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Iloprosttherapie bei systemischer Sklerodermie

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Zusammenfassung

Die systemische Sklerodermie ist eine seltene, mehrere Organsysteme betreffende Autoimmunerkrankung unklarer Genese. Der Krankheitsverlauf ist mit den heutigen therapeutischen Möglichkeiten schwer zu kontrollieren. Iloprost, ein stabiles Prostazyklinanalogon, kann durch seinen antifibrotischen Effekt einen wichtigen Schritt in der Pathogenese der Sklerodermie beeinflussen. In dieser Übersichtsarbeit wurde aus der Literatur die optimale Dosierung und Therapiedauer herausgearbeitet. In 3 verschiedenen Studien erwies sich eine Infusionstherapie über 5 Tage in einer vom Patienten am besten vertragenen Maximaldosis (1–2 ng/kg/min) als wirksam mit einem signifikanten Effekt auf die Hautveränderungen wie Schwere und Zahl der Raynaud-Anfälle, die Heilung der Ulzera und die digitale Durchblutung. Diese Wirkung hält etwa 4 Wochen an und kann durch Wiederholung der Infusionen 1-mal/Monat aufrechterhalten werden. Obwohl noch weitere Studien erforderlich sind, um einen Langzeiteffekt zu überprüfen, dürfte eine intermittierende Dauertherapie mit Iloprost zu einer Verbesserung oder Stabilisierung der Hautveränderungen bei Sklerodermie führen.

Abstract

Systemic sclerosis is an autoimmune disease of unknown origin affecting multiple organ systems. The management of this disease is challenging. Many therapeutic attempts have only been moderately successful. Iloprost, a stable prostacyclin analogue, with its antifibrotic effect can influence an important step in the pathogenesis of systemic sclerosis. In this review we analyze the published data for the optimal dose and duration of treatment. In three different studies, iloprost given for 5 days in the highest tolerated dose of 1–2 ng/kg/min provided a significant improvement of the disease measured by the number and intensity of Raynaud attacks, healing of digital ulcers, and digital perfusion. This improvement lasted for about one month. When the infusions were repeated once a month, treatment effect could be maintained. Although the effect of this treatment regimen should be proven in further long-term studies, we think that an intermittent continuous therapy with iloprost could result in an improvement or stabilization of systemic sclerosis.

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Literatur

  1. Aigner N, Petje G, Steinboeck G et al. (2001) Treatment of bone-marrow oedema of the talus with the prostacyclin analogue iloprost. J Bone Joint Surg 83-B:855–858

    Google Scholar 

  2. Belch J (2000) Therapy of Raynaud's phenomenon and vasculitis. Schering Symposium, Berlin 18.–19.2.2000, S 42–43

  3. Belch J, Capell HA, Cooke ED et al. (1995) Oral iloprost as a treatment for Raynaud's syndrome: a double blind multicentre placebo controlled study. Ann Rheum Dis 54:197–200

    CAS  PubMed  Google Scholar 

  4. Biasi D, Carletto A, Caramaschi P et al. (1998) Iloprost as cyclic five-day infusions in the treatment of scleroderma. Rev Rhum 65:745–750

    CAS  Google Scholar 

  5. Ceru S, Pancera P, Sansone S et al. (1997) Effects of five-day versus one-day infusion of iloprost on the peripheral microcirculation in patients with systemic sclerosis. Clin Exp Rheumatol 15:381–385

    CAS  PubMed  Google Scholar 

  6. Clements PJ, Lanchenbruch PA, Cheng S et al. (1990) Skin score. A semiquantitative measure of cutaneous involvement that improves prediction of prognosis in systemic sclerosis. Arthritis Rheum 33:1256–1263

    CAS  PubMed  Google Scholar 

  7. Della Bella S, Molteni M, Mascagni B et al. (1997) Cytokine production in scleroderma patients: effects of therapy with either iloprost or nifedipine. Clin Exp Rheum 15:135–141

    Google Scholar 

  8. Della Bella S, Molteni M, Mocellin C et al. (2001) Novel mode of action of iloprost: in vitro down-regulation of endothelial cell adhesion molecules. Prostaglandins Lipid Mediators 65:73–83

    Article  Google Scholar 

  9. Fagrell B (2000) Iloprost—a stable prostacyclin analogue. Schering Symposium Berlin, 18.–19.2.2000, S 6–8

  10. Filaci G, Cutolo M, Scudeletti M et al.(1999) Cyclosporin A and iloprost treatment of systemic sclerosis: clinical results and interleukin-6 serum changes after 12 months of therapy. J Rheumatol 38:992–996

    Article  CAS  Google Scholar 

  11. Gröchenig E (1994) Treatment of frostbite with iloprost. Lancet 344:1152–1153

    Google Scholar 

  12. Gröchenig E (2002) Gefäßmedizin. ABW Wissenschaftsverlag Berlin Leiben, S 94–96

  13. Kahaleh MB (1994) Raynaud's phenomenon and vascular disease in scleroderma. Curr Opin Rheumatol 6:621–627

    CAS  PubMed  Google Scholar 

  14. Keller J, Kaltenecker A, Schricker KT et al. (1984) Behandlung des Raynaud-Phänomens bei Sklerodermie-Patienten mit einem neuen stabilen Prostacyclin-Derivat. Dtsch Med Wochenschr 109:1433–1438

    CAS  PubMed  Google Scholar 

  15. LeRoy EC, Krieg T, Black C et al. (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202–205

    CAS  PubMed  Google Scholar 

  16. Lüllmann H, Mohr K (1999) Pharmakologie und Toxikologie, 14. Aufl. Thieme, Stuttgart New York, S 269–272

  17. Mazzone A, Mazzucchelli I, Fossati G et al. (1996) Iloprost effects on phagocytes in patients suffering from ischaemic diseases: in vivo evidence for down-regulation of αMβ2 integrin. Eur J Clin Invest 26:860–866

    CAS  PubMed  Google Scholar 

  18. Mazzone A, Cusa C, Bucci L et al. (1999) The effects of iloprost infusion on microcirculation is independent of nitric oxide metabolites and endothelin-1 in chronic peripheral ischaemia. Eur J Clin Invest 29:1–5

    Article  CAS  Google Scholar 

  19. Mazzone A, Cusa C, Mazzucchelli I et al. (1999) Pyoderma gangrenosum complicative ulcerative colitis: an alternative treatment with Iloprost. Current Therapeutic Res 60:357–362

    Article  CAS  Google Scholar 

  20. Medsger TA, Silman AJ, Steen VD et al. (1999) A disease severity scale for systemic sclerosis: development and testing. J Rheumatol 26:2159–2167

    PubMed  Google Scholar 

  21. Mittag M, Beckheinrich P, Haustein UF (2001) Systemic sclerosis-related Raynaud's phenomenon: effects of Iloprost infusion therapy on serum cytokine, growth factor and soluble adhesion molecule levels. Acta Derm Venereol 81:294–297

    Article  CAS  PubMed  Google Scholar 

  22. Olschewski H, Walmrath D, Schermuly R et al. (1996) Aerosolised prostacyclin and iloprost in severe pulmonary hypertension. Ann Intern Med 124:820–824

    CAS  PubMed  Google Scholar 

  23. Olschewski H, Ghofrani A, Schmedl T et al. (2000) Inhaled iloprost to treat severe pulmonary hypertension. Ann Intern Med 132:435–443

    CAS  PubMed  Google Scholar 

  24. Rademaker M, Thomas RHM, Provost G et al. (1987) Prolonged increase in digital blood flow following iloprost infusion in patients with systemic sclerosis. Postgrad Med J 63:617–620

    CAS  PubMed  Google Scholar 

  25. Riemekasten G, Jepsen H, Burmester GR, Hiepe F (1998) Iloprostgabe über 21 Tage als wirksame Therapie bei der progressiven systemischen Sklerodermie—Fallbeschreibung und Literaturübersicht. Z Rheumatol 57:118–124

    CAS  PubMed  Google Scholar 

  26. Righi A, Matucci Cerinic M (2002) New treatments in scleroderma: the rheumatologic perspective. JEADV 16:431–432

    Article  CAS  PubMed  Google Scholar 

  27. Schuller-Petrovic S (2000) Iloprost in the treatment of venous ulcers. Schering Symposium Berlin 18.–19.2.2000, S 20–21

  28. Schulte KL (2000) Dosing alternatives for iloprost in the treatment of peripheral arterial disease (PAD). Schering Symposium Berlin, 18.–19.2.2000, S 12–13

  29. Smiley JD (1992) The many faces of scleroderma. Am J Med Sci 304:319–333

    CAS  PubMed  Google Scholar 

  30. Stratton R, Shiwen X, Martini G et al. (2001) Iloprost suppresses connective tissue growth factor production in fibroblasts and in the skin of scleroderma patients. J Clin Invest 108:241–250

    Article  CAS  PubMed  Google Scholar 

  31. Tu JH, Eisen AZ (1999) Scleroderma. In: Freedberg IM, Eisen AZ, Wolff K et al. (eds) Dermatology in general medicine, Vol 2, 5th edn. Mc Graw-Hill Inc., New York, pp 2023–2033

  32. Wighley FM, Sule SD (2001) Novel therapy in the treatment of scleroderma. Exp Opin Invest Drug 20s 101:31–48

    Google Scholar 

  33. Wigley FM, Wise RA, Seibold JR et al. (1994) Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. Ann Intern Med 120:199–206

    CAS  PubMed  Google Scholar 

  34. Williamson DJ, Wallman L, Jones R et al. (2000) Hemodynamic effects of Bosentan, an endothelin receptor antagonist in patients with pulmonary hypertension. Circulation 102:411–418

    CAS  PubMed  Google Scholar 

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Bali, G., Aberer, E. Iloprosttherapie bei systemischer Sklerodermie. Hautarzt 54, 845–851 (2003). https://doi.org/10.1007/s00105-003-0578-2

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