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Schnitzler Syndrome

How to pronounce it: Schnitzler - shh-nitz-ler


AiArthritis defines Schnitzler Syndrome as:

Schnitzler syndrome is a rare autoinflammatory disease. People with this condition usually have non-itchy hives (urticaria) and abnormal levels of certain proteins in their blood, especially a protein called monoclonal IgM gammopathy.

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  • Characteristic red, hive-like rash on forearm typical of Schnitzler Syndrome's chronic urticaria

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  • Doctor examining patient's lymph nodes, as enlarged lymph nodes are a common feature of Schnitzler Syndrome

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  • Person experiencing bone and joint pain in leg, a hallmark symptom of Schnitzler Syndrome that often affects the larger bones

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Symptoms & Characteristics

Common in All AiArthritis Diseases

Flares: Periods of worsening symptoms are called flares. A flare can last for hours, days, weeks, or months.


Physical Activity: Condition improves with activity and exercise and worsens with rest.


Comorbidities: 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.


Family History: Autoimmune diseases often run in families, indicating a potential genetic predisposition where that gene can cause disease. Autoinflammatory diseases can occur multiple times in a family, but is based off of genetic mutation. It is not a gene that causes the disease— but a mutation on the gene that can cause the disease which can then be passed on to the next generation.

"Auto" Symptoms

Fatigue: Severe fatigue or exhaustion that may not be helped by caffeine/stimulants and can happen even after a long period of rest.


Cognitive Dysfunction: 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 (with exception of juvenile idiopathic arthritis) and higher grade in autoinflammatory (% strongly varies per disease).


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


View Early Symptom Study

Inflammatory Arthritis Symptoms

Stiffness: Severe stiffness in one or more joints, especially in the morning or after sitting for long periods of time. 


Joint Pain: 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 specialist.


Symptoms Often Associated with Schnitzler Syndrome

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

  • Diagnosing Schnitzler Syndrome

    There’s no single test that can confirm a Schnitzler syndrome diagnosis, so rheumatologists rely on a combination of physical exams, blood tests, imaging, and family history of autoimmune or autoinflammatory disease.


    To diagnose Schnitzler syndrome, patients must meet both of the major criteria:


    Recurrent urticarial (hive-like) rash

    Monoclonal IgM gammopathy (abnormal protein in the blood)


    Additionally, they must have at least two of the following minor criteria:


    • Recurrent fever
    • Objective signs of abnormal bone remodeling
    • Elevated CRP levels or leukocytosis (high white blood cell count)
    • Neutrophilic infiltrate on skin biopsy
    • Joint pain and inflammation
    • Enlarged lymph nodes
    • Enlarged organs (e.g., spleen or liver)
    • Elevated red blood cell count or sedimentation rate (ESR)

    Alternatively, a diagnosis can also be made if someone has monoclonal IgM gammopathy and three of the minor criteria.


    While there are no specific ACR or EULAR guidelines for Schnitzler syndrome, these organizations emphasize the importance of systematic evaluation and differentiating between similar autoinflammatory disorders, ensuring accurate diagnosis and proper management.


    For a more definitive diagnosis, additional tests may include skin biopsies, lumbar punctures, and hearing or eye exams, especially when neurological or sensory symptoms are present. Genetic testing is often critical in diagnosing CAPS, as it can identify mutations in the NLRP3 gene commonly associated with the condition.


    While there are no specific ACR or EULAR guidelines for CAPS, both organizations emphasize the importance of genetic testing and comprehensive clinical evaluation to differentiate CAPS from other autoinflammatory syndromes, ensuring an accurate diagnosis and proper treatment approach.


  • Schnitzler Syndrome Treatment Options

    Treatments are tailored to each individual's disease, but visit our Treatment Options page to learn more about the different types of treatments that are used for Schnitzler Syndrome.

    Treatment Options

  • What Fellow Schnitzler Syndrome Patients Want You to Know

    We need YOUR advice! Fill out this form to give peer advice to those with your disease.

    Submit YOUR Advice

  • Interesting Facts about Schnitzler Syndrome

    • Schnitzler syndrome was first described by Dr. Liliane Schnitzler in 1972, who noted a combination of chronic urticaria (hives) and monoclonal gammopathy in patients. The condition is named after her, and it remains a rare autoinflammatory disorder that affects both the skin and immune system.

    • 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.

  • Schnitzler Syndrome Awareness Days/Months



  • Other Schnitzler Syndrome Resources

    AiArthritis 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: 10/31/2024

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