401 results match your criteria Schnitzler Syndrome

Hepatic encephalopathy.

Nat Rev Dis Primers 2022 Jun 23;8(1):43. Epub 2022 Jun 23.

Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.

Hepatic encephalopathy (HE) is a prognostically relevant neuropsychiatric syndrome that occurs in the course of acute or chronic liver disease. Besides ascites and variceal bleeding, it is the most serious complication of decompensated liver cirrhosis. Ammonia and inflammation are major triggers for the appearance of HE, which in patients with liver cirrhosis involves pathophysiologically low-grade cerebral oedema with oxidative/nitrosative stress, inflammation and disturbances of oscillatory networks in the brain. Read More

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Absence of NLRP3 somatic mutations and VEXAS-related UBA1 mutations in a large cohort of patients with Schnitzler syndrome.

Allergy 2022 Jun 17. Epub 2022 Jun 17.

Sorbonne Université, Inserm, Childhood Genetic Disorders and Département de Génétique Médicale, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital Armand-Trousseau, Paris, France.

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Schnitzler Syndrome after COVID-19 Vaccination.

Intern Med 2022 May 31. Epub 2022 May 31.

Department of Hematology, Hyogo Prefectural Nishinomiya Hospital, Japan.

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Altered performance monitoring in Tourette Syndrome: an MEG investigation.

Sci Rep 2022 May 18;12(1):8300. Epub 2022 May 18.

Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine-University, 40225, Duesseldorf, Germany.

The error-related negativity (ERN) is an event-related potential component indexing processes of performance monitoring during simple stimulus-response tasks: the ERN is typically enhanced for error processing and conflicting response representations. Investigations in healthy participants and different patient groups have linked the ERN to the dopamine system and to prefrontal information processing. As in patients with Tourette Syndrome (TS) both dopamine release and prefrontal information processing are impaired, we hypothesized that performance monitoring would be altered, which was investigated with magnetencephalography (MEG). Read More

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Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome.

Front Immunol 2022 20;13:894464. Epub 2022 Apr 20.

Department of Hematology, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing, China.

Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact the underlying monoclonal gammopathy. Herein, we described a female patient who presented with urticarial rash, recurrent fevers, and fatigue for 7 years. Read More

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Comparative analysis of machine learning algorithms for multi-syndrome classification of neurodegenerative syndromes.

Alzheimers Res Ther 2022 May 3;14(1):62. Epub 2022 May 3.

Clinic for Cognitive Neurology, University Clinic Leipzig, Leipzig, Germany.

Importance: The entry of artificial intelligence into medicine is pending. Several methods have been used for the predictions of structured neuroimaging data, yet nobody compared them in this context.

Objective: Multi-class prediction is key for building computational aid systems for differential diagnosis. Read More

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Cutaneous manifestations of monoclonal gammopathy.

Blood Cancer J 2022 04 11;12(4):58. Epub 2022 Apr 11.

Division of Hematology, Mayo Clinic Rochester, Mayo Clinic, MN, USA.

Monoclonal gammopathy associated with dermatological manifestations are a well-recognized complication. These skin disorders can be associated with infiltration and proliferation of a malignant plasma cells or by a deposition of the monoclonal immunoglobulin in a nonmalignant monoclonal gammopathy. These disorders include POEMS syndrome, light chain amyloidosis, Schnitzler syndrome, scleromyxedema and TEMPI syndrome. Read More

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A patient with urticarial lesions, recurrent fever, and IgM-type monoclonal gammopathy.

Acta Dermatovenerol Alp Pannonica Adriat 2022 03;31(Suppl):S27-S29

Department of Dermatovenereology, Ljubljana University Medical Centre, Ljubljana, Slovenia.

Schnitzler syndrome is a rare acquired autoinflammatory syndrome. It presents with an urticarial rash and a monoclonal gammopathy, usually of the IgM kappa type. In addition, patients can present with bone and/or joint pain, recurrent fever, asthenia, weight loss, myalgia, headache, lymphadenopathy, hepatomegaly, or splenomegaly. Read More

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The Expanding Spectrum of Autoinflammatory Diseases.

Intern Med 2022 Mar 19. Epub 2022 Mar 19.

Department of Rheumatology, Fukushima Medical University School of Medicine, Japan.

Autoinflammatory diseases are systemic disorders caused by genetic or acquired abnormalities in certain signaling pathways of the innate immune system. Dysregulated activation of the inflammasome, i.e. Read More

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A man in his fifties with recurrent urticaria, fever and joint pain.

Tidsskr Nor Laegeforen 2022 03 28;142(4). Epub 2022 Feb 28.

Background: Schnitzler's syndrome is a rare, acquired and probably underdiagnosed disorder. It is a type of autoinflammatory condition with late onset.

Case Presentation: A man in his fifties had had recurrent urticaria, fever and chronic joint pain during the previous year. Read More

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Neutralizing antibody activity against the B.1.617.2 (delta) variant 8 months after two-dose vaccination with BNT162b2 in health care workers.

Clin Microbiol Infect 2022 Jul 3;28(7):1024.e7-1024.e12. Epub 2022 Feb 3.

Department of Nephrology, University of Heidelberg, Heidelberg, Germany; Department of Molecular Medicine Partnership Unit Heidelberg, European Molecular Biology Laboratory, Heidelberg, Germany.

Objectives: Humoral immunity wanes over time after two-dose BNT162b2 vaccination. Emerging variants of concern, such as the B.1. Read More

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Brain volume patterns in corticobasal syndrome versus idiopathic Parkinson's disease.

J Neuroimaging 2022 Jan 31. Epub 2022 Jan 31.

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

Background And Purpose: Patients with a corticobasal syndrome (CBS) present a rare form of atypical parkinsonism characterized by asymmetric clinical symptoms and progressive motor and nonmotor impairment, such as apraxia, alien limb phenomenon, aphasia, myoclonus, dystonia, and cognitive impairment. At early stages, clinical differentiation between CBS and idiopathic Parkinson's disease (IPD) can be challenging.

Methods: Using high-resolution T1-weighted images and voxel-based morphometry (VBM), we sought to identify disease-specific patterns of brain atrophy in a small sample of CBS and IPD patients at early stages of disease. Read More

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January 2022

Schnitzler syndrome and Schnitzler-like syndromes.

Cong-Qiu Chu

Chin Med J (Engl) 2022 Jan 27. Epub 2022 Jan 27.

Division of Arthritis and Rheumatic Diseases, Oregon Health & Science University and Rheumatology Section, VA Portland Health Care System, Portland, OR 97239, USA.

Abstract: Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. Read More

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January 2022

Neutralization of SARS-CoV-2 Variants of Concern in Kidney Transplant Recipients after Standard COVID-19 Vaccination.

Clin J Am Soc Nephrol 2022 01 22;17(1):98-106. Epub 2021 Dec 22.

Department of Nephrology, University of Heidelberg, Heidelberg, Germany

Background And Objectives: Antibody response after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination is impaired in kidney transplant recipients. Emerging variants, such as B.1. Read More

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January 2022

Genetics of somatic auto-inflammatory disorders.

Semin Hematol 2021 10 9;58(4):212-217. Epub 2021 Oct 9.

Leeds Institute of Rheumatological and Musculoskeletal Medicine, University of Leeds, UK. Electronic address:

Systemic autoinflammatory disorders (SAIDs) encompass a heterogeneous group of monogenic disorders characterized by recurrent episodes of systemic and organ-specific inflammation. Genetic studies have facilitated the identification of Mendelian forms of SAIDs but many patients still remain without a diagnosis. Recent studies have uncovered that somatic (acquired) mutations can cause later-onset SAIDs. Read More

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October 2021

[Autoinflammation-differences between children and adults].

Z Rheumatol 2022 Feb 11;81(1):45-54. Epub 2021 Nov 11.

Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland.

Autoinflammatory diseases present as multisystemic inflammation and often manifest in early childhood. In contrast, in a few diseases, e.g. Read More

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February 2022

AA amyloidosis complicating monoclonal gammopathies, an unusual feature validating the concept of "monoclonal gammopathy of inflammatory significance"?

Int J Clin Pract 2021 Nov 21;75(11):e14817. Epub 2021 Sep 21.

Department of Internal Medicine, Centre de référence des maladies auto-inflammatoires et des amyloses d'origine inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, Paris, France.

Introduction: AL amyloidosis is caused by the proliferation of an immunoglobulin-secreting B cell clone. AA amyloidosis is a rare complication of chronic inflammation. However, some patients present with diseases combining monoclonal immunoglobulin production and chronic inflammation. Read More

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November 2021

Refractory serum immunoglobulin M elevation during anti-interleukin (IL)-1- or IL-6-targeted treatment in four patients with Schnitzler syndrome.

J Dermatol 2021 Nov 26;48(11):1789-1792. Epub 2021 Aug 26.

Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Schnitzler syndrome is characterized by chronic urticarial rash, neutrophilic dermal infiltrate, recurrent fever, bone pain, elevated C-reactive protein, and neutrophilic leukocytosis. The pathophysiology of Schnitzler syndrome is unknown, but it is considered to be an acquired form of an autoinflammatory disease because of the resemblance to clinical phenotypes of cryopyrin-associated periodic syndrome, in which a gain-of-function mutation in NLRP3 causes overexpression of interleukin (IL)-1β. Schnitzler syndrome is generally accompanied by a monoclonal immunoglobulin (Ig)M gammopathy with a long-term risk of lymphoproliferation that is possibly associated with an MYD88 mutation. Read More

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November 2021

Evaluation of accuracy, exclusivity, limit-of-detection and ease-of-use of LumiraDx™: An antigen-detecting point-of-care device for SARS-CoV-2.

Infection 2022 Apr 12;50(2):395-406. Epub 2021 Aug 12.

Division of Clinical Tropical Medicine, Heidelberg University Hospital, Heidelberg, Germany.

Purpose: Rapid antigen-detecting tests (Ag-RDTs) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can transform pandemic control. Thus far, sensitivity (≤ 85%) of lateral-flow assays has limited scale-up. Conceivably, microfluidic immunofluorescence Ag-RDTs could increase sensitivity for SARS-CoV-2 detection. Read More

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Killing Two Birds with One Stone: the Therapeutic Role of Ibrutinib in Schnitzler Syndrome.

Chen Wang

J Clin Immunol 2021 10 27;41(7):1706-1707. Epub 2021 Jul 27.

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

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October 2021

Anakinra-induced psoriasis in a patient with Schnitzler's syndrome.

Clin Exp Rheumatol 2022 01 7;40(1):191-192. Epub 2021 Jul 7.

Department of Dermatology, Hospital Universitari de Bellvitge, University of Barcelona, Spain.

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January 2022

High rate of HSV-1 reactivation in invasively ventilated COVID-19 patients: Immunological findings.

PLoS One 2021 1;16(7):e0254129. Epub 2021 Jul 1.

Department of Gastroenterology, University Hospital Heidelberg, Heidelberg, Germany.

SARS-CoV-2 infection can lead to severe acute respiratory distress syndrome with the need of invasive ventilation. Pulmonary herpes simplex-1 (HSV-1) reactivation in invasively ventilated patients is a known phenomenon. To date very little is known about the frequency and the predisposing factors of HSV-1 reactivation in COVID-19. Read More

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Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome.

Vnitr Lek 2021 ;67(E-3):15-23

Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome Schnitzler´s syndrome is a very rare, adult-onset, apparently acquired autoinflammatory disease. Chronic urticarial rash and symptoms of systemic inflammation including fever, arthralgia and bone pain with the presence of monoclonal immunoglobulin M (IgM), rarely IgG, are among hallmarks of the disease. We performed a retrospective study of 6 patients (5 men, 1 woman) diagnosed with Schnitzler´s syndrome fulfilling the Strasbourg criteria who had been treated at our centre in the University Hospital Brno from 2007 to 2021. Read More

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Early Humoral Responses of Hemodialysis Patients after COVID-19 Vaccination with BNT162b2.

Clin J Am Soc Nephrol 2021 07 24;16(7):1073-1082. Epub 2021 May 24.

Department of Nephrology, University of Heidelberg, Heidelberg, Germany.

Background And Objectives: Patients receiving hemodialysis are at high risk for both severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and severe coronavirus disease 2019. A lifesaving vaccine is available, but sensitivity to vaccines is generally lower in patients on dialysis. Little is yet known about antibody responses after coronavirus disease 2019 (COVID-19) vaccination in this vulnerable group. Read More

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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 08 19;41(6):1380-1383. 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