Schnitzler syndrome

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Schnitzler syndrome
Other namesSchnitzler's syndrome
SpecialtyImmunology Edit this on Wikidata

Schnitzler syndrome or Schnitzler's syndrome is a

swollen lymph glands and enlarged spleen and liver.[1][2]

Schnitzler syndrome is considered an

interleukin 1. This treatment controls the condition but does not cure it. Around 15% of people develop complications, but the condition generally does not shorten life.[1]

Classification

Schnitzler syndrome is a late-onset

autoinflammatory disorder.[1]

Signs and symptoms

The typical onset is at around 55 years old, and the symptoms are recurrent hives, mostly on the torso and limbs, often with recurring fever, joint pain, bone pain, muscle pain, headache, fatigue, and loss of weight.[1]

Cause

As of 2017 the cause of the disease was not understood.[1]

Diagnosis

IgG is raised has been described, which appears to be one-tenth as common. The immunoglobulins may show up in the urine as Bence Jones proteins. Signs of inflammation are often present: these include an increased white blood cell count (leukocytosis) and a raised erythrocyte sedimentation rate and C-reactive protein. There can be anemia of chronic disease.[3] Bone abnormalities can be seen on radiological imaging (often increased density or osteosclerosis) or biopsy.[1]

There are two sets of diagnostic criteria, the Lipsker criteria published in 2001 and the Strasbourg criteria that were produced at a meeting in that city in 2012.[1]

The Lipsker criteria require hives, the presence of monoclonal IgM, and at least 2 of the following: fever, joint pain or arthritis, bone pain, swollen lymph nodes, enlarged spleen or liver, elevated erythrocyte sedimentation rate, high levels of white blood cells, and findings of problems in bone imaging.[1][3]

In the Strasbourg criteria, the person must have hives and the presence of monoclonal IgM or IgG. Schnitzler's is diagnosed if the person has IgM and two of the following, or IgG and three of the following: recurrent fevers, abnormalities in bone imaging, with or without bone pain, findings of neutrophil infiltration in a skin biopsy, high levels of white blood cells or C-reactive protein.[1][4]

neonatal onset multisystem inflammatory disease or Muckle–Wells syndrome.[1][5]

Treatment

As of 2017, no drug was approved to treat Schnitzlers. Drugs that inhibit

interferon alpha may be effective.[1] A 2020 review reported that canakinumab was "an effective long-term treatment with a favorable safety profile in patients with Schnitzler syndrome".[6]

In June 2018 NHS England published a Clinical Commissioning Policy: Anakinra to treat periodic fevers and autoinflammatory disorders (all ages) which stated that "Anakinra may be used as a first line treatment in patients with a documented diagnosis of Schnitzler syndrome".[7]

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.[8] Anakinra is not curative, however; symptoms recur soon after treatment stops.[9]

Outcomes

Generally treatment with anakinra prevents outbreaks but they resume if treatment is stopped.

Waldenström's macroglobulinemia, develops. AA amyloidosis has also been reported in people with Schnitzler syndrome.[1]

The life span in patients with Schnitzler syndrome has not been shown to differ much from the general population.[10]

Epidemiology

It is a rare condition; as of September 2014, 281 cases had been reported[9] and as of 2017 around 300 cases had been reported.[1]

History

The disease is named after the French dermatologist Liliane Schnitzler who first described this syndrome in 1972.[11][9] A Delphi study on the taxonomy and definition of auto-inflammatory diseases, published in 2018, considered the alternative name "late onset gammopathy with recurrent urticaria and fever" but this received little support.[12]

See also

References

  1. ^
    S2CID 13780498
    .
  2. PMID 35089885
    .
  3. ^
    S2CID 6023980
    .
  4. S2CID 12831354
    .
  5. PMID 21886751
    .
  6. S2CID 219398134
    .
  7. ^ NHS England (29 June 2018). Clinical Commissioning Policy: Anakinra to treat periodic fevers and autoinflammatory disorders (all ages) (PDF). p. 18. Retrieved 11 July 2018.
  8. PMID 27564982
    .
  9. ^
    PMID 25905009
    .
  10. PMID 17586002
    .
  11. ^ L. Schnitzler, Lésions urticariennes chroniques permanentes (érythème pétaloïde?) Cas cliniques No 46 B, J Dermatol Angers (1972) Abstract 46.
  12. PMID 30100561
    .

External links

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