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Background and Clinical Features of a Unique and Mysterious Autoinflammatory Disease, Schnitzler Syndrome.
Int J Mol Sci
January 2025
Immunology Division, Department of Internal Medicine and Hematology, Semmelweis University, 1088 Budapest, Hungary.
Schnitzler syndrome is a unique autoinflammatory disease, of which 747 cases have been described worldwide to date. The main features of the syndrome are a triad of recurrent urticaria, monoclonal IgM gammopathy, systemic inflammation associated with recurrent fever, joint and bone pain, and atypical bone remodeling (osteosclerosis). The abnormal activation of the NLRP3 inflammasome produces IL-1, which drives the disease pathology, but it also involves IL-6 and IL-18.
View Article and Find Full Text PDFSchnitzler 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.
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.
Subacute thyroiditis (also known as granulomatous thyroiditis, giant cell thyroiditis, de Quervain's disease, or SAT) is an inflammatory disease of the thyroid gland, usually spontaneously remitting, that lasts for weeks to months. However, recurrent forms sometimes occur which may have a genetic basis. In our paper, we have focused on the pathogenetics, symptoms, and treatment of SAT.
View Article and Find Full Text PDFProposal of a new clinical entity: Paraprotein negative IL-1 mediated inflammatory dermatosis (PANID) that may precede Schnitzler syndrome.
World Allergy Organ J
September 2023
UCARE Centre, Clinical and Diagnostic Immunology, St. James's Hospital, Dublin, Ireland.
Article Synopsis
- There are variations of SchS that do not meet the full criteria but still respond well to IL-1 blockade, and some patients may experience a rash years before developing the paraprotein.
- The manuscript presents three case studies: one meeting SchS criteria after a long diagnostic delay, another initially not fitting the criteria but later developing a paraprotein, and a third with 12 years of symptoms yet undiagnosed, all of whom improved dramatically
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