508 results match your criteria: "Schnitzler Syndrome"
Investigator-initiated, multi-center, single-arm, open-label study of the effectiveness of canakinumab in Japanese patients with Schnitzler syndrome.
Allergol Int
December 2024
Department of Dermatology, Hyogo Medical University Graduate School of Medicine, Nishinomiya, Japan.
Background: Schnitzler syndrome is an adult-onset autoinflammatory disease characterized by an urticaria-like rash and monoclonal gammopathy with fever and fatigue. Although some treatments have shown efficacy in clinical trials, no approved treatment exists. We aimed to assess canakinumab, an anti-IL-1β monoclonal antibody, in Japanese patients.
View Article and Find Full Text PDFThe population based cognitive testing in subjects with SARS-CoV-2 (POPCOV2) study: longitudinal investigation of remote cognitive and fatigue screening in PCR-positive cases and negative controls.
Front Hum Neurosci
November 2024
Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich Heine University Düsseldorf, Düsseldorf, Germany.
Background: The majority of people infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) only show mild respiratory symptoms. However, some patients with SARS-CoV-2 display neurological symptoms. Data on the exact prevalence and course of cognitive symptoms are often limited to patient reported outcomes or studies recruited at specialized centers.
View Article and Find Full Text PDFAcquired autoinflammatory disorders: A dermatologist's perspective.
Clin Exp Dermatol
December 2024
Department of Dermatology, Venereology and Leprology; Postgraduate Institute of Medical Education and Research; Chandigarh, India.
Autoinflammatory disorders are characterized by dysregulated and disproportionately heightened response of innate immune system (IIS) to molecular patterns associated with damage and pathogens/microbes (DAMPs/PAMPs) with crucial role played by neutrophils and macrophages in disease pathogenesis. They closely resemble connective tissue diseases (CTDs). However, anti-nuclear antibodies (ANA), typically considered a marker of CTDs, are negative in autoinflammatory disorders.
View Article and Find Full Text PDFFACS-based detection of extracellular ASC specks from NLRP3 inflammasomes in inflammatory diseases.
Clin Exp Immunol
December 2024
Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, St James' University Hospital, Leeds, United Kingdom.
Article Synopsis
- The study focuses on the detection of ASC aggregates, which are important for inflammasome assembly, particularly the NLRP3 inflammasome, after pyroptotic cell death.
- Researchers developed a method using human monocytes, macrophages, and ASC reporter cells to identify ASC/NLRP3-positive events via flow cytometry.
- Results showed increased ASC/NLRP3 specks in the sera of patients with inflammatory conditions, indicating that FACS is a reliable detection method with potential diagnostic uses in autoinflammatory diseases.
Recurrent urticaria with fever, arthralgia, and biclonal gammopathy.
JAAD Case Rep
November 2024
Department of Dermatology, Apollo Hospital, Hyderabad, Telangana, India.
S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) - Part 2: Supportive therapy of EN in the acute and post-acute stages.
J Dtsch Dermatol Ges
November 2024
Department of Dermatology, Venereology and Allergology, Division of Evidence Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Article Synopsis
- Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are severe, drug-induced skin conditions that can be life-threatening and are now viewed as different levels of the same disease known as epidermal necrolysis (EN).
- A new guideline has been created based on scientific literature and expert consensus to help medical professionals in diagnosing and treating EN.
- This guideline targets various specialists like dermatologists and intensive care doctors, as well as informing patients, families, insurers, and policymakers about EN and includes recommendations for acute care and follow-up treatment.
Physical therapy in patients with Parkinson's disease treated with Deep Brain Stimulation: a Delphi panel study.
medRxiv
September 2024
"Aldo Ravelli" Center for Neurotechnology and Experimental Brain Therapeutics, Department of Health Sciences, University of Milan, Via Antonio di Rudinì 8, 20142 Milan, Italy.
Article Synopsis
- Deep brain stimulation of the subthalamic nucleus (STN-DBS) offers motor benefits for Parkinson's disease patients, but its effectiveness on postural and gait issues remains uncertain.
- Physical therapy (PT) has shown effectiveness in improving mobility and stability in non-surgically treated Parkinson's patients, but its role after STN-DBS is less explored.
- A study involving 21 experts highlighted that while PT could enhance motor function and quality of life post-STN-DBS, there’s a preference for conventional therapies over massage or manual techniques.
Multiplex Microscopy Assay for Assessment of Therapeutic and Serum Antibodies against Emerging Pathogens.
Viruses
September 2024
Department of Infectious Diseases, Virology, Medical Faculty Heidelberg, Heidelberg University, 69120 Heidelberg, Germany.
Article Synopsis
- Novel pathogens like SARS-CoV-2 demonstrate the urgent need for quick and flexible diagnostic tools to evaluate their effects on health and inform public health actions in future pandemics.
- The study presents an automated multiplex microscopy assay combined with machine learning for detecting antibodies through a unique barcoding strategy using HeLa cell lines expressing different viral antigens.
- This high-throughput approach allows for the analysis of patient sera and monoclonal antibodies, and can be quickly adapted to detect other variants or new pathogens, enhancing pandemic preparedness.
S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) - Part 1: Diagnosis, initial management, and immunomodulating systemic therapy.
J Dtsch Dermatol Ges
October 2024
Department of Dermatology, Venereology and Allergology, Division of Evidence Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Article Synopsis
- Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious, mostly drug-related conditions that affect the skin and mucous membranes, categorized under the umbrella term epidermal necrolysis (EN), which varies in severity.* -
- A new guideline for diagnosing and treating SJS/TEN was created based on extensive scientific research and consensus among experts, involving various medical specialties to provide a comprehensive approach to patient care.* -
- The guideline is designed for healthcare professionals across multiple fields, as well as patients, their families, insurers, and policymakers, with the first part specifically addressing diagnosis, initial treatment, and systemic immunotherapy.*
Definition and diagnosis of Parkinson's disease: guideline "Parkinson's disease" of the German Society of Neurology.
J Neurol
November 2024
Fachklinik Paracelsus-Elena-Klinik, Kassel, Germany.
Article Synopsis
- Accurate diagnosis of Parkinson's disease (PD) requires modern criteria that consider clinical, imaging, biomarker, and genetic information.
- The revised guidelines from the German Society for Neurology adopt a broader understanding of PD, recognizing both idiopathic and hereditary forms, and emphasize the 2015 Movement Disorders Society criteria.
- Enhanced diagnostic practices through long-term monitoring, the inclusion of non-motor symptoms, and the use of imaging and genetic testing support better patient-centered care for those with PD.
Adaptive Deep Brain Stimulation in Parkinson's Disease: A Delphi Consensus Study.
medRxiv
August 2024
"Aldo Ravelli" Center for Neurotechnology and Experimental Brain Therapeutics, Department of Health Sciences, University of Milan, Via Antonio di Rudinì 8, 20142 Milan, Italy.
Article Synopsis
- The evolution of Deep Brain Stimulation (DBS) is shifting from conventional methods to adaptive techniques (aDBS), which are expected to become standard practice in the next decade, similar to past advancements in cardiac pacing.
- Research involving 21 experts highlighted the challenges and potential of aDBS, showing consensus on its safety and effectiveness compared to conventional DBS, particularly for Parkinson's Disease patients with motor fluctuations and dyskinesias.
- Further studies are necessary to refine the algorithms used in aDBS, ensuring its widespread and effective use in clinical settings.
Mimicking urticaria: a Schnitzler syndrome case.
An Bras Dermatol
August 2024
Department of Immunology, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Inflammatory turmoil within: an exploration of autoinflammatory disease genetic underpinnings, clinical presentations, and therapeutic approaches.
Adv Rheumatol
August 2024
Universidade de Sao Paulo, Faculdade de Medicina (USP FM), Sao Paulo, Brazil.
Article Synopsis
- - Systemic autoinflammatory diseases (SAIDs) result from an overactive innate immune system, leading to widespread inflammation and are caused by various genetic defects that complicate diagnosis.
- - Despite advancements in genetic testing, around 40% of SAIDs patients still do not have a clear genetic diagnosis, and the disorders are grouped based on their unique pathophysiological features including inflammasomopathies and relopathies.
- - Targeted treatments are crucial, utilizing agents like JAK inhibitors and IL-1 blockers, to manage the distinct clinical presentations and inflammation associated with these complex diseases.
Schnitzler-Like Syndrome Presenting With IgG Kappa Monoclonal Gammopathy: A Case Report and Review of Diagnostic and Therapeutic Challenges.
Cureus
July 2024
Internal Medicine, Memorial Medical Center, Springfield, USA.
Article Synopsis
- - Schnitzler syndrome (SS) is a rare autoinflammatory disorder marked by chronic rash, recurrent fever, joint pain, and a specific type of immunoglobulin abnormality (IgM monoclonal gammopathy).
- - Schnitzler-like syndromes share similar symptoms but don’t meet the exact criteria for SS, leading to complexities in diagnosis.
- - The case report discusses a 40-year-old male with Schnitzler-like syndrome featuring IgG kappa monoclonal gammopathy, emphasizing the need for thorough diagnostic methods and specialized treatments.
Schnitzler Syndrome: Insights into Its Pathogenesis, Clinical Manifestations, and Current Management.
Biomolecules
May 2024
Dermatologic Clinic, University Hospital of Strasbourg, 67091 Strasbourg, France.
Article Synopsis
- * The disease shows similarities with other autoinflammatory disorders and highlights the significant role of interleukin-1 inhibitors in its treatment, pointing to this cytokine's importance in its development.
- * Despite some insights, the exact causes of Schnitzler syndrome and its connection between autoinflammatory symptoms and monoclonal gammopathy remain largely unknown, warranting further investigation into its diagnosis and treatment options.
Article Synopsis
- - The study focused on the experiences of 17 patients diagnosed with Schnitzler's syndrome (SchS) over a 10-year period in a multicenter Russian cohort, all meeting the Strasbourg diagnostic criteria.
- - Patients experienced various symptoms including fatigue, skin rashes, bone pain, and fever, with significant variations in disease duration before diagnosis and other clinical manifestations.
- - All patients exhibited monoclonal gammopathy, but specific types varied, with no presence of Ben-Jones protein, indicating a specific set of laboratory findings associated with the syndrome.
Magnetic Resonance Imaging Measures to Track Atrophy Progression in Progressive Supranuclear Palsy in Clinical Trials.
Mov Disord
August 2024
Department of Neurology, University Hospital, LMU Munich, Munich, Germany.
Article Synopsis
- - The study investigates various MRI measures as biomarkers for tracking brain atrophy progression in patients with progressive supranuclear palsy (PSP), focusing on comparing different staging systems and assessing their effectiveness for clinical trials.
- - 508 participants were analyzed, including PSP patients and healthy controls, with specific attention given to the brainstem and midbrain volumes, which showed the most significant changes over one year.
- - Results suggest that automated atlas-based volumetry is more sensitive and requires smaller sample sizes than other methods for detecting atrophy progression in PSP, potentially aiding in the evaluation of treatment efficacy in clinical trials.
[Translated article] Schnitzler Syndrome, and the MEFV Gene Variant.
Actas Dermosifiliogr
July 2024
Servicio de Dermatología, Hospital Universitario Santa María, Lérida, Spain.
[The most frequent febrile syndromes and autoinflammatory diseases in adulthood].
Z Rheumatol
June 2024
Nephrologie, Rheumatologie, Endokrinologie, III. Medizinische Klinik und Poliklinik, Martinistr. 52, 20251, Hamburg, Deutschland.
Article Synopsis
- - Autoinflammatory diseases are marked by recurring inflammation in various body systems, often leading to symptoms like fever, joint pain, and skin issues, alongside signs of inflammation in blood tests.
- - These diseases can be classified into rare monogenic types, such as familial Mediterranean fever and cryopyrin-associated periodic syndrome, and more common multifactorial or polygenic forms, like adult-onset Still's disease.
- - Monogenic diseases have specific genetic causes, while multifactorial diseases involve a combination of genetic and environmental factors, highlighting the complexity of autoinflammatory disorders.
The co-occurrence of SAT, hypophysitis, and Schnitzler syndrome after COVID-19 vaccination: the first described case.
Hormones (Athens)
December 2024
Clinic of Endocrinology, Diabetology and Internal Medicine, School of Medicine, Collegium Medicum, University of Warmia and Mazury in Olsztyn, 10-957, Olsztyn, Poland.
Article Synopsis
A case of Schnitzler syndrome with unusual immunoglobulin A gammopathy exacerbated by COVID-19 infection.
J Dermatol
November 2024
Department of Dermatology, Shiga University of Medical Science, Otsu, Shiga, Japan.
Article Synopsis
A case of Schnitzler-like syndrome with basement membrane IgM deposition but without monoclonal gammopathy.
JAAD Case Rep
May 2024
Department of Dermatology, Oregon Health & Science University, Portland, Oregon.
Neutrophilic Urticarial Dermatosis.
Dermatol Clin
April 2024
Department of Dermatology, Mayo Clinic, 4500 San Pablo Road S, Jacksonville, FL 32224, USA; Department of Laboratory Medicine & Pathology, Mayo Clinic, 4500 San Pablo Road S, Jacksonville, FL 32224, USA. Electronic address:
Article Synopsis
- - Neutrophilic urticarial dermatosis (NUD) is a rare skin condition characterized by specific types of neutrophil infiltrates, but its exact causes and mechanisms are not well understood.
- - Clinically, NUD appears as recurrent, non-itchy pink to reddish patches or plaques that typically resolve within a day, leaving no lasting marks.
- - The condition is often linked to various systemic diseases, including Schnitzler syndrome, lupus, adult-onset Still's disease, and certain periodic syndromes related to cryopyrin.
Eponyms that honor Jewish dermatologists: A celebration and a remembrance, Part three: Jewish physicians who practiced during the Holocaust and in its aftermath.
Clin Dermatol
June 2024
Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Article Synopsis
Clinical Presentation of Schnitzler's Syndrome: A Rare Autoimmune Disease.
J Investig Med High Impact Case Rep
December 2023
University of Florida Health, Gainesville, USA.
Article Synopsis
- - Schnitzler's Syndrome (SS) is a rare, late-onset autoinflammatory disorder characterized by chronic urticaria and a specific type of antibody (monoclonal IgM-kappa), along with symptoms like joint pain and recurrent fevers.
- - Diagnosis requires meeting two major criteria (chronic urticaria and monoclonal IgM) plus at least two minor criteria, with early detection being crucial due to a 15-20% risk of related cancers.
- - A case study of a 39-year-old female highlights the challenges in diagnosing SS; after ineffective treatments, she was successfully managed with an IL-1 inhibitor, illustrating the need for awareness of this rare condition in clinical practice.