nach oben

Current Rheumatology Reports

Erschienen in:

01.08.2017 | Orphan Diseases (B Manger, Section Editor)

Schnitzler Syndrome: a Review

verfasst von: L. Gusdorf, D. Lipsker

Erschienen in: Current Rheumatology Reports | Ausgabe 8/2017

Einloggen, um Zugang zu erhalten

Abstract

Purpose of Review

We focus on recent advances in diagnosis and therapeutic strategies, as well as on pathogenesis of Schnitzler syndrome.

Recent Findings

New diagnostic criteria were established, and their external validity was assessed in a retrospective cohort study. The cytokine interleukin-1 (IL-1) plays a crucial role in the pathogenesis of the Schnitzler syndrome, and this explains the spectacular efficiency of IL-1 blocking therapies.

Summary

The Schnitzler syndrome is now considered as a late-onset acquired autoinflammatory syndrome in which the cytokine IL-1 plays a crucial role. IL-1 blocking therapies are efficient on the inflammation-linked symptoms but not on the monoclonal component. Therefore, they probably don’t reduce the risk of the development of lymphoproliferative disorders that remains the main prognostic issue. The link between autoinflammation and the monoclonal component needs to be further elucidated.

Schnitzler L. Lésions urticariennes chroniques permanentes (érythème pétaloïde?). Journées Dermatologiques d’Angers. 1972; Cas Clinique. N°46b.

•• Lipsker D. Monoclonal gammopathy of cutaneous significance: review of a relevant concept. J Eur Acad Dermatol Venereol. 2017;31:45–52. This article introduces the concept of monoclonal gammopathy of cutaneous significance and reviews the main entities that fit into it. CrossRefPubMed

De Koning HD, van Gijn ME, Stoffels M, Jongekrijg J, Zeeuwen PLJM, Elferink MG, et al. Myeloid lineage-restricted somatic mosaicism of NLRP3 mutations in patients with variant Schnitzler syndrome. J Allergy Clin Immunol. 2015;135:561–4.CrossRefPubMed

Ryan JG, de Koning HD, Beck LA, Booty MG, Kastner DL, Simon A. IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission. J Allergy Clin Immunol. 2008;121:260–2.CrossRefPubMed

De Koning HD, Schalkwijk J, Stoffels M, Jongekrijg J, Jacobs JFM, Verwiel E, et al. The role of interleukin-1 beta in the pathophysiology of Schnitzler’s syndrome. Arthritis Res Ther. 2015;17:187.CrossRefPubMedPubMedCentral

De Koning HD, van Vlijmen-Willems IMJJ, Rodijk-Olthuis D, van der Meer JWM, Zeeuwen PLJM, Simon A, et al. Mast-cell interleukin-1beta, neutrophil interleukin-17 and epidermal antimicrobial proteins in the neutrophilic urticarial dermatosis in Schnitzler’s syndrome. Br J Dermatol. 2015;173:448–56.CrossRefPubMed

Zielinski CE, Mele F, Aschenbrenner D, Jarrossay D, Ronchi F, Gattorno M, et al. Pathogen-induced human TH17 cells produce IFN-gamma or IL-10 and are regulated by. Nature. 2012;484:514–8.CrossRefPubMed

Noster R, de Koning HD, Maier E, Prelog M, Lainka E, Zielinski CE. Dysregulation of proinflammatory versus anti-inflammatory human TH17 cell functionalities in the autoinflammatory Schnitzler syndrome. J Allergy Clin Immunol. 2016;138:1161–9.CrossRefPubMed

Mulla E, Neame R. Delayed development of the IgM paraprotein in Schnitzler’s syndrome. Scand J Rheumatol. 2015;44:521–2.CrossRefPubMed

10.

Treon SP, Xu L, Yang G, Zhou Y, Liu X, Cao Y, et al. MYD88 L265P somatic mutation in Waldenstrom’s macroglobulinemia. N Engl J Med. 2012;367:826–33.CrossRefPubMed

11.

• De Koning HD. Schnitzler’s syndrome: lessons from 281 cases. Clin Transl Allergy. 2014;4:41. This study summarizes the clinical, biological, histological, and morphological data of all the patients with a Schnitzler syndrome reported to date. CrossRefPubMedPubMedCentral

12.

Akimoto R, Yoshida M, Matsuda R, Miyasaka K, Itoh M. Schnitzler’s syndrome with IgG kappa gammopathy. J Dermatol. 2002;29:735–8.CrossRefPubMed

13.

Welsh B, Tate B. Schnitzler’s syndrome: report of a case with progression to Waldenstrom’s macroglobulinaemia. Australas J Dermatol. 1999;40:201–3.CrossRefPubMed

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.CrossRef

15.

Simon A, Asli B, Braun-Falco M, De Koning H, Fermand J-P, Grattan C, et al. Schnitzler’s syndrome: diagnosis, treatment, and follow-up. Allergy. 2013;68:562–8.CrossRefPubMed

16.

•• Gusdorf L, Asli B, Barbarot S, Neel A, Masseau A, Puechal X, et al. Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients. Allergy. 2017;72:177–82. This study confirms the applicability and the validity of the existing diagnostic criteria in real-life patients. CrossRefPubMed

17.

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.CrossRef

18.

Broekaert SMC, Boer-Auer A, Kerl K, Herrgott I, Schulz X, Bonsmann G, et al. Neutrophilic epitheliotropism is a histopathological clue to neutrophilic urticarial dermatosis. Am J Dermatopathol. 2016;38:39–49.CrossRefPubMed

19.

Gusdorf L, Bessis D, Lipsker D. Lupus erythematosus and neutrophilic urticarial dermatosis: a retrospective study of 7 patients. Medicine (Baltimore). 2014;93:e351.CrossRef

20.

Lipsker D. The Schnitzler syndrome. Orphanet J Rare Dis. 2010;5:38.CrossRefPubMedPubMedCentral

21.

Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome: the Mayo Clinic experience (1972-2011). J Am Acad Dermatol. 2012;67:1289–95.CrossRefPubMed

22.

Carlesimo M, Abruzzese C, Narcisi A, La Verde G, De Marco G, Verga E, et al. Chronic vasculitis urticaria associated to a monoclonal gammopathy of IgM and IgA type, a Schnitzler syndrome? Eur J Dermatol. 2010;20:838–9.PubMed

23.

Ricci M, Bettazzi LG, Varenna M, Marchesoni A. Atypical bone sclerosis. Arthritis Rheumatol Hoboken NJ. 2016;68:816.CrossRef

24.

Niederhauser BD, Dingli D, Kyle RA, Ringler MD. Imaging findings in 22 cases of Schnitzler syndrome: characteristic para-articular osteosclerosis, and the “hot knees” sign differential diagnosis. Skelet Radiol. 2014;43:905–15.CrossRef

25.

Willekens I, Walgraeve N, Goethals L, De Geeter F. Correlative bone imaging in a case of Schnitzler’s syndrome and brief review of the literature. Hell J Nucl Med. 2015;18:71–3.PubMed

26.

Ferrando FJ, Pujol J, Hortells JL, Navarro M, Pinol J, Carapeto FJ. Schnitzler’s syndrome: report of a case with bone osteolysis. J Investig Allergol Clin Immunol. 1994;4:203–5.PubMed

27.

Terpos E, Asli B, Christoulas D, Brouet J-C, 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.CrossRefPubMedPubMedCentral

28.

Martinez-Taboada VM, Fontalba A, Blanco R, Fernandez-Luna JL. Successful treatment of refractory Schnitzler syndrome with anakinra: comment on the article by Hawkins et al. Arthritis Rheum. 2005;52:2226–7.CrossRefPubMed

29.

Lipsker D, Lenormand C. Management of Schnitzler’s syndrome. Expert Opin Orphan Drugs. 2014;2:947–55.CrossRef

30.

Neel A, Henry B, Barbarot S, Masseau A, Perrin F, Bernier C, et al. Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler’s syndrome: a French multicenter study. Autoimmun Rev. 2014;13:1035–41.CrossRefPubMed

31.

Perrin F, Neel A, Graveleau J, Ruellan A-L, Masseau A, Hamidou M. Two cases of anakinra-induced neutropenia during auto-inflammatory diseases: drug reintroduction can be successful. Presse Med. 2014;43:319–21.CrossRefPubMed

32.

De Koning HD, Schalkwijk J, van der Ven-Jongekrijg J, Stoffels M, van der Meer JWM, 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.CrossRefPubMed

33.

Vanderschueren S, Knockaert D. Canakinumab in Schnitzler syndrome. Semin Arthritis Rheum. 2013;42:413–6.CrossRefPubMed

34.

Pesek R, Fox R. Successful treatment of Schnitzler syndrome with canakinumab. Cutis. 2014;94:E11–2.PubMed

35.

Krause K, Tsianakas A, Wagner N, Fischer J, Weller K, Metz M, et al. Efficacy and safety of canakinumab in Schnitzler syndrome: a multicenter randomized placebo-controlled study. J Allergy Clin Immunol. 2017;139:1311–20.CrossRefPubMed

36.

Krause K, Weller K, Stefaniak R, Wittkowski H, Altrichter S, Siebenhaar F, et al. Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study. Allergy. 2012;67:943–50.CrossRefPubMed

37.

Krause K, Feist E, Fiene M, Kallinich T, Maurer M. Complete remission in 3 of 3 anti-IL-6-treated patients with Schnitzler syndrome. J Allergy Clin Immunol. 2012;129:848–50.CrossRefPubMed

38.

Asli B, Brouet JC, Fermand JP. Spontaneous remission of Schnitzler syndrome. Ann Allergy Asthma Immunol Off Publ Am Coll Allergy Asthma Immunol. 2011;107:87–8.CrossRef

39.

Conlon NP, Hayden P, Barnes L, Doran M, O’Shea F, Feighery C. Schnitzler’s syndrome: a case highlighting the complications of long-standing acquired autoinflammation. Eur J Dermatol. 2014;24:405–6.PubMed

40.

Gameiro A, Gouveia M, Pereira M, Tellechea O, Goncalo M. Clinical characterization and long-term follow-up of Schnitzler syndrome. Clin Exp Dermatol. 2016;41:461–7.CrossRefPubMed

41.

Margerin F, Gottenberg JE, Lipsker D. Occurrence of rheumatoid arthritis in a patient treated with anakinra for Schnitzler syndrome: a case report. J Rheumatol. 2016;43:1447.CrossRefPubMed

Titel: Schnitzler Syndrome: a Review
verfasst von: L. Gusdorf
D. Lipsker
Publikationsdatum: 01.08.2017
Verlag: Springer US
Erschienen in: Current Rheumatology Reports / Ausgabe 8/2017
Print ISSN: 1523-3774
Elektronische ISSN: 1534-6307
DOI: https://doi.org/10.1007/s11926-017-0673-5

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Innere Medizin

24.04.2024 | DGIM 2024 | Kongressbericht | Nachrichten

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Schnitzler Syndrome: a Review

Abstract

Purpose of Review

Recent Findings

Summary

Leitlinien kompakt für die Innere Medizin

Neu im Fachgebiet Innere Medizin

Metformin rückt in den Hintergrund

Myokarditis nach Infekt – richtig schwierig wird es bei Profisportlern

Thrombophiliescreening – Wenn, dann richtig und nur bei therapeutischer Konsequenz

Bei Senioren mit Prostatakarzinom auf Anämie achten!

Update Innere Medizin

Springer Medizin

Abstract

Purpose of Review

Recent Findings

Summary

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 8/2017

Contribution of Circulatory Disturbances in Subchondral Bone to the Pathophysiology of Osteoarthritis

Direct Oral Anticoagulants Use in Antiphospholipid Syndrome: Are These Drugs an Effective and Safe Alternative to Warfarin? A Systematic Review of the Literature: Comment

New Insights on Platelets and Platelet-Derived Microparticles in Systemic Lupus Erythematosus

How to Identify High-Risk APS Patients: Clinical Utility and Predictive Values of Validated Scores

Recent Advances in Tissue Engineering Strategies for the Treatment of Joint Damage

Innate Immune Responses and Osteoarthritis

Leitlinien kompakt für die Innere Medizin

Neu im Fachgebiet Innere Medizin

Metformin rückt in den Hintergrund

Myokarditis nach Infekt – richtig schwierig wird es bei Profisportlern

Thrombophiliescreening – Wenn, dann richtig und nur bei therapeutischer Konsequenz

Bei Senioren mit Prostatakarzinom auf Anämie achten!

Update Innere Medizin