Schnitzler Syndrome

How to pronounce it:

Schnitzler - shh-nitz-ler


AiArthritis defines Schnitzler syndrome as:


Schnitzler syndrome is a rare autoinflammatory disease. It is characterized with patients having non-pruritic urticaria (or non-itchy hives) and blood abnormalities including elevated levels of specific proteins (monoclonal IgM gammopathy).



Symptoms

Symptoms of AiArthritis Diseases Regardless of Diagnosis

Common in All AiArthritis Diseases



  • Periods of worsening symptoms are called flares. A flare can last for hours, days, weeks, or months.
  • Condition improves with activity and exercise and worsens with rest.
  • When inflammation is left uncontrolled due to lack of proper treatment, comorbidities can develop. 70% of patients with chronic, lifelong disease will develop comorbidities, including dual or triple diagnoses.

 “Auto” Symptoms






  • Severe fatigue or exhaustion that may not be helped by caffeine/stimulants and can happen even after a long period of rest.
  • “Brain fog” - or periods of time where thinking gets clouded and it becomes difficult to concentrate.
  • Flu-like symptoms, without having the flu- nausea, muscle weakness, and general malaise.
  • Fever, typically low grade in autoimmune and higher grade in autoinflammatory (% strongly varies per disease).


Reference: Early Symptoms of AiArthritis Study, AiArthritis, 2019.


View Early Symptoms Study

Inflammatory Arthritis Symptoms


  • Severe stiffness in one or more joints, especially in the morning or after sitting for long periods of time. 
  • Episodes of joint pain that may last for hours, days, or even weeks, that can appear and disappear suddenly. Often described as “jumping pain” into different locations.


Typically the joint pain will coincide with one or more “Auto” symptoms and start and stop suddenly - for no apparent reason (which is called a "flare").  Some people will experience all of the above symptoms, others only a few.  If you have any of the arthritis features, and at least one of the “Auto” features, please consult your physician about a referral to a rheumatologist.


Symptoms Often Associated with Schnitzler Syndrome:

  • Chronic non-pruritic urticaria (non-itchy hives)
  • Organomegaly (increased size of several organs)
  • Bone pain
  • Enlarged lymph nodes


What fellow patients want you to know

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More About Schnitzler Syndrome

Diagnosing Schnitzler Syndrome

There’s no single definitive test that can confirm a Schnitzler syndrome diagnosis, so rheumatologists look at a combination of physical exam, blood tests, and imaging, along with a family history of autoimmune or autoinflammatory disease.


For Schnitzler syndrome diagnosis, patient must have both of the major criteria:

  • recurrent urticarial rash
  • monoclonal IgM gammopathy


Plus at least 2 of the following minor criteria:

  • recurrent fever
  • objective signs of abnormal bone remodeling
  • elevated CRP level or leukocytosis
  • neutrophilic infiltrate on skin biopsy
  • joint pain and inflammation
  • enlarged lymph nodes
  • enlarged organs
  • elevated red blood cells or sedimentation rate


Someone can also get diagnosed if they have monoclonal IgM gammopathy and 3 of the secondary symptoms.

Interesting Facts about this Disease

  • Schnitzler syndrome was first described in the medical literature in 1972, by a French dermatologist named Liliane Schnitzler.
  • There are only about 300 or so known cases of Schnitzler in the world.
  • Most of the reported cases of Schnitzler syndrome have been from Europe, particularly France, but cases from Australia, Japan and the United States have been reported too.

Other Resources

In an effort to ensure this page has the most accurate and up-to-date information, this page is currently awaiting medical review. Some information is subject to change.


Page Last Updated: 9/1/2023

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