schnitzler's syndrome - HEALTHIES
ascopubs.org: Ibrutinib for the Management of Schnitzler Syndrome: A Novel Therapy for a Rare Condition Ibrutinib for the Management of Schnitzler Syndrome: A Novel Therapy for a Rare Condition Schnitzler syndrome or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives (urticaria) and periodic fever, bone and joint pain (sometimes with joint inflammation), weight loss, malaise, fatigue, swollen lymph glands and enlarged spleen and liver. [1][2] Schnitzler syndrome is characterized by an immunoglobulin M (IgM) monoclonal gammopathy and a unique urticarial rash, and it is often associated with additional symptoms such as bone or joint pain, enlarged lymph nodes, recurrent fever, liver or spleen enlargement, and elevated inflammatory markers such as C-reactive protein (CRP). Schnitzler’s Syndrome (SS) is a rare late-onset acquired autoinflammatory disorder which consists of chronic urticaria associated with a monoclonal IgM-kappa gammopathy, arthralgias, skeletal hyperostosis, lymphadenopathy, and recurrent constitutional symptoms.
Understanding the Context
Schnitzler syndrome is rare. It was named after a French dermatologist, Dr Liliane Schnitzler, who described the condition in 1972. What causes Schnitzler syndrome? The cause of Schnitzler syndrome is unknown.
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Key Insights
Alterations in the cytokine network have been reported. Schnitzler syndrome is an autoinflammatory disease characterized by chronic, nonpruritic urticaria in association with monoclonal gammopathy. Associated sympoms include recurrent fever, bone... Schnitzler syndrome is an unusual late-onset acquired autoinflammatory syndrome. It must be considered in patients with refractory chronic urticarial rash associated with IgM monoclonal gammopathy.
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Schnitzler Syndrome is a rare and underdiagnosed autoinflammatory disease first described by French dermatologist Liliane Schnitzler in 1972. It is characterized by a combination of chronic urticarial rash, monoclonal gammopathy (typically IgM), and systemic inflammation. Schnitzler syndrome is a rare autoinflammatory condition. Signs and symptoms of the condition vary but may include urticaria; recurrent fevers; joint pain and inflammation; organomegaly (abnormally enlarged organs); and/or blood abnormalities. Schnitzler syndrome | About the Disease | GARD - Genetic and Rare ... If you have been referred to us because of suspicion that you may have a fever syndrome such as Schnitzler’s syndrome, this does NOT mean that you have amyloidosis.
Learn about Schnitzler Syndrome, including symptoms, causes, and treatments. If you or a loved one is affected by this condition, visit NORD to find resources.