Schnitzler syndrome

Common Name(s)

Schnitzler syndrome

Schnitzler syndrome is a rare auto-inflammatory disorder that causes long lasting (chronic) skin rashes and frequent (recurrent) fevers. The rashes feature red, swollen bumps known as hives (urticaria) that usually cover the torso, arms, and legs. Unlike most cases of hives, the bumps seen in Schnitzler syndrome are not itchy in most affected individuals. A fever may occur with the rash, but may also occur separately. The condition may also cause joint pain, bone pain, or muscle pain.

Symptoms of the disease usually appear around 50 years of age and the condition affects men slightly more often than women. Schnitzler syndrome usually does not cause serious issues, but affected individuals should have regular check-ups with a doctor, as many people may develop additional medical complications. About 50% of affected individuals do not have enough red blood cells (anemia), which are needed for transporting oxygen around the body. About 15% of individuals with Schnitzler syndrome develop a specific cancer of the white blood cells.

The exact cause of Schnitzler syndrome is unknown, but the disorder seems to be due to a response from the body’s immune system that causes recurrent episodes of inflammation (auto-inflammatory). The condition is considered in a person who has recurring episodes of hives and fevers. A doctor may also order a blood test to measure a protein called immunoglobulin M (IgM), which is usually elevated in people with Schnitzler syndrome. Treatment usually involves medications that help decrease the response of the immune system. If you have been diagnosed with this condition, talk with your doctor about all treatment options. Support groups can provide additional information and connect you with others affected by this condition.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Schnitzler syndrome" for support, advocacy or research.

Autoinflammatory Alliance

The Autoinflammatory Alliance is a 501 (c)(3) non-profit public charity dedicated to promoting awareness, proper diagnosis and treatment, and improved care for people with autoinflammatory diseases. We started in 2006 under the name, The NOMID Alliance with the same basic goal: to help patients with CAPS and other autoinflammatory diseases. We have a IRS approval as tax-exempt under both names.

Last Updated: 24 Jul 2015

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Schnitzler syndrome" for support, advocacy or research.

Autoinflammatory Alliance

The Autoinflammatory Alliance is a 501 (c)(3) non-profit public charity dedicated to promoting awareness, proper diagnosis and treatment, and improved care for people with autoinflammatory diseases. We started in 2006 under the name, The NOMID Alliance with the same basic goal: to help patients with CAPS and other autoinflammatory diseases. We have a IRS approval as tax-exempt under both names.

http://www.autoinflammatory.org

Last Updated: 24 Jul 2015

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Schnitzler syndrome" returned 11 free, full-text research articles on human participants. First 3 results:

Dysregulation of proinflammatory versus anti-inflammatory human TH17 cell functionalities in the autoinflammatory Schnitzler syndrome.
 

Author(s): Rebecca Noster, Heleen D de Koning, Elisabeth Maier, Martina Prelog, Elke Lainka, Christina E Zielinski

Journal: J. Allergy Clin. Immunol.. 2016 Oct;138(4):1161-1169.e6.

 

TH17 cells have so far been considered to be crucial mediators of autoimmune inflammation. Two distinct types of TH17 cells have been described recently, which differed in their polarization requirement for IL-1β and in their cytokine repertoire. Whether these distinct TH17 phenotypes ...

Last Updated: 14 Aug 2016

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Schnitzler syndrome.
 

Author(s): Jesica Gallo, Sergio Paira

Journal: Reumatol Clin. ;11(2):124-5.

 

Last Updated: 2 Mar 2015

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Schnitzler syndrome: an under-diagnosed clinical entity.
 

Author(s): Tania Jain, Chetan P Offord, Robert A Kyle, David Dingli

Journal: Haematologica. 2013 Oct;98(10):1581-5.

 

Schnitzler syndrome is considered to be a rare disorder characterized by a monoclonal IgM protein and chronic urticaria that is associated with considerable morbidity. We hypothesized that the syndrome may be under-recognized and patients may be deprived of highly effective therapy ...

Last Updated: 4 Oct 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Schnitzler syndrome" returned 2 free, full-text review articles on human participants. First 3 results:

The Schnitzler syndrome.
 

Author(s): Dan Lipsker

Journal:

 

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. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial ...

Last Updated: 11 Jan 2011

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The Schnitzler syndrome. Four new cases and review of the literature.
 

Author(s): D Lipsker, Y Veran, F Grunenberger, B Cribier, E Heid, E Grosshans

Journal: Medicine (Baltimore). 2001 Jan;80(1):37-44.

 

The Schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver ...

Last Updated: 5 Feb 2001

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Tocilizumab in Patients With Schnitzler's Syndrome
 

Status: Not yet recruiting

Condition Summary: Schnitzler's Syndrome

 

Last Updated: 9 Feb 2017

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