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: 122 relevant articles (16 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Urticaria (Hives)
2.	Cryopyrin-Associated Periodic Syndromes
3.	Rare Diseases (Rare Disease)
4.	Inflammation (Inflammations)
5.	Waldenstrom Macroglobulinemia (Macroglobulinemia)

Experts

1.	Lipsker, D: 6 articles (08/2017 - 05/2000)
2.	Lipsker, Dan: 5 articles (10/2020 - 12/2010)
3.	Adam, Z: 5 articles (01/2014 - 12/2008)
4.	Krause, Karoline: 4 articles (01/2021 - 04/2017)
5.	de Koning, Heleen D: 4 articles (10/2016 - 12/2007)
6.	Simon, Anna: 4 articles (12/2011 - 12/2007)
7.	Hájek, R: 4 articles (01/2011 - 12/2008)
8.	Maurer, Marcus: 3 articles (01/2021 - 04/2017)
9.	Asli, Bouchra: 3 articles (01/2020 - 08/2007)
10.	Fischer, Jörg: 3 articles (01/2020 - 07/2012)

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." 12/01/2010 - "[Schnitzler syndrome: A dramatic improvement with anakinra]." 01/01/2009 - "Schnitzler syndrome: a dramatic improvement with anakinra." 07/26/2023 - "After the diagnostic criteria for Schnitzler syndrome were met, treatment with anakinra was initiated and resulted in an improvement of the symptoms. " 01/01/2018 - "Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra. "
2.	Interleukin-1 (Interleukin 1)IBA 01/01/2008 - "IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission." 05/20/2022 - "Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases." 05/20/2022 - "Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. " 01/01/2021 - "Can therapeutic response to IL-1 blockade confirm the diagnosis of Schnitzler syndrome?" 01/01/2020 - "The Schnitzler Syndrome (SchS) is an acquired, autoinflammatory condition successfully treated with IL-1 inhibition. "
3.	canakinumabFDA Link 01/01/2020 - "Long-term efficacy of canakinumab in the treatment of Schnitzler syndrome." 01/01/2020 - "We systematically searched PubMed and Embase to include all types of studies on canakinumab treatment in Schnitzler syndrome published until March 16, 2020. " 04/01/2017 - "Efficacy and safety of canakinumab in Schnitzler syndrome: A multicenter randomized placebo-controlled study." 02/01/2013 - "The present trial was designed to test canakinumab in patients with Schnitzler syndrome. " 01/01/2023 - "Here, we present a patient with Schnitzler syndrome treated successfully using canakinumab for over 10 years. "
4.	Interleukin-1 Receptors (Interleukin 1 Receptor)IBA 09/01/2016 - "Skin symptoms include the main features of monogenic inflammasomopathies, such as Cryopyrin-Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF), Schnitzler Syndrome, Hyper-IgD Syndrome (HIDS), PAPA Syndrome, and Deficiency of IL-1 Receptor Antagonist (DIRA). " 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. " 11/01/2009 - "Complete remission of symptoms has been reported recently in patients with Schnitzler syndrome treated with anakinra, an interleukin-1 receptor antagonist. " 10/01/2016 - "Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome." 05/01/2011 - "We describe here two patients affected by Schnitzler syndrome, both refractory to corticosteroids and immunosuppressive therapy, successfully treated with the interleukin-1 receptor antagonist Anakinra. "
5.	tocilizumab (atlizumab)FDA Link 12/01/2020 - "A Chinese case series of Schnitzler syndrome and complete remission in one tocilizumab-treated patient." 01/01/2021 - "Tocilizumab treatment in patients with Schnitzler syndrome: An open-label study." 01/01/2019 - "Variable Responses to Tocilizumab in Four Patients with Schnitzler Syndrome." 04/01/2009 - "Tocilizumab as a potential treatment in Schnitzler syndrome." 11/01/2021 - "Although approved treatment options for Schnitzler syndrome are lacking, our patients were treated with IL-1-targeted therapy (anakinra or canakinumab) or anti-IL-6 (tocilizumab). "
6.	Rituximab (Mabthera)FDA Link 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." 05/01/2007 - "Schnitzler syndrome: a case report of successful treatment using the anti-CD20 monoclonal antibody rituximab." 01/01/2015 - "Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone."
7.	ibrutinibIBA 10/01/2021 - "Killing Two Birds with One Stone: the Therapeutic Role of Ibrutinib in Schnitzler Syndrome." 01/01/2018 - "Ibrutinib for the Management of Schnitzler Syndrome: A Novel Therapy for a Rare Condition." 01/01/2021 - "Dramatic Efficacy of Ibrutinib in a Schnitzler Syndrome Case with Indolent Lymphoma." 01/01/2022 - "Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome." 01/01/2022 - "She fulfilled the diagnosis of Schnitzler syndrome and was treated with the Bruton tyrosine kinase inhibitor ibrutinib in combination with prednisone. "
8.	Ficusin (Psoralen)IBA 11/01/2001 - "Efficacy of Psoralen-UV-A therapy in 3 cases of Schnitzler syndrome."
9.	Dexamethasone (Maxidex)FDA LinkGeneric 01/01/2015 - "Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone."
10.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2015 - "Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone."

Therapies and Procedures

1.	Therapeutics 10/01/2016 - "Patients with Schnitzler syndrome were analyzed for changes in TH17 cell functions before and during therapy with IL-1β-blocking drugs. " 06/01/2016 - "We report a 50-year-old woman with Schnitzler syndrome for 10 years, with major impact on her quality of life and refractory to conventional therapies. " 07/26/2023 - "Therapy resistant urticaria as a long-term symptom of an incomplete Schnitzler syndrome." 01/01/2018 - "Schnitzler syndrome co-occurring with idiopathic multicentric Castleman disease that responds to anti-IL-1 therapy: A case report and clue to pathophysiology." 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. "
2.	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. "
3.	Contraindications 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. "
4.	Radiotherapy 11/01/2017 - "Radiotherapy for a breast cancer patient with Schnitzler syndrome: Report of acute toxicity and early follow-up." 12/01/2009 - "Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy."
5.	Hip Replacement Arthroplasty (Total Hip Replacement) 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."