Schnitzler Syndrome
Encyclopedias as Topic
Urticaria
Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. (1/9)
BACKGROUND: Schnitzler's syndrome is an inflammatory disorder characterised by chronic urticarial rash and monoclonal gammopathy, accompanied by periodic fever, arthralgia or arthritis, and bone pain. The cause and treatment are still unknown. OBJECTIVE: To assess treatment with thalidomide and an interleukin 1 receptor antagonist, anakinra, in Schnitzler's syndrome. CASE REPORTS: Three patients with Schnitzler's syndrome are described, one with IgM gammopathy, two with IgG type. In one patient, thalidomide induced complete remission, but was stopped because of polyneuropathy. Anakinra 100 mg daily in all three patients led to disappearance of fever and skin lesions within 24 hours. After a follow up of 6-18 months, all patients are free of symptoms. CONCLUSION: Anakinra proved to be effective in three patients with Schnitzler's syndrome. This treatment is preferable to thalidomide, which induced a complete remission in one of our patients, as it has fewer side effects. (+info)Time-course analysis of serum hepcidin, iron and cytokines in a C282Y homozygous patient with Schnitzler's syndrome treated with IL-1 receptor antagonist. (2/9)
(+info)Dysfunctional inflammasome in Schnitzler's syndrome. (3/9)
(+info)Cryopyrin-associated periodic syndrome: an update on diagnosis and treatment response. (4/9)
(+info)The Schnitzler syndrome. (5/9)
(+info)Effects of the histone deacetylase inhibitor ITF2357 in autoinflammatory syndromes. (6/9)
(+info)Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: new insight into the biology of Schnitzler syndrome. (7/9)
(+info)Effect of in vitro and in vivo anakinra on cytokines production in Schnitzler syndrome. (8/9)
(+info)Schnitzler Syndrome is a rare autoinflammatory disorder characterized by the recurrent occurrence of erythema (skin rash), often resembling chronic urticaria, and arthralgia or arthritis (joint pain or inflammation). It is typically associated with monoclonal gammopathy, usually of IgM type. Other common features may include fever, lymphadenopathy (swollen lymph nodes), hepatosplenomegaly (enlarged liver and spleen), bone pain, and fatigue. The exact cause of Schnitzler Syndrome is not known, but it is thought to be related to an abnormal immune response. Treatment typically involves the use of medications that suppress the immune system, such as steroids or biologic agents.
An encyclopedia is a comprehensive reference work containing articles on various topics, usually arranged in alphabetical order. In the context of medicine, a medical encyclopedia is a collection of articles that provide information about a wide range of medical topics, including diseases and conditions, treatments, tests, procedures, and anatomy and physiology. Medical encyclopedias may be published in print or electronic formats and are often used as a starting point for researching medical topics. They can provide reliable and accurate information on medical subjects, making them useful resources for healthcare professionals, students, and patients alike. Some well-known examples of medical encyclopedias include the Merck Manual and the Stedman's Medical Dictionary.
Urticaria, also known as hives, is an allergic reaction that appears on the skin. It is characterized by the rapid appearance of swollen, pale red bumps or plaques (wheals) on the skin, which are often accompanied by itching, stinging, or burning sensations. These wheals can vary in size and shape, and they may change location and appear in different places over a period of hours or days. Urticaria is usually caused by an allergic reaction to food, medication, or other substances, but it can also be triggered by physical factors such as heat, cold, pressure, or exercise. The condition is generally harmless, but severe cases of urticaria may indicate a more serious underlying medical issue and should be evaluated by a healthcare professional.
Monoclonal gammopathy of undetermined significance (MGUS) is a medical condition characterized by the presence of a monoclonal protein, or M-protein, in the blood or urine, but without any signs or symptoms of related disorders. The M-protein is produced by a single clone of plasma cells, which are a type of white blood cell found in the bone marrow.
In MGUS, the level of M-protein is typically low (less than 3 grams per deciliter), and there are no signs of damage to organs such as the bones, kidneys, or nervous system. However, people with MGUS have a higher risk of developing certain related conditions, such as multiple myeloma, amyloidosis, or lymphoplasmacytic lymphoma, compared to those without MGUP.
MGUS is usually detected through routine blood or urine tests and is typically asymptomatic. However, in some cases, people with MGUS may experience symptoms such as fatigue, bone pain, or recurrent infections. If these symptoms occur, further testing may be necessary to determine if MGUS has progressed to a more serious condition.
It's important to note that MGUS is not a cancer itself, but rather a potential precursor to certain types of cancer. Regular monitoring with blood or urine tests and physical examinations is recommended for people diagnosed with MGUS to monitor for any changes that may indicate progression to a more serious condition.
Schnitzler syndrome
Liliane Schnitzler
Immunoglobulin M
Anakinra
Thought disorder
Rudiger syndrome
Echophenomenon
Imitative learning
Echopraxia
Marcus Sedgwick
Echolalia
Osteosclerosis
Autoimmune urticaria
Schnitzler
Periodic fever syndrome
CHD3
List of syndromes
List of MeSH codes (C20)
Autoinflammatory diseases
Neonatal-onset multisystem inflammatory disease
Roland Mertelsmann
MLH1
Angela Winkler
CHD4
Bat
List of eponymous diseases
Hypomagnesemia with secondary hypocalcemia
Alemtuzumab
List of skin conditions
Judith Thompson
Schnitzler syndrome - Wikipedia
Schnitzler Syndrome: Practice Essentials, Background, Pathophysiology
Schnitzler Syndrome: Practice Essentials, Background, Pathophysiology
Schnitzler syndrome stories | Diseasemaps
Schnitzler Syndrome - I Need Medic
Canakinumab (Ilaris) - Medical Clinical Policy Bulletins | Aetna
Autoinflammatory syndromes in dermatology]
Clinical symptoms and molecular investigations in 13 patients with Schnitzler syndrome identified at the single UK centre |...
Striking effect of the IL-1 receptor antagonist anakinra in chronic urticarial rash with polyclonal increase in IgA and IgG
Prof. Patrick Ragert, PhD | Max Planck Institute for Human Cognitive and Brain Sciences
Complete Publications | Max Planck Institute for Human Cognitive and Brain Sciences
Chronic recurrent wheals - If not chronic spontaneous urticaria, what else? - International Association for the Study of Pain ...
MEA2100-Systems | www.multichannelsystems.com
MEA2100-Systems | www.multichannelsystems.com
Carlos Antonio Torres-Cabala | MD Anderson Cancer Center
Jahresbericht 2016 AG Atypische Parkinson-Syndrome - DPG e. V.
Asthma Allergy Immunology
Asthma Allergy Immunology
Specific PHGKB|Rare Diseases PHGKB|PHGKB
Taselisib in Combination with Fulvestrant for Advanced ER Positive, HER2-negative Breast Cancer - Second Line After Aromatase...
Canakinumab
Dr. Igor Vujic - Wissenschaftliche Arbeiten & Forschung
Kprp Mouse Gene Details | keratinocyte expressed, proline-rich | International Mouse Phenotyping Consortium
Mouse IL-1 alpha / IL-1F1 Protein, His Tag (MALS verified) - ACROBiosystems
Search All Tech Briefings - Page 3 - NIHR
Autoinflammatory syndromes - Biomedicalfacts
Waldenstrom Macroglobulinemia Clinical Presentation: History, Physical Examination
Toe syndactyly (Concept Id: C0265660) - MedGen - NCBI
Schnitzlers syndrom
Cryopyrin-associated periodic syndromes3
- Urticarial vasculitis (UV) and autoinflammatory syndromes, i.e., cryopyrin-associated periodic syndromes (CAPS) and Schnitzler syndrome (SchS), can mimic CSU-like rash but represent rare disorders with systemic symptoms including fever, headache, conjunctivitis, and arthralgia. (iasp-pain.org)
- It is used to treat cryopyrin-associated periodic syndromes, familial mediterranean fever, juvenile arthritis, and mevalonate kinase deficiency in the USA. (pharmakb.com)
- It has been approved in Europe to treat cryopyrin-associated periodic syndromes, gouty arthritis, and juvenile arthritis. (pharmakb.com)
Liliane Schnitzler2
- The disease is named after the French dermatologist Liliane Schnitzler who first described this syndrome in 1972. (wikipedia.org)
- It was first described by Dr. Liliane Schnitzler in 1972, and since then, the understanding of the disease has grown significantly. (ineedmedic.com)
SchS2
- Schnitzler syndrome (SchS) was first described in 1972 and to date 281 cases have been reported. (biomedcentral.com)
- However, the mechanism leading to the increased IL-1 Se hela listan på ojrd.biomedcentral.com Schnitzler syndrome (SchS) is a late-onset autoinflammatory disease characterized by the association of a chronic urticarial rash and monoclonal gammopathy with signs and symptoms of systemic inflammation. (web.app)
Periodic4
- Schnitzler syndrome or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives (urticaria) and periodic fever, bone pain and joint pain (sometimes with joint inflammation), weight loss, malaise, fatigue, swollen lymph glands and enlarged spleen and liver. (wikipedia.org)
- Other conditions which can cause periodic fevers, paraproteins or chronic hives that should be ruled out, include (and are not limited to) autoimmune or autoinflammatory disorders such as adult-onset Still's disease, angioedema, hematological disorders such as lymphoma or monoclonal gammopathy of undetermined significance (MGUS), other causes of hives, cryoglobulinemia, mastocytosis, chronic neonatal onset multisystem inflammatory disease or Muckle-Wells syndrome. (wikipedia.org)
- Mutations in the NLRP3 gene (nucleotide-binding oligoisomerization domain [NOD]-like [NLR] family pyrin domain containing 3) that are also found in cryopyrin-associated periodic syndrome (CAPS) has been found in a few Schnitzler syndrome patients. (medscape.com)
- Hereditary periodic fever syndromes, also called autoinflammatory syndromes, are characterized by relapsing fever and additional manifestations such as skin rashes, mucosal manifestations, or arthralgias. (nih.gov)
Late-onset autoinflammatory1
- Schnitzler syndrome is a late-onset autoinflammatory disorder. (wikipedia.org)
20201
- Dec 4, 2020 Alberth and Schnitzler reported a 10% incidence of irreversible mydriasis following PKP, but IOP was normal in all patients. (web.app)
Diagnosis4
- Because anakinra is so highly and rapidly effective for inducing complete remission of Schnitzler syndrome, it has been suggested that in patients who do not respond to anakinra, the diagnosis should be reconsidered. (wikipedia.org)
- The diagnosis of Schnitzler syndrome can be challenging, and it often requires the exclusion of other causes of chronic urticarial rash. (ineedmedic.com)
- The diagnosis rests on clinical criteria, a Schnitzler Syndrome. (web.app)
- Still's disease is a syndrome, meaning that the diagnosis is based on excluding other causes (that may look like Still's) and having typical symptoms, findings, and lab results compatible with a Still's diagnosis. (stillsnow.com)
Autoinflammatory disease characterized1
- Schnitzler syndrome is an autoinflammatory disease characterized by chronic, nonpruritic urticaria in association with monoclonal gammopathy. (medscape.com)
Schnitzler's4
- 2] Background/Purpose: Schnitzler's syndrome is characterized by chronic urticaria, intermittent fever, arthralgia, bone pain, gammopathy and marked systemic inflammation. (web.app)
- The striking response to IL-1 blockade suggests that Schnitzler's syndrome is an IL-1 mediated condition of the expanding spectrum of systemic autoinflammatory disorders. (web.app)
- Se hela listan på ctajournal.biomedcentral.com Schnitzler's syndrome is characterized by recurrent urticarial rash and monoclonal gammopathy, associated with clinical and biological signs of inflammation and a long‐term risk of AA amyloidosis and overt lymphoproliferation. (web.app)
- Schnitzler's syndrome: Increased levels of bone formation and angiogenesis factors are reduced after successful pefloxacin treatment. (allthingsmanifestation.com)
Monoclonal gammopathy5
- Schnitzler syndrome is a rare autoinflammatory disorder that is characterized by chronic urticarial rash and monoclonal gammopathy. (ineedmedic.com)
- Schnitzler syndrome is also associated with monoclonal gammopathy, which is characterized by the presence of a monoclonal immunoglobulin in the blood. (ineedmedic.com)
- The monoclonal gammopathy is usually of the IgM subtype, and it is often found in the serum and/or urine of patients with Schnitzler syndrome. (ineedmedic.com)
- The presence of monoclonal gammopathy is a distinguishing feature of Schnitzler syndrome, and it is not seen in other forms of chronic urticaria. (ineedmedic.com)
- It has been suggested that the monoclonal gammopathy seen in Schnitzler syndrome is the result of clonal expansion of B-cells, which are responsible for the production of immunoglobulins. (ineedmedic.com)
Rash3
- Rash of Schnitzler syndrome. (medscape.com)
- The hallmark of Schnitzler syndrome is a chronic urticarial rash, which is often described as pruritic and recurrent. (ineedmedic.com)
- Schnitzler syndrome is a rare disorder characterized by a chronic reddish rash that resembles hives (urticaria) and elevated levels of a specific protein in the blood (monoclonal IgM gammopathy). (web.app)
Etiology1
- The etiology of Schnitzler syndrome remains unknown. (web.app)
Interleukin1
- Schnitzler syndrome is considered an autoinflammatory disorder and is generally treated with anakinra, which inhibits interleukin 1. (wikipedia.org)
Familial1
- Schnitzler syndrome is a sporadic disorder, meaning it is not inherited in a familial pattern. (ineedmedic.com)
Recurrent1
- When to Suspect Autoinflammatory/Recurrent Fever Syndromes. (biomedicalfacts.com)
Biomarkers1
- Discrimination of COVID-19 from inflammation-induced cytokine storm syndromes by disease-related blood biomarkers. (immunaid.eu)
Clinical2
- Schnitzler syndrome may be present in up to 1.5% of patients with a monoclonal IgM in their serum and likely under-recognized as a clinical syndrome. (web.app)
- Identification of Critical Transcriptomic Signalling Pathways in Patients with H Syndrome and Rosai-Dorfman Disease, Journal of Clinical Immunology volume 41, pages 441-457 (2021). (immunaid.eu)
Abnormalities1
- Infiltration of the CNS by the malignant clone can cause a syndrome of confusion, memory loss, disorientation, and motor abnormalities called the Bing-Neel syndrome. (medscape.com)
Pathophysiology2
- Schnitzler syndrome is an autoinflammatory disease for which the exact pathophysiology remains unclear but seems to involve the innate immune system. (medscape.com)
- The pathophysiology of Schnitzler syndrome is not fully understood, but it is thought to be related to abnormal activation of the immune system. (ineedmedic.com)
Vasculitis1
- Urticaria needs to be differentiated from other medical conditions where wheals, angioedema, or both can occur, e.g. anaphylaxis, autoinflammatory syndromes, urticarial vasculitis, orbradykinin-mediated angioedema including hereditary angioedema (HAE). (cyberderm.net)
Pathogenesis1
- Another possible mechanism for the pathogenesis of Schnitzler syndrome is the activation of the adaptive immune system. (ineedmedic.com)
Mutations2
- Several genetic mutations have been found in Schnitzler syndrome patients, though not consistently. (medscape.com)
- Novel somatic mutations in UBA1 as a cause of VEXAS syndrome Blood. (immunaid.eu)
Fever1
- Autoinflammatory syndromes: fever is not always a sign of infection. (biomedicalfacts.com)
Chronic urticaria1
- Schnitzler syndrome - Schnitzler syndrome, which is often unrecognized, is a form of chronic urticaria associated with monoclonal … The autoinflammatory diseases: An overview View in Chinese …consideration of cyclic neutropenia, which may be of childhood or adult onset. (web.app)
Disorder3
- Craniofrontonasal syndrome is an X-linked developmental disorder that shows paradoxically greater severity in heterozygous females than in hemizygous males. (nih.gov)
- However, when the human body is frequently flooded with larg Down syndrome, or trisomy 21, is a genetic disorder and chromosomal condition characterized by a third copy of chromosome 21. (web.app)
- However, approximately 10-15% of people with Schnitzler syndrome develop a lymphoproliferative disorder. (web.app)
Auto-inflammatory2
- 19 A central role for IL-1β is illustrated by the high efficacy of anti-IL-1β therapy in patients with Schnitzler syndrome… 2019-02-14 The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. (web.app)
- The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. (web.app)
Patients6
- The life span in patients with Schnitzler syndrome has not been shown to differ much from the general population. (wikipedia.org)
- Radiologic evaluation shows evidence of hyperostosis in 35% of Schnitzler syndrome patients. (medscape.com)
- Patients with Schnitzler syndrome can also present with bone pain, which is often located in the long bones of the extremities. (ineedmedic.com)
- In one study, a genetic variant in the NLRP3 gene, which is involved in the inflammasome pathway, was identified in a small number of patients with Schnitzler syndrome. (ineedmedic.com)
- Interhemispheric motor networks are abnormal in patients with Gilles de la Tourette syndrome. (uniklinik-duesseldorf.de)
- Imitation in patients with Gilles de la Tourette syndrome--a behavioral study. (uniklinik-duesseldorf.de)
Amyloidosis1
- AA amyloidosis has also been reported in people with Schnitzler syndrome. (wikipedia.org)
Tourette2
- Altered pattern of motor cortical activation-inhibition during voluntary movements in Tourette syndrome. (uniklinik-duesseldorf.de)
- Increased sensory feedback in Tourette syndrome. (dagstuhl.de)
Incidence1
- Schnitzler syndrome is a rare disease, and the true incidence and prevalence are not well-established due to underdiagnosis and misdiagnosis. (ineedmedic.com)
Therapy1
- Therapy options in irritable bowel syndrome Eur J Gastroenterol Hepatol 22(12): 1402-1411. (uniklinik-duesseldorf.de)
Disorders1
- Autoinflammatory syndromes (AIS) are a group of related inflammatory disorders. (biomedicalfacts.com)
Disease2
- Schnitzler syndrome is a rare disease, with an estimated prevalence of less than 1 per million individuals. (ineedmedic.com)
- Schnitzler syndrome is a very rare immunological disease. (web.app)
Variant1
- Other variants, including a chronic ataxic neuropathy known as Miller-Fisher syndrome (a variant of Guillain-Barré syndrome), have been described. (medscape.com)
Systemic2
- Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/systemic/schnitzler.jpg). (medscape.com)
- Sjögren syndrome (SS) is a systemic long-term auto-immune condition which affects glands in the body's moisture-producing surfaces. (wikenigma.org)
Investigation1
- Investigation of a nosocomial outbreak of severe acute respiratory syndrome (SARS) in Toronto, Canada. (cdc.gov)
Severe3
- GLI3-related Pallister-Hall syndrome (GLI3-PHS) is characterized by a spectrum of anomalies ranging from polydactyly, asymptomatic bifid epiglottis, and hypothalamic hamartoma at the mild end to laryngotracheal cleft with neonatal lethality at the severe end. (nih.gov)
- Antibody response after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination is impaired in kidney transplant recipients. (lww.com)
- Foundations of the severe acute respiratory syndrome preparedness and response plan for healthcare facilities. (cdc.gov)
People4
- The long-term outlook (prognosis) for people with Schnitzler syndrome is generally good. (web.app)
- For people with Down syndrome, family members, caregivers and professionals. (advocatehealth.com)
- GERD is common in people with Down syndrome (DS). (advocatehealth.com)
- It is more common in people with Down syndrome. (advocatehealth.com)
Genetic1
- However, some cases of Schnitzler syndrome have been reported in families, suggesting a possible genetic predisposition. (ineedmedic.com)
Years1
- However, cases of Schnitzler syndrome have been reported in individuals as young as 17 years and as old as 83 years. (ineedmedic.com)