Schnitzler syndrome: Difference between revisions
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Schnitzler syndrome is considered an [[autoinflammatory disorder|autoinflammatory]] and [[autoimmune disorder]].<ref name="Tinazzi">{{cite journal|last1=Tinazzi|first1=Elisa|last2=Puccetti|first2=Antonio|last3=Patuzzo|first3=Giuseppe|last4=Sorleto|first4=Michele|last5=Barbieri|first5=Alessandro|last6=Lunardi|first6=Claudio|title=Schnitzler syndrome, an autoimmune–autoinflammatory syndrome: Report of two new cases and review of the literature|journal=Autoimmunity Reviews|date=May 2011|volume=10|issue=7|pages=404–409|doi=10.1016/j.autrev.2011.01.003}}<!--|accessdate=15 February 2015--></ref> Chronic hives and a [[monoclonal gammopathy]] have been proposed as the major criteria, while the others represent minor criteria.<ref name="pmid11204501">{{cite journal |vauthors=Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E |title=The Schnitzler syndrome. Four new cases and review of the literature |journal=Medicine |volume=80 |issue=1 |pages=37–44 |date=January 2001 |pmid=11204501 |doi= 10.1097/00005792-200101000-00004|url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0025-7974&volume=80&issue=1&spage=37}}</ref> |
Schnitzler syndrome is considered an [[autoinflammatory disorder|autoinflammatory]] and [[autoimmune disorder]].<ref name="Tinazzi">{{cite journal|last1=Tinazzi|first1=Elisa|last2=Puccetti|first2=Antonio|last3=Patuzzo|first3=Giuseppe|last4=Sorleto|first4=Michele|last5=Barbieri|first5=Alessandro|last6=Lunardi|first6=Claudio|title=Schnitzler syndrome, an autoimmune–autoinflammatory syndrome: Report of two new cases and review of the literature|journal=Autoimmunity Reviews|date=May 2011|volume=10|issue=7|pages=404–409|doi=10.1016/j.autrev.2011.01.003}}<!--|accessdate=15 February 2015--></ref> Chronic hives and a [[monoclonal gammopathy]] have been proposed as the major criteria, while the others represent minor criteria.<ref name="pmid11204501">{{cite journal |vauthors=Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E |title=The Schnitzler syndrome. Four new cases and review of the literature |journal=Medicine |volume=80 |issue=1 |pages=37–44 |date=January 2001 |pmid=11204501 |doi= 10.1097/00005792-200101000-00004|url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0025-7974&volume=80&issue=1&spage=37}}</ref> |
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==Classification== |
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Schnitzler syndrome is a late-onset [[autoinflammatory disorder]].<ref name=Gusdorf2017>{{cite journal|last1=Gusdorf|first1=L|last2=Lipsker|first2=D|title=Schnitzler Syndrome: a Review.|journal=Current rheumatology reports|date=August 2017|volume=19|issue=8|pages=46|doi=10.1007/s11926-017-0673-5|pmid=28718061}}</ref> |
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==Signs and symptoms== |
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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.<ref name=Gusdorf2017> |
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==Cause== |
==Cause== |
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==Treatment== |
==Treatment== |
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As of 2017 no drug was approved to treat Schnitlzers.<ref name=Gusdorf2017> Drugs that inhibit [[interleukin 1]] activity have been the preferred treatment since they emerged in 2005; since 2012 a consensus guideline has recommended treatment with [[anakirna]].<ref name=Gusdorf2017> [[Immunosuppressant]] drugs such as [[corticosteroids]], [[cyclooxygenase inhibitors]], [[interferon alpha]] may be effective.<ref name=Gusdorf2017> |
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[[Antihistamines]] are not effective in treating the hives in this condition. It may respond to [[immunosuppressant]] drugs such as [[corticosteroids]], [[cyclooxygenase inhibitors]], [[interferon alpha]], [[interleukin 1 receptor antagonist]]s ([[Anakinra]]),<ref name="pmid16096327">{{cite journal |vauthors=de Koning HD, Bodar EJ, Simon A, van der Hilst JC, Netea MG, van der Meer JW |title=Beneficial response to anakinra and thalidomide in Schnitzler's syndrome |journal=Annals of the Rheumatic Diseases |volume=65 |issue=4 |pages=542–4 |date=April 2006 |pmid=16096327 |doi=10.1136/ard.2005.045245 |url=http://ard.bmj.com/cgi/pmidlookup?view=long&pmid=16096327 |pmc=1798111}}</ref> [[perfloxacin]], [[colchicine]], [[cyclosporine]] or [[thalidomide]]. The hives may respond to treatment with [[PUVA]], and the bone pain may respond to [[bisphosphonates]]. |
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==Prognosis== |
==Prognosis== |
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Revision as of 04:49, 23 March 2018
| Schnitzler syndrome | |
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| Specialty | Immunology  |
Schnitzler syndrome is a rare disease characterised by chronic hives (urticaria) and periodic fever, bone pain and joint pain (sometimes with joint inflammation), weight loss, malaise, fatigue, swollen lymph glands and enlarged spleen and liver.[1]
The urticarial rash is non-itching in more than half of cases, which is unusual for hives. It is most prominent on the trunk, arms and legs, sparing the palms, soles, head and neck. Associated angioedema has been reported in a few patients. A review of 94 cases found a mean age at onset of 51 years, and only four patients developed symptoms before the age of 35.[1] The cause and disease mechanism of Schnitzler syndrome remain largely unknown.[1]
Schnitzler syndrome is considered an autoinflammatory and autoimmune disorder.[2] Chronic hives and a monoclonal gammopathy have been proposed as the major criteria, while the others represent minor criteria.[3]
Classification
Schnitzler syndrome is a late-onset autoinflammatory disorder.[4]
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.Cite error: A <ref> tag is missing the closing </ref> (see the help page).), it is important to rule out other conditions which can cause periodic fevers, paraproteins or chronic hives. These include (and are not limited to) autoimmune or autoinflammatory disorders such as adult-onset Still's disease, angioedema, hematological disorders such as lymphoma or monoclonal gammopathy of undetermined significance (MGUS), other causes of hives, cryoglobulinemia, mastocytosis, chronic neonatal onset multisystem inflammatory disease or Muckle–Wells syndrome.
It is, however, possible to have more than one rare condition, as seen by a patient with both Schnitzler's syndrome and cold-induced urticaria.[5]
A meeting of experts, including Dr Liliane Schnitzler (then retired) took place in Strasbourg in May 2012 and drew up diagnostic criteria, known as the "Strasbourg Criteria". These included two obligate criteria (chronic urticarial rash and monoclonal IgM or IgG) and several minor criteria; a definite diagnosis requires the two obligate criteria, plus two minor criteria if IgM; three if IgG. A probable diagnosis requires the two obligate criteria, plus one (IgM) or two (IgG) minor criteria.[6]
Treatment
As of 2017 no drug was approved to treat Schnitlzers.Cite error: A <ref> tag is missing the closing </ref> (see the help page).[7]
See also
Footnotes
- ^ a b c de Koning HD, Bodar EJ, van der Meer JW, Simon A (December 2007). "Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment". Seminars in arthritis and rheumatism. 37 (3): 137–48. doi:10.1016/j.semarthrit.2007.04.001. PMID 17586002.
- ^ Tinazzi, Elisa; Puccetti, Antonio; Patuzzo, Giuseppe; Sorleto, Michele; Barbieri, Alessandro; Lunardi, Claudio (May 2011). "Schnitzler syndrome, an autoimmune–autoinflammatory syndrome: Report of two new cases and review of the literature". Autoimmunity Reviews. 10 (7): 404–409. doi:10.1016/j.autrev.2011.01.003.
- ^ Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E (January 2001). "The Schnitzler syndrome. Four new cases and review of the literature". Medicine. 80 (1): 37–44. doi:10.1097/00005792-200101000-00004. PMID 11204501.
- ^ Gusdorf, L; Lipsker, D (August 2017). "Schnitzler Syndrome: a Review". Current rheumatology reports. 19 (8): 46. doi:10.1007/s11926-017-0673-5. PMID 28718061.
- ^ Kurian A, Lee JK, Vadas P (2010). "Schnitzler syndrome with cold-induced urticaria". J Dermatol Case Rep. 4: 50–3. doi:10.3315/jdcr.2010.1060. PMC 3157821. PMID 21886751.
{{cite journal}}: CS1 maint: multiple names: authors list (link) - ^ Simon, A.; Asli, B.; Braun-Falco, M.; De Koning, H.; Fermand, J-P.; Grattan, C.; Krause, K.; Lachmann, H.; Lenormand, C.; Martinez-Taboada, V.; Maurer, M.; Peters, M.; Rizzi, R.; Rongioletti, F.; Ruzicka, T.; Schnitzler, L.; Schubert, B.; Sibilia, J; Lipsker, D. (2013). "Schnitzler's syndrome: diagnosis, treatment, and follow-up". Allergy. 68: 562–568. doi:10.1111/all.12129. Retrieved 27 October 2017.
- ^ Cite error: The named reference
281 caseswas invoked but never defined (see the help page).