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Digitale Ulzerationen bei systemischer Sklerodermie

Digital ulcers in systemic scleroderma

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Zusammenfassung

Digitale Ulzerationen (DUs) sind eines der Hauptsymptome der systemischen Sklerodermie und treten bei ungefähr 60 % der Patienten auf. Sie erfordern aufgrund möglicher Komplikationen wie Infektionen, trockene/feuchte Gangrän oder Amputation regelmäßige Kontrollen und eine angepasste Wundbehandlung durch Arzt und Pflegepersonal. Die „European League against Rheumatism“ (EULAR) hat im Jahr 2009 Leitlinien zur Therapie der digitalen Ulzeration veröffentlicht. Dabei wurde eine Verbesserung der Heilung unter Iloprost beschrieben. Unter Bosentan wurde die Häufigkeit des Auftretens neuer DUs signifikant reduziert. In einigen kleinen Studien konnte ebenfalls eine Verbesserung der Heilung für Phosphodiesterase-Typ-5 (PDE-5)-Inhibitoren gezeigt werden. Weitere Studien zu anderen Therapieansätzen werden in den nächsten Jahren folgen.

Abstract

Digital ulcers (DU’s) are one of the main symptoms of systemic scleroderma and occur in approximately 60 % of all scleroderma patients. Due to possible complications such as infections, gangrene or amputation, they require regular medical attention and a good wound treatment by doctors and nursing staff. A definition of DU’s has not yet been established. In 2009 the European League Against Rheumatism (EULAR) published guidelines for the treatment of DU’s. An improvement of the healing of active ulcers has been described with Iloprost. Bosentan significantly reduced the frequency of occurrence of new DU’s. In some small studies PDE-5 inhibitors appear helpful. Further studies with other therapeutic approaches will follow in the next few years.

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Literatur

  1. Hunzelmann N (2013) Systemic scleroderma. Hautarzt 64(4):299–310

    Article  CAS  PubMed  Google Scholar 

  2. Steen V, Denton CP, Pope JE et al (2009) Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatolog. 48(Suppl 3):iii19–24

    Google Scholar 

  3. Amanzi L, Braschi F, Fiori G, Galluccio F, Miniati I, Guiducci S, Conforti ML, Kaloudi O, Nacci F, Sacu O, Candelieri A, Pignone A, Rasero L, Conforti D, Matucci-Cerinic M (2010) Digital ulcers in scleroderma: staging, characteristics and sub-setting through observation of 1614 digital lesions. Rheumatology (Oxford) 49(7):1374–1382

    Article  Google Scholar 

  4. Wigley FM (2009) Vascular disease in scleroderma. Clin Rev Allergy Immunol 36:150–175

    Article  PubMed  Google Scholar 

  5. European Pressure Ulcer Advisory Panel (2006) Science and practice of pressure ulcer management. Springer, London

    Google Scholar 

  6. Korn JH, Mayes M, Matucci Cerinic M et al (2004) Digital ulcers in systemic sclerosis: prevention by treatment with Bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 50:3985–3993

    Article  CAS  PubMed  Google Scholar 

  7. Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM, Carpentier P, Wigley FM, Black CM, Fessler BJ, Merkel PA, Pope JE, Sweiss NJ, Doyle MK, Hellmich B, Medsger TA Jr, Morganti A, Kramer F, Korn JH, Seibold JR (2011) Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 70(1):32–38

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  8. Nihtyanova SI, Brough GM, Black CM, Denton CP (2008) Clinical burden of digital vasculopathy in limited and diffuse systemic sclerosis. Ann Rheum Dis 67:120–123

    Article  CAS  PubMed  Google Scholar 

  9. Abou-Raya A, Abou-Raya S, Helmii M (2008) Statins: potentially useful in therapy of systemic sclerosis related Raynaudʼs phenomenon and digital ulcers. J Rheumatol 35:1801–1808

    CAS  PubMed  Google Scholar 

  10. Tiev KP, Diot E, Clerson P, Dupuis-Simeon F, Hachulla E, Hatron P et al (2009) Clinical features of scleroderma patients with or without prior or current ischemic digital ulcers: post-hoc analysis of a nationwide multicenter cohort (ItinérAIR-Sclérodermie). J Rheumatol 36:1470–1476

    Article  PubMed  Google Scholar 

  11. Sunderkotter C, Herrgott I, Bruckner C, Moinzadeh P, Pfeiffer C, Gerss J, Hunzelmann N et al (2009) Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors. Br J Dermatol 160:835–843

    Article  CAS  PubMed  Google Scholar 

  12. Ennis H, Vail A, Wragg E, Taylor A, Moore T, Murray A, Muir L, Griffiths CE, Herrick AL (2013) A prospective study of systemic sclerosis-related digital ulcers: prevalence, location, and functional impact. Scand J Rheumatol 42(6):483–486

    Article  CAS  PubMed  Google Scholar 

  13. Bogoch ER, Gross DK (2005) Surgery of the hand in patients with systemic sclerosis: outcomes and considerations. J Rheumatol 32:642–648

    PubMed  Google Scholar 

  14. Ferri C, Valentini G, Cozzi F et al (2002) Systemic Sclerosis Study Group of the Italian Society of Rheumatology (SIR-GSSSc). Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine 81:139–153

    Article  PubMed  Google Scholar 

  15. Hachulla E, Clerson P, Launay D et al (2007) Natural history of ischemic digital ulcers in systemic sclerosis: single center retrospective longitudinal study. J Rheumatol 34:2423–2430

    PubMed  Google Scholar 

  16. Nihtyanova SI, Brough GM, Black CM, Denton CP (2008) Clinical burden of digital vasculopathy in limited and diffuse systemic sclerosis. Ann Rheum Dis 67:120–123

    Article  CAS  PubMed  Google Scholar 

  17. Ostoijc P, Damjanov P, Pavlov-Dolijanovic S, Radunovic G (2004) Peripheral vasculopathy in patients with systemic sclerosis. Clin Hemoreol Microcirc 31:281–285

    Google Scholar 

  18. Ennis H, Moore T, Murray A, Vail A, Herrick AL (2014) Further confirmation that digital ulcers are associated with the severity of abnormality on nailfold capillaroscopy in patients with systemic sclerosis. Rheumatology (Oxford). 53(2):376–377

    Article  Google Scholar 

  19. Smith V, Decuman S, Sulli A et al (2012) Do worsening scleroderma capillaroscopic patterns predict future severe organ involvement? A pilot study. Ann Rheum Dis 71:1636–1639

    Article  PubMed  Google Scholar 

  20. Sebastiani M, Manfredi A, Colaci M et al (2009) Capillaroscopic skin ulcer risk index: a new prognostic tool for digital skin ulcer development in systemic sclerosis patients. Arthritis Rheum 61:688–944

    Article  CAS  PubMed  Google Scholar 

  21. Smith V, De Keyser F, Pizzorni C et al (2011) Nailfold capillaroscopy for day-to-day clinical use: construction of a simple scoring modality as a clinical prognostic index for digital trophic lesions. Ann Rheum Dis 70:180–183

    Article  PubMed  Google Scholar 

  22. Kowal-Bielecka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L et al (2009) EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR). Ann Rheum Dis 68:620–628

    Article  CAS  PubMed  Google Scholar 

  23. Tingey T, Shu J, Smuczek J, Pope J (2013) Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res (Hoboken) 65(9):1460–1471

    Article  CAS  Google Scholar 

  24. Bali G, Aberer E (2003) Iloprost therapy in systemic sclerosis. Hautarzt 54(9):845–851

    Article  CAS  PubMed  Google Scholar 

  25. Wigley FM, Seibold JR, Wise RA, McCloskey DA, Dole WP (1992) Intravenous iloprost treatment of Raynaudʼs phenomenon and ischemic ulcers secondary to systemic sclerosis. J Rheumatol 19:1407–1414

  26. Wigley FM, Wise RA, Seibold JR, McCloskey DA, Kujala G, Medsger TA Jr et al (1994) Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med 120:199–206

    Article  CAS  PubMed  Google Scholar 

  27. Fries R, Shariat K, von Wilmowsky H, Bohm M (2005) Sildenafil in the treatment of Raynaud’s phenomenon resistant to vasodilatory therapy. Circulation 112:2980–2985

    CAS  PubMed  Google Scholar 

  28. Brueckner CS, Becker MO, Kroencke T et al (2010) Effect of sildenafil on digital ulcers in systemic sclerosis: analysis from a single centre pilot study. Ann Rheum Dis 69:1475–1478

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  29. Shenoy PD, Kumar S, Jha LK et al (2010) Efficacy of tadalafil in secondary Raynaudʼs phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Rheumatol 49:2420–2428

  30. Herrick AL (2011) Contemporary management of Raynaudʼs phenomenon and digital ischaemic complications. Curr Opin Rheumatol 23(6):555–561

    Article  PubMed  Google Scholar 

  31. Junker JP, Kamel RA, Caterson EJ, Eriksson E (2013) Clinical impact upon wound healing and inflammation in moist, wet, and dry environments. Adv Wound Care (New Rochelle) 2(7):348–356

    Article  Google Scholar 

  32. Moore ZE, Cowman S (2013) Wound cleansing for pressure ulcers. Cochrane Database Syst Rev 3:CD004983

    PubMed  Google Scholar 

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Interessenkonflikt

D. Belz, N. Hunzelmann und P. Moinzadeh geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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  1. Klinik und Poliklinik für Dermatologie und Venerologie, Uniklinik Köln, Kerpener Str. 62, 50937, Köln, Deutschland

    D. Belz, N. Hunzelmann & P. Moinzadeh

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  1. D. Belz
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  2. N. Hunzelmann
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  3. P. Moinzadeh
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Correspondence to D. Belz.

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Belz, D., Hunzelmann, N. & Moinzadeh, P. Digitale Ulzerationen bei systemischer Sklerodermie. Hautarzt 65, 944–948 (2014). https://doi.org/10.1007/s00105-014-3528-2

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