nervous system (CNS) by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been assumed to play a direct role in the respiratory failure of patients with Corona virus disease 2019 (COVID-19) through affection of medullary cardiorespiratory centers resulting in neurological complications and sequelae. We used a multimodal electrophysiological approach combined with neuropsychological investigations to study functional alteration of both the PNS and CNS in four patients with severe COVID-19. We found electrophysiological evidence for affection of both the PNS and CNS, and particularly affection of brain stem function. Read More

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November 2020

Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients.

Arthritis Res Ther 2020 11 18;22(1):272. Epub 2020 Nov 18.

Department of Internal Medicine Interne, CHU Nantes, Nantes, France.

Objective: To report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS).

Methods: A retrospective cohort study of patients with SchS followed between 2000 and 2020. Inclusion criteria included a diagnosis of SchS (Strasbourg criteria). Read More

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November 2020

Efficacy of interleukin-1 blockade in Schnitzler's syndrome without detectable monoclonal gammopathy: a case-based review.

Clin Rheumatol 2021 Jul 11;40(7):2973-2977. Epub 2020 Nov 11.

Rheumatology Section, Department of Medicine, University of Verona, Policlinico G.B. Rossi, Piazzale L.A. Scuro 10, 37134, Verona, Italy.

Schnitzler's syndrome (SchS) is a rare autoinflammatory disorder characterized by urticarial rash and monoclonal gammopathy which is currently regarded as IL-1 mediated disease. We present the case of a 21-year-old woman presenting with urticarial rash, arthralgias, and elevated inflammatory markers. She has been suffering these symptoms for 2 years and was treated with antihistamines, omalizumab, steroids, and non-steroidal anti-inflammatory drugs (NSAIDs) without success. Read More

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Refractory Urticaria with Raised Antistreptolysin O (ASO) Titer: An Intriguing Case of Adult-Onset Still's Disease.

Am J Case Rep 2020 Nov 5;21:e927936. Epub 2020 Nov 5.

Department of Pathology, King Saud University, Riyadh, Saudi Arabia.

BACKGROUND Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disease with a myriad of clinical presentations. The diagnosis is often challenging because there is no specific confirmatory test. Uncommon presentations can delay the proper diagnosis and management. Read More

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November 2020

Case Report: Extensive Phosphorylation of Interleukin-1 Receptor-Associated Kinase 4 in a Patient With Schnitzler Syndrome.

Front Immunol 2020 30;11:576200. Epub 2020 Sep 30.

Division of Rheumatology and Immunology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.

Schnitzler syndrome (SchS) is a rare autoinflammatory disease, characterized by urticarial rash, recurrent fever, osteo-articular pain/arthritis with bone condensation, and monoclonal gammopathy. Diagnosis may be difficult due to overlapping signs with other diseases. Here, we describe the case of a 62-year-old man with SchS, who was initially misdiagnosed with multicentric Castleman disease (MCD). Read More

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Schnitzler Syndrome in a 27-Year-Old Man: Diagnostic and Therapeutic Dilemma in Adult Auto-Inflammatory Syndromes A Case Report and Literature Review.

Int J Gen Med 2020 25;13:713-719. Epub 2020 Sep 25.

Department of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine in Warsaw, Warsaw, Poland.

A 32-year-old-man, with a history of chronic urticaria from the age of 27, diagnosed with an adult-onset Still's disease and received a low dose of glucocorticoids, methotrexate and tocilizumab. Despite the long-term combined treatments, he suffered from chronic urticaria, low-grade fever and bone pain. He was found to have high inflammatory markers, hypogammaglobulinemia, monoclonal IgM - kappa light chain in serum and increased radiotracer uptake in the whole bone scintigraphy. Read More

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September 2020

Schnitzler syndrome with basophil infiltration.

J Dtsch Dermatol Ges 2020 09 13;18(9):1034-1036. Epub 2020 Sep 13.

Department of Dermatology, National Defense Medical College, Tokorozawa, Japan.

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September 2020

Paraprotein associated syndrome treated successfully with chemotherapy.

BMJ Case Rep 2020 Sep 9;13(9). Epub 2020 Sep 9.

Department of Applied Medicine, University of Aberdeen, Aberdeen, UK

We report a 50-year-old Caucasian woman who presented with recurrent erythema nodosum, leg swelling, malaise, weight loss and abdominal pain associated with an IgM lambda paraprotein. She was treated with six courses of an anti-CD20 monoclonal antibody and bendamustine chemotherapy over 6 months with a good clinical response. Read More

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September 2020

SARS-CoV-2 RNA Extraction Using Magnetic Beads for Rapid Large-Scale Testing by RT-qPCR and RT-LAMP.

Viruses 2020 08 7;12(8). Epub 2020 Aug 7.

Center of Infectious Diseases, Virology, Heidelberg University Hospital, 69120 Heidelberg, Germany.

Rapid large-scale testing is essential for controlling the ongoing pandemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The standard diagnostic pipeline for testing SARS-CoV-2 presence in patients with an ongoing infection is predominantly based on pharyngeal swabs, from which the viral RNA is extracted using commercial kits, followed by reverse transcription and quantitative PCR detection. As a result of the large demand for testing, commercial RNA extraction kits may be limited and, alternatively, non-commercial protocols are needed. Read More

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[Schnitzler syndrome].

Rev Med Liege 2020 Jul;75(7-8):553-557

Service de Médecine interne générale et Maladies infectieuses, CHU Liège, Belgique.

This is the case report of a 57-year-old women with a 10-year long history of urticarial-like exanthema and monoclonal immunoglobulin M Kappa gammopathy, associated to arthralgia with pain of the lower limbs. A cutaneous biopsy and an inflammatory syndrome on laboratory testing helped to diagnose an urticarial vasculitis. A treatment with colchicine was set up but the response to therapy was not satisfactory. Read More

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A colorimetric RT-LAMP assay and LAMP-sequencing for detecting SARS-CoV-2 RNA in clinical samples.

Sci Transl Med 2020 08 27;12(556). Epub 2020 Jul 27.

Center for Molecular Biology of Heidelberg University (ZMBH), Heidelberg, Germany.

The coronavirus disease 2019 (COVID-19) pandemic caused by the SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) coronavirus is a major public health challenge. Rapid tests for detecting existing SARS-CoV-2 infections and assessing virus spread are critical. Approaches to detect viral RNA based on reverse transcription loop-mediated isothermal amplification (RT-LAMP) have potential as simple, scalable, and broadly applicable testing methods. Read More

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Incomplete Schnitzler Syndrome.

Acta Dermatovenerol Croat 2020 Jul;28(1):38-40

Mattias A.S. Henning, MD, Department of Dermatology, Zealand University Hospital, Sygehusvej 5, 4000 Roskilde, Denmark;

Schnitzler syndrome (SS) is a rare autoinflammatory disease that presents with chronic urticaria and monoclonal immunoglobulin (Ig) M or G, accompanied by fever, abnormal bone remodeling, skin biopsy with a neutrophilic dermal infiltrate, leukocytosis, or elevated C-reactive protein. It is usually refractory to antihistamines and immunosuppression. We present a case report of clinical SS without monoclonal Ig with robust response to interleukin-1 inhibitor anakinra. Read More

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

Neurobiol Aging 2020 10 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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October 2020

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

Clin Rheumatol 2020 Dec 9;39(12):3847-3852. 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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December 2020

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

Semin Arthritis Rheum 2020 08 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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