schnitzler syndrome

Summary

Summary: 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.

Top Publications

  1. Crouch R, Akhras V, Sarkany R. Schnitzler's syndrome: successful treatment with anakinra. Australas J Dermatol. 2007;48:178-81 pubmed
    ..The use of anakinra in our patient resulted in resolution of symptoms and has enabled cessation of oral prednisone. Our patient remains symptom free on anakinra after 14 months of follow up...
  2. Tanneberger O, Buchner S, Zimmerli L. [Schnitzler's syndrome with urticaria vasculitis]. Internist (Berl). 2007;48:1432-5 pubmed
    ..Up to now, treatment was often difficult and disappointing. Interleukin-1 receptor antagonists offer a new therapeutic option...
  3. Soubrier M. Schnitzler syndrome. Joint Bone Spine. 2008;75:263-6 pubmed publisher
    b>Schnitzler syndrome is characterized by monoclonal IgM gammopathy, urticaria, recurrent fever, evidence of inflammation, bone pain, and arthralgia, occasionally in combination with lymphadenopathy and/or hepatosplenomegaly...
  4. Migliorini P, Del Corso I, Tommasi C, Boraschi D. Free circulating interleukin-18 is increased in Schnitzler syndrome: a new autoinflammatory disease?. Eur Cytokine Netw. 2009;20:108-11 pubmed publisher
    b>Schnitzler syndrome is a rare disease characterised by chronic urticaria and arthralgia...
  5. Besada E, Nossent H. Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler's syndrome: a case report and literature review. Clin Rheumatol. 2010;29:567-71 pubmed publisher
    ..The current case history and the literature review already suggest an important role for IL-1 as a mediator in the pathophysiology of Schnitzler's syndrome...
  6. Murota H, Shoda Y, Ishibashi T, Sugahara H, Matsumura I, Katayama I. Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy. J Am Acad Dermatol. 2009;61:1070-5 pubmed publisher
    b>Schnitzler syndrome is a rare condition defined by chronic urticaria, osteosclerotic bone lesions, and monoclonal IgM gammopathy...
  7. Kieffer C, Cribier B, Lipsker D. Neutrophilic urticarial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature. Medicine (Baltimore). 2009;88:23-31 pubmed publisher
    ..systemic diseases: adult-onset Still disease (3 patients), systemic lupus erythematosus (3 patients), and Schnitzler syndrome (1 patient)...
  8. Pizzirani C, Falzoni S, Govoni M, La Corte R, Donadei S, Di Virgilio F, et al. Dysfunctional inflammasome in Schnitzler's syndrome. Rheumatology (Oxford). 2009;48:1304-8 pubmed publisher
    ..IL-1beta plays a key role in the pathogenesis of Schnitzler's syndrome (SS). We have investigated inflammasome activity in peripheral blood mononuclear cells (PBMCs) from a patient affected by a variant type of SS...
  9. Lipsker D, Spehner D, Drillien R, Schmitt P, Cribier B, Heid E, et al. Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions. Br J Dermatol. 2000;142:954-9 pubmed
    The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy...

More Information

Publications62

  1. Gilson M, Abad S, Larroche C, Dhote R. Treatment of Schnitzler's syndrome with anakinra. Clin Exp Rheumatol. 2007;25:931 pubmed
  2. Eiling E, Möller M, Kreiselmaier I, Brasch J, Schwarz T. Schnitzler syndrome: treatment failure to rituximab but response to anakinra. J Am Acad Dermatol. 2007;57:361-4 pubmed
    b>Schnitzler syndrome is a rare disease characterized by a chronic urticarial rash and a monoclonal gammopathy. The exact pathogenesis is still uncertain and treatment remains a challenge...
  3. Tomková H, Shirafuji Y, Arata J. Schnitzler's syndrome versus adult onset Still's disease. Eur J Dermatol. 1998;8:118-21 pubmed
  4. Ryan J, de Koning H, Beck L, Booty M, Kastner D, Simon A. IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission. J Allergy Clin Immunol. 2008;121:260-2 pubmed
  5. Westhoff T, Zidek W, Uharek L, Steinhoff Georgieva J, van der Giet M. Impairment of renal function in Schnitzler's syndrome. J Nephrol. 2006;19:660-3 pubmed
    ..Immunofixation of immunoglobulins constitutes the crucial diagnostic step, since monoclonal gammopathy is a constant clinical sign in this entity...
  6. Dalle S, Balme B, Sebban C, Pariset C, Berger F, Thomas L. Schnitzler syndrome associated with systemic marginal zone B-cell lymphoma. Br J Dermatol. 2006;155:827-9 pubmed
    b>Schnitzler syndrome is a rare condition defined by chronic urticaria and monoclonal IgM gammopathy. Malignant evolution to Waldenström disease (WD) has been reported in several cases...
  7. de Koning H, Bodar E, Simon A, van der Hilst J, Netea M, van der Meer J. Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. Ann Rheum Dis. 2006;65:542-4 pubmed
    ..Schnitzler's syndrome is an inflammatory disorder characterised by chronic urticarial rash and monoclonal gammopathy, accompanied by periodic fever, arthralgia or arthritis, and bone pain. The cause and treatment are still unknown...
  8. Lecompte M, Blais G, Bisson G, Maynard B. Schnitzler's syndrome. Skeletal Radiol. 1998;27:294-6 pubmed
    ..We report on a case of Schnitzler's syndrome, focusing on pattern of bone involvement and its differential diagnosis...
  9. Martinez Taboada V, Fontalba A, Blanco R, Fernandez Luna J. Successful treatment of refractory Schnitzler syndrome with anakinra: comment on the article by Hawkins et al. Arthritis Rheum. 2005;52:2226-7 pubmed
  10. Welsh B, Tate B. Schnitzler's syndrome: report of a case with progression to Waldenström's macroglobulinaemia. Australas J Dermatol. 1999;40:201-3 pubmed
    ..To date, despite six cycles of chemotherapy with oral chlorambucil, his disease remains persistent but stable...
  11. Germain P, Fach J, Bui N, Traissac T, Delbrel X, Le Bras M, et al. [Schnitzler syndrome: a rare cause of systemic urticaria]. Rev Med Interne. 2000;21:285-9 pubmed
    ..The Schnitzler's syndrome first described in 1972, associates urticaria, bone pain, and monoclonal IgM gammapathy...
  12. Kastritis E, Katoulis A, Terpos E, Panayiotides I, Gavriatopoulopu M, Dimopopoulos M. Schnitzler's syndrome: increased levels of bone formation and angiogenesis factors are reduced after successful pefloxacin treatment. Clin Lymphoma Myeloma. 2008;8:359-62 pubmed publisher
    ..Furthermore, pefloxacin was proven to be an effective treatment for patients with Schnitzler's syndrome...
  13. Sanchez G, Ano M, García Avilés C, Diéguez I. Schnitzler syndrome: a case study. J Investig Allergol Clin Immunol. 2000;10:41-3 pubmed
    b>Schnitzler syndrome is a clinical entity of unknown etiopathogenesis and is an unusual cause of chronic urticaria. It is characterized by the presentation of a monoclonal band, especially IgM, as well as fever, asthenia and arthralgia...
  14. Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E. The Schnitzler syndrome. Four new cases and review of the literature. Medicine (Baltimore). 2001;80:37-44 pubmed
    The Schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy...
  15. de Koning H, Bodar E, Van der Meer J, Simon A. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum. 2007;37:137-48 pubmed
    b>Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy, accompanied by intermittent fever, arthralgia or arthritis, bone pain, and lymphadenopathy...
  16. Schneider S, Gaubitz M, Luger T, Bonsmann G. Prompt response of refractory Schnitzler syndrome to treatment with anakinra. J Am Acad Dermatol. 2007;56:S120-2 pubmed
  17. Schuster C, Kranke B, Aberer E, Arbab E, Sturm G, Aberer W. Schnitzler syndrome: response to anakinra in two cases and a review of the literature. Int J Dermatol. 2009;48:1190-4 pubmed publisher
    b>Schnitzler syndrome is a rare disease characterized by a chronic urticarial eruption and monoclonal gammopathy, as well as clinical and laboratory signs of inflammation...
  18. Lipsker D. The Schnitzler syndrome. Orphanet J Rare Dis. 2010;5:38 pubmed publisher
    The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease...
  19. Lauwers A, Chouvy V, Mosnier J, Misery L, Alexandre C. A case of Schnitzler's syndrome with nodular regenerative hyperplasia of the liver. Rev Rhum Engl Ed. 1999;66:281-3 pubmed
    ..The pathogenesis of Schnitzler's syndrome remains unknown, but the possibility of progression to a hematological malignancy requires prolonged follow-up...
  20. Adam Z, Krejci M, Pour L, Neubauer J, Prasek J, Hajek R. [Schnitzler syndrome--report on a fourteen-year course of the disease and an overview of information on the disease]. Vnitr Lek. 2008;54:1140-53 pubmed
    b>Schnitzler syndrome is a rare disease characterised by chronic urticaria and the presence of monoclonal IgM immunoglobulin, and by other symptoms. We report ourexperience with 14-year treatment of a patient...
  21. Famularo G, Barracchini A, Minisola G. Severe thrombophilia with antiphospholipid syndrome and hyperhomocysteinemia in a patient with Schnitzler's syndrome. Clin Exp Rheumatol. 2003;21:366-8 pubmed
    ..The systemic clotting disorder, the association with the antiphospholipid syndrome and hyperhomocysteinemia, and the biclonal rather than monoclonal IgM gammopathy were striking features of this atypical case of Schnitzler's syndrome...
  22. Frischmeyer Guerrerio P, Rachamalla R, Saini S. Remission of Schnitzler syndrome after treatment with anakinra. Ann Allergy Asthma Immunol. 2008;100:617-9 pubmed publisher
  23. Flórez A, Gallardo Agromayor E, García Barredo R, Landeras Alvaro R, Gorriño Angulo M, Martinez Taboada V, et al. Radiological aid to clinical diagnosis of Schnitzler's syndrome: multimodality imaging approach. Clin Rheumatol. 2008;27:107-10 pubmed
    ..On the other hand, although our patient had an excellent clinical response to anakinra, the sclerotic lesion remained unchanged on follow-up X-ray examinations...
  24. Asli B, Bienvenu B, Cordoliani F, Brouet J, Uzunhan Y, Arnulf B, et al. Chronic urticaria and monoclonal IgM gammopathy (Schnitzler syndrome): report of 11 cases treated with pefloxacin. Arch Dermatol. 2007;143:1046-50 pubmed
    b>Schnitzler syndrome is characterized by chronic urticarial rash and monoclonal IgM gammopathy and is sometimes associated with periodic fever, arthralgias, and bone pain. Current treatment is unsatisfactory.
  25. Claes K, Bammens B, Delforge M, Evenepoel P, Kuypers D, Vanrenterghem Y. Another devastating complication of the Schnitzler syndrome: AA amyloidosis. Br J Dermatol. 2008;158:182-4 pubmed
  26. Carbone J, Paravisini A, Sarmiento E, Rodriguez Molina J, Fernandez Cruz E. Partial response to cyclosporine in a patient with Schnitzler's syndrome. Allergol Immunopathol (Madr). 2007;35:71-3 pubmed
    ..Partial but maintained remission of the urticaria was also observed, allowing corticosteroid doses to be decreased...
  27. Larocca C, McEvoy J, Ellis C, Junkins Hopkins J, Kolb T, Baer A, et al. Schnitzler's syndrome associated with pancreatitis: a disease of IL-1 dysregulation. Clin Rheumatol. 2012;31:169-74 pubmed publisher
    ..She also had a history of chronic pancreatitis as well as a family history of recurrent pancreatitis. The diagnosis of Schnitzler's syndrome was made, and she was successfully treated with the IL-1 receptor antagonist, anakinra...
  28. Lewicki M, Lewicka K, Kotyla P, Kotulska A, Goździk J, Błach A, et al. [Schnitzler's syndrome]. Pol Arch Med Wewn. 2004;111:735-41 pubmed
  29. Henry B, Neel A, Barbarot S, Masseau A, Hamidou M. [Schnitzler's syndrome]. Rev Med Interne. 2013;34:224-9 pubmed publisher
    b>Schnitzler syndrome (SS) is a rare clinical entity, which belongs to the spectrum of monoclonal gammapathy-associated systemic disorders...
  30. de Koning H, van der Meer J, Simon A. Comment on: Schnitzlers syndrome--exacerbation after anti-TNF treatment. Rheumatology (Oxford). 2007;46:1741 author reply 1741 pubmed
  31. Pascual Lopez M, Hernández Núñez A, Sanchez Perez J, Fernandez Herrera J, Garcia Diez A. Schnitzler's syndrome with monoclonal IgG kappa gammopathy: good response to cyclosporin. J Eur Acad Dermatol Venereol. 2002;16:267-70 pubmed
    ..Two previous similar cases with clinical features of SS and monoclonal IgG immunoglobulin have been described. We suggest our case also represents a variant of SS with IgG gammopathy...
  32. Cosserat J, Bouffaut P, Quint L, Gayraud M. [His name could have been Arthur...]. Rev Med Interne. 2004;25 Suppl 2:S265-6 pubmed
  33. Worm M, Kolde G. Schnitzler's syndrome: successful treatment of two patients using thalidomide. Br J Dermatol. 2003;148:601-2 pubmed
  34. Eiling E, Schröder J, Gross W, Kreiselmaier I, Mrowietz U, Schwarz T. The Schnitzler syndrome: chronic urticaria and monoclonal gammopathy--an autoinflammatory syndrome?. J Dtsch Dermatol Ges. 2008;6:626-31 pubmed publisher
    b>Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, fever of uncertain origin, hepato- or splenomegaly, ..
  35. de Koning H, Schalkwijk J, van der Ven Jongekrijg J, Stoffels M, Van der Meer J, Simon A. Sustained efficacy of the monoclonal anti-interleukin-1 beta antibody canakinumab in a 9-month trial in Schnitzler's syndrome. Ann Rheum Dis. 2013;72:1634-8 pubmed publisher
    ..Canakinumab is a selective human monoclonal anti-IL-1? antibody with a long half-life. We investigated the long-term efficacy and safety of canakinumab in Schnitzler's syndrome...
  36. Terpos E, Asli B, Christoulas D, Brouet J, Kastritis E, Rybojad M, et al. Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: new insight into the biology of Schnitzler syndrome. Haematologica. 2012;97:1699-703 pubmed publisher
    b>Schnitzler syndrome is a rare plasma cell disorder the pathogenesis of which is still not fully understood...
  37. Tinazzi E, Puccetti A, Patuzzo G, Sorleto M, Barbieri A, Lunardi C. Schnitzler syndrome, an autoimmune-autoinflammatory syndrome: report of two new cases and review of the literature. Autoimmun Rev. 2011;10:404-9 pubmed publisher
    b>Schnitzler syndrome is a rare disorder characterized clinically by chronic urticarial rash accompanied by fever, arthralgia or arthritis, bone pain and lymphoadenopathy and biochemically by monoclonal gammopathy and elevation of ..
  38. de Koning H, Schalkwijk J, Van der Meer J, Simon A. Successful canakinumab treatment identifies IL-1? as a pivotal mediator in Schnitzler syndrome. J Allergy Clin Immunol. 2011;128:1352-4 pubmed publisher
  39. Maurer M, Magerl M, Metz M, Siebenhaar F, Weller K, Krause K. Practical algorithm for diagnosing patients with recurrent wheals or angioedema. Allergy. 2013;68:816-9 pubmed publisher
    ..AIM AND RESULT: In order to not miss these conditions, we have developed a symptom-based diagnostic algorithm for the management of patients with wheals and/or angioedema...
  40. Iwafuchi Y, Morita T, Hata K, Nakamura A, Miyazaki S. Schnitzler syndrome complicated by membranous nephropathy. Clin Nephrol. 2012;78:497-500 pubmed
    b>Schnitzler syndrome is a rare clinical entity characterized by the association of chronic urticarial rash and monoclonal immunoglobulin M gammopathy. A 62-year old male developed nephrotic syndrome with Schnitzler syndrome...
  41. Carlesimo M, La Pietra M, Arcese A, Orsini D, Marmo G, Fidanza L, et al. Chronic urticaria and IgG paraproteinaemia: unusual spectrum of Schnitzler Syndrome. Clin Ter. 2011;162:e85-7 pubmed
    b>Schnitzler Syndrome (SS) is a rare clinical entity of unknown etio-pathogenesis characterizated by non itching chronic urticaria, associated with an IgM monoclonal paraprotein; other symptoms as bone pain, poliarthralgia, elevated ..
  42. Lipsker D. The Schnitzler syndrome - a treatment at last?. Dermatology. 2002;205:1-2 pubmed
  43. Szturz P, Adam Z, Klabusay M, Fojtik Z, Kadanka Z, Stehlíková O, et al. [Schnitzler syndrome: case report, the experience with glucocorticoid and anakinra (Kineret) therapies and monitoring of systemic cytokine response]. Vnitr Lek. 2011;57:97-112 pubmed
    b>Schnitzler syndrome is a rare idiopathic disease characterized by chronic urtica, presence of monoclonal IgM immunoglobuline and further, less common symptoms...
  44. Singh B, Ezziddin S, Rabe E, Biersack H, Ahmadzadehfar H, Ahmadzadefar H. Bone scan appearance supportive of Schnitzler's syndrome: report of two new cases. Clin Nucl Med. 2006;31:151-3 pubmed
  45. Bodar E, Simon A, Van der Meer J. Effects of the histone deacetylase inhibitor ITF2357 in autoinflammatory syndromes. Mol Med. 2011;17:363-8 pubmed publisher
    ..syndrome (TRAPS), three patients with hyper-IgD and periodic fever syndrome (HIDS) and four patients with Schnitzler syndrome were closely followed during 90 d of ITF2357 treatment...
  46. Gran J, Midtvedt Ø, Haug S. [A woman with recurrent urticaria, joint pain and fever]. Tidsskr Nor Laegeforen. 2011;131:135-6 pubmed publisher
    ..After 13 years of skin disease, she developed monoclonal IgM gammopathy, myalgia and joint pain. She was diagnosed with Schnitzler's syndrome and successfully treated with the IL-1 receptor antagonist anakinra...
  47. Vanderschueren S, Knockaert D. Canakinumab in Schnitzler syndrome. Semin Arthritis Rheum. 2013;42:413-6 pubmed publisher
    ..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, ..
  48. Sediva A, Polouckova A, Podrazil M, Fronkova E, Kalina T. Characterization of the B-cell compartment in a patient with Schnitzler syndrome. Scand J Rheumatol. 2011;40:158-60 pubmed publisher
  49. Oborilová A, Adam Z. [Schnitzler's syndrome]. Vnitr Lek. 1998;44:423-7 pubmed
    ..The combination of these symptoms corresponds with the so-called Schnitzler syndrome which is analyzed in detail in the discussion...
  50. Blaise S, Vallat J, Tabaraud F, Bonnetblanc J. [Sensitive chronic inflammatory demyelinating polyradiculoneuropathy in Schnitzler's syndrome]. Ann Dermatol Venereol. 2003;130:348-51 pubmed
    ..More than fifty patients with this syndrome have been reported since Schnitzler reported the first case in 1974, but neuropathies are seen only in a few cases...
  51. Jain T, Offord C, Kyle R, Dingli D. Schnitzler syndrome: an under-diagnosed clinical entity. Haematologica. 2013;98:1581-5 pubmed publisher
    b>Schnitzler syndrome is considered to be a rare disorder characterized by a monoclonal IgM protein and chronic urticaria that is associated with considerable morbidity...
  52. Kuenzli S, Buchet S, Saurat J. Successful treatment of Schnitzler's syndrome with interferon alfa-2b. Dermatology. 2002;205:74 pubmed
  53. Yu J, Leslie K. Cryopyrin-associated periodic syndrome: an update on diagnosis and treatment response. Curr Allergy Asthma Rep. 2011;11:12-20 pubmed publisher
    ..It also has become clear that the NLRP3 inflammasome plays a critical role in innate immune defense and therefore has wider implications for other inflammatory disease states...