Schnitzler Syndrome

An extremely rare condition manifested as monoclonal IMMUNOGLOBULIN M dysproteinemia without features of lymphoproliferative disease, but with chronic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, and increased erythrocyte sedimentation rate.

Networked: 62 relevant articles (11 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Urticaria (Hives)
2.	Cryopyrin-Associated Periodic Syndromes
3.	Waldenstrom Macroglobulinemia (Macroglobulinemia)
4.	Fever (Fevers)
5.	Exanthema (Rash)

Experts

1.	Adam, Z: 5 articles (01/2014 - 12/2008)
2.	Simon, Anna: 4 articles (12/2011 - 12/2007)
3.	Hájek, R: 4 articles (01/2011 - 12/2008)
4.	Szturz, P: 3 articles (01/2014 - 01/2011)
5.	Mayer, J: 3 articles (01/2014 - 01/2011)
6.	Lipsker, D: 3 articles (06/2012 - 05/2000)
7.	de Koning, Heleen D: 3 articles (12/2011 - 12/2007)
8.	van der Meer, Jos W M: 3 articles (12/2011 - 12/2007)
9.	Prásek, J: 3 articles (01/2011 - 12/2008)
10.	Neubauer, J: 3 articles (01/2011 - 12/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Schnitzler Syndrome:

1.	Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link 11/01/2009 - "Based on published data, monotherapy with anakinra is currently the most promising treatment for Schnitzler syndrome, because it is able to induce complete remission of symptoms." 11/01/2009 - "We report two cases of nearly complete remission of symptoms in Schnitzler syndrome after the initiation of anakinra therapy, and the first observation of a relapse under continuous daily anakinra therapy. " 12/01/2010 - "[Schnitzler syndrome: A dramatic improvement with anakinra]." 01/01/2009 - "Schnitzler syndrome: a dramatic improvement with anakinra." 01/01/2014 - "In Schnitzler syndrome, anakinra represents an effective, verified and safe medication with potentionally longterm administration not compromising its original efficacy and subjective tolerance. "
2.	Interleukin-1 (Interleukin 1)IBA 01/01/2008 - "IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission." 06/01/2012 - "IL-1 antagonists are a first-line treatment in patients with Schnitzler syndrome and cryopyrinopathies. " 01/01/2015 - "Other agents, such as interleukin-1 inhibitors, have proved beneficial for Schnitzler syndrome and cryopyrin-associated periodic syndromes (CAPS), diseases associated with urticaria. " 08/01/2012 - "Other agents require further evaluation for possible efficacy in the treatment of chronic spontaneous urticaria, including antibody to CD20, a B-lymphocyte cell surface marker, anti-TNFα, and anti-Interleukin 1. Thus far, targeting TNFα has been disappointing for this indication, while targeting Interleukin I has dramatically ameliorated autoinflammatory disorders with urticaria or urticaria-vasculitic-like lesions such as cold-induced autoinflammatory syndrome, Muckle-Wells syndrome, and Schnitzler syndrome." 12/01/2011 - "A diagnostic of Schnitzler syndrome was suggested and treatment with anakinra, an interleukin-1 receptor antagonist, was started, leading to a rapid, complete and sustained remission of symptoms. "
3.	rituximab (Mabthera)FDA Link 05/01/2007 - "Schnitzler syndrome: a case report of successful treatment using the anti-CD20 monoclonal antibody rituximab." 12/01/2009 - "Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy." 08/01/2007 - "Schnitzler syndrome: treatment failure to rituximab but response to anakinra." 01/01/2015 - "Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone."
4.	Ficusin (Psoralen)IBA 11/01/2001 - "Efficacy of Psoralen-UV-A therapy in 3 cases of Schnitzler syndrome."
5.	Dexamethasone (Maxidex)FDA LinkGeneric 01/01/2015 - "Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone."
6.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2015 - "Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone."
7.	canakinumabFDA Link 02/01/2013 - "The present trial was designed to test canakinumab in patients with Schnitzler syndrome. " 09/01/2014 - "Successful treatment of Schnitzler syndrome with canakinumab." 02/01/2013 - "Canakinumab has potential for the treatment of Schnitzler syndrome (ClinicalTrials.gov.number, NCT01245127)." 02/01/2013 - "A patient with Schnitzler syndrome was treated with canakinumab, 150 mg subcutaneously injection every 8 weeks for 6 consecutive months. " 02/01/2013 - "Canakinumab in Schnitzler syndrome."
8.	Interleukin-18 (Interleukin 18)IBA 09/01/2012 - "Elevated interleukin-18 secretion from monoclonal IgM+ B cells in a patient with Schnitzler syndrome." 09/01/2009 - "While no IL-1beta was detected in serum, free circulating IL-18 was increased in patients with Schnitzler syndrome, despite low IL-18 gene expression in monocytes. " 09/01/2009 - "Free circulating interleukin-18 is increased in Schnitzler syndrome: a new autoinflammatory disease?" 09/01/2009 - "To examine the possibility that Schnitzler syndrome may be considered to be an autoinflammatory disease, in this study we measured the serum levels of IL-18, another cytokine of the IL-1 family that is cleaved by caspase-1, in two recently diagnosed Schnitzler patients before and after treatment with IL-1Ra. " 11/01/2009 - "Adult-onset Still's disease (AoSD), Schnitzler syndrome, and cases of adult-onset autoinflammatory syndromes [10-15% of cases of familial Mediterranean fever (FMF) and tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS)] are characterized by a genetic predisposition, with increased interleukin (IL)-1beta and IL-18 production and TNF-alpha signaling, respectively. "
9.	Methotrexate (Mexate)FDA LinkGeneric 08/01/2008 - "Schnitzler syndrome successfully treated with methotrexate." 11/01/2009 - "Recently published studies have shown that patients with Schnitzler syndrome, methotrexate-refractory AoSD, and colchicine-refractory FMF or contraindications to colchicines in FMF respond well to treatment with the soluble IL-1 receptor antagonist anakinra. "
10.	rilonaceptFDA Link 07/01/2012 - "Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study." 06/01/2012 - "A Medline search was performed combining the keywords: "anakinra; canakinumab; rilonacept" AND "skin; neutrophilic dermatoses; Sweet syndrome; pyoderma gangrenosum; hidradenitis suppurativa; Schnitzler syndrome; Still disease". "

Therapies and Procedures

1.	Aftercare (After-Treatment) 06/01/2008 - "Remission of Schnitzler syndrome after treatment with anakinra." 09/01/2009 - "To examine the possibility that Schnitzler syndrome may be considered to be an autoinflammatory disease, in this study we measured the serum levels of IL-18, another cytokine of the IL-1 family that is cleaved by caspase-1, in two recently diagnosed Schnitzler patients before and after treatment with IL-1Ra. "
2.	Radiotherapy 12/01/2009 - "Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy."
3.	Injections 02/01/2013 - "Daily injections of anakinra, an interleukin-1-receptor antagonist, have been reported to control effectively the symptoms and signs of Schnitzler syndrome, a rare acquired autoinflammatory disorder, presenting in adulthood by intermittent fever, urticarial rash, and paraproteinemia, usually IgM. "
4.	Biological Therapy 01/01/2011 - "Concerning the growing numbers of the reports on successful biological therapy with anakinra and our positive experience, we propose that the therapeutic response to anakinra should be included within the diagnostic criteria of Schnitzler syndrome, which is significant above all in differential diagnosis thereof."
5.	Arthroplasty 04/01/2010 - "We suggest prophylaxis against heterotopic ossification when performing hip arthroplasty on patients with Schnitzler syndrome." 04/01/2010 - "We present the case of a patient affected by Schnitzler syndrome who developed Della Valle type C heterotopic ossification after total hip arthroplasty. " 04/01/2010 - "Severe heterotopic ossification after total hip arthroplasty in Schnitzler syndrome."