Skip to main content
main-content

Tipp

Weitere Artikel dieser Ausgabe durch Wischen aufrufen

Erschienen in: hautnah 3/2019

08.04.2019 | Bindegewebserkrankung

Aktuelle Therapie der systemischen Sklerodermie

verfasst von: Prof. Dr. Nicolas Hunzelmann

Erschienen in: hautnah | Ausgabe 3/2019

Einloggen, um Zugang zu erhalten
share
TEILEN

Zusammenfassung

Die systemische Sklerose (SSc, Sklerodermie) ist eine schwere chronisch entzündliche Bindegewebserkrankung der Haut, des muskuloskeletalen Systems und vieler innerer Organe. Der heterogene, langjährige Verlauf dieser Erkrankung mit dem Befall multipler Organe stellt eine besondere Herausforderung an den betreuenden Arzt dar. Die therapeutischen Möglichkeiten haben sich in den vergangenen Jahren in verschiedenen Bereichen entscheidend verbessert. Diese positiven Entwicklungen haben dazu geführt, dass in verschiedenen Arbeitsgruppen abgestimmte Empfehlungen zur Behandlung der SSc veröffentlicht wurden. Ziel dieser Übersicht ist es, die wesentlichen Empfehlungen vorzustellen und in ein Konzept einer modernen interdisziplinären Betreuung einzubetten.
Literatur
1.
2.
Zurück zum Zitat Belz D, Hunzelmann N, Moinzadeh P (2014) Digital ulcers in scleroderma. Hautarzt 65(11):944–948 CrossRefPubMed Belz D, Hunzelmann N, Moinzadeh P (2014) Digital ulcers in scleroderma. Hautarzt 65(11):944–948 CrossRefPubMed
3.
Zurück zum Zitat Coleiro B, Marshall SE, Denton CP, Howell K, Blann A, Welsh KI et al (2001) Treatment of Raynaud’s phenomenon with the selective serotonin reuptake inhibitor fluoxetine. Rheumatology 40:1038–1043 CrossRefPubMed Coleiro B, Marshall SE, Denton CP, Howell K, Blann A, Welsh KI et al (2001) Treatment of Raynaud’s phenomenon with the selective serotonin reuptake inhibitor fluoxetine. Rheumatology 40:1038–1043 CrossRefPubMed
4.
Zurück zum Zitat Denton CP, Hughes M, Gak N, Vila J, Buch MH, Chakravarty K, Fligelstone K, Gompels LL, Griffiths B, Herrick AL, Pang J, Parker L, Redmond A, van Laar J, Warburton L, Ong VH (2016) BSR and BHPR guideline for the treatment of systemic sclerosis. Rheumatology 55(10):1906–1910 CrossRefPubMed Denton CP, Hughes M, Gak N, Vila J, Buch MH, Chakravarty K, Fligelstone K, Gompels LL, Griffiths B, Herrick AL, Pang J, Parker L, Redmond A, van Laar J, Warburton L, Ong VH (2016) BSR and BHPR guideline for the treatment of systemic sclerosis. Rheumatology 55(10):1906–1910 CrossRefPubMed
5.
Zurück zum Zitat Dobrota R, Maurer B, Graf N, Jordan S, Mihai C, Kowal-Bielecka O, Allanore Y, Distler O (2016) EUSTAR coauthors.A Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis. Ann Rheum Dis 75(10):1743–1748 CrossRefPubMed Dobrota R, Maurer B, Graf N, Jordan S, Mihai C, Kowal-Bielecka O, Allanore Y, Distler O (2016) EUSTAR coauthors.A Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis. Ann Rheum Dis 75(10):1743–1748 CrossRefPubMed
6.
Zurück zum Zitat Elhai M, Meunier M, Matucci-Cerinic M et al (2013) EUSTAR (EULAR Scleroderma Trials and Research group). Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study. Ann Rheum Dis 72(7):1217–1220 CrossRefPubMed Elhai M, Meunier M, Matucci-Cerinic M et al (2013) EUSTAR (EULAR Scleroderma Trials and Research group). Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study. Ann Rheum Dis 72(7):1217–1220 CrossRefPubMed
7.
Zurück zum Zitat Hansi N, Thoua N, Carulli M, Chakravarty K, Lal S et al (2014) Consensus best practice pathway of the UK Scleroderma study group: gastrointestinal manifestations of systemic sclerosis. Clin Exp Rheumatol 32(Suppl 86):214–221 Hansi N, Thoua N, Carulli M, Chakravarty K, Lal S et al (2014) Consensus best practice pathway of the UK Scleroderma study group: gastrointestinal manifestations of systemic sclerosis. Clin Exp Rheumatol 32(Suppl 86):214–221
8.
Zurück zum Zitat Herrick AL (2012) The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol 8:469–479 CrossRefPubMed Herrick AL (2012) The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol 8:469–479 CrossRefPubMed
9.
Zurück zum Zitat Hoffmann-Vold AM, Aaløkken TM, Lund MB, Garen T, Midtvedt Ø, Brunborg C, Gran JT, Molberg Ø (2015) Predictive value of serial HRCT analyses and concurrent lung function tests in systemic sclerosis. Arthritis Rheumatol 67:2205–2212 CrossRefPubMed Hoffmann-Vold AM, Aaløkken TM, Lund MB, Garen T, Midtvedt Ø, Brunborg C, Gran JT, Molberg Ø (2015) Predictive value of serial HRCT analyses and concurrent lung function tests in systemic sclerosis. Arthritis Rheumatol 67:2205–2212 CrossRefPubMed
10.
Zurück zum Zitat van den Hoogen FH, Boerbooms AM, Swaak AJ, Rasker JJ, van Lier HJ, van de Putte LB (1996) Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol 35(4):364–372 CrossRefPubMed van den Hoogen FH, Boerbooms AM, Swaak AJ, Rasker JJ, van Lier HJ, van de Putte LB (1996) Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol 35(4):364–372 CrossRefPubMed
11.
Zurück zum Zitat Hughes M, Ong VH, Anderson ME, Hall F, Moinzadeh P, Griffiths B et al (2015) Consensus best practice pathway of the UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis. Rheumatology 54:2015–2024 CrossRefPubMed Hughes M, Ong VH, Anderson ME, Hall F, Moinzadeh P, Griffiths B et al (2015) Consensus best practice pathway of the UK Scleroderma Study Group: digital vasculopathy in systemic sclerosis. Rheumatology 54:2015–2024 CrossRefPubMed
12.
Zurück zum Zitat Hunzelmann N, Genth E, Krieg T et al (2008) The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology 47:1185–1192 CrossRefPubMedPubMedCentral Hunzelmann N, Genth E, Krieg T et al (2008) The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology 47:1185–1192 CrossRefPubMedPubMedCentral
13.
Zurück zum Zitat Knobler R, Moinzadeh P, Hunzelmann N et al (2017) European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol 31:1401–1424 CrossRefPubMed Knobler R, Moinzadeh P, Hunzelmann N et al (2017) European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol 31:1401–1424 CrossRefPubMed
14.
Zurück zum Zitat Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I et al (2017) 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 CrossRef Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I et al (2017) 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 CrossRef
15.
Zurück zum Zitat Kreuter A, Breuckmann F, Uhle A, Brockmeyer N, von Kobyletzki G et al (2004) Low-dose UVA1 phototherapy in systemic sclerosis: effects on acrosclerosis. J Am Acad Dermatol 50:740–747 CrossRefPubMed Kreuter A, Breuckmann F, Uhle A, Brockmeyer N, von Kobyletzki G et al (2004) Low-dose UVA1 phototherapy in systemic sclerosis: effects on acrosclerosis. J Am Acad Dermatol 50:740–747 CrossRefPubMed
16.
Zurück zum Zitat Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I et al (2013) Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomized controlled trials. Lancet Respir Med 1:369–376 CrossRefPubMedPubMedCentral Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I et al (2013) Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomized controlled trials. Lancet Respir Med 1:369–376 CrossRefPubMedPubMedCentral
17.
Zurück zum Zitat Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM et al (2011) Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 70:32–38 CrossRefPubMed Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM et al (2011) Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 70:32–38 CrossRefPubMed
18.
Zurück zum Zitat Mihai C, Landewé R, van der Haijde D, Walker UA, Constantin PI et al (2015) Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis 75:681–686 CrossRefPubMed Mihai C, Landewé R, van der Haijde D, Walker UA, Constantin PI et al (2015) Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis 75:681–686 CrossRefPubMed
19.
Zurück zum Zitat Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L (2014) Scleroderma renal crisis. J Rheumatol 41:1040–1048 CrossRefPubMed Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L (2014) Scleroderma renal crisis. J Rheumatol 41:1040–1048 CrossRefPubMed
20.
Zurück zum Zitat Riemekasten G, Hoffmann U, Sunderkötter C, Weiss N, Kuhn A (2012) angiologisch-dermatologisch-rheumatologische DU-Expertenboard. Management of digital ulcers in patients with systemic sclerosis. Dtsch Med Wochenschr 137:34–40 CrossRefPubMed Riemekasten G, Hoffmann U, Sunderkötter C, Weiss N, Kuhn A (2012) angiologisch-dermatologisch-rheumatologische DU-Expertenboard. Management of digital ulcers in patients with systemic sclerosis. Dtsch Med Wochenschr 137:34–40 CrossRefPubMed
21.
Zurück zum Zitat Roustit M, Blaise S, Allanore Y, Carpentier PH, Caglayan E, Cracowski JL (2013) Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud’s phenomenon: systematic review and meta-analysis of randomised trials. Ann Rheum Dis 72:1696–1699 CrossRefPubMed Roustit M, Blaise S, Allanore Y, Carpentier PH, Caglayan E, Cracowski JL (2013) Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud’s phenomenon: systematic review and meta-analysis of randomised trials. Ann Rheum Dis 72:1696–1699 CrossRefPubMed
25.
Zurück zum Zitat Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B, Mayes MD, Nash RA et al (2018) Myeloablative Autologous stem-cell transplantation for severe Scleroderma. N Engl J Med 378:35–47 CrossRefPubMedPubMedCentral Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B, Mayes MD, Nash RA et al (2018) Myeloablative Autologous stem-cell transplantation for severe Scleroderma. N Engl J Med 378:35–47 CrossRefPubMedPubMedCentral
26.
Zurück zum Zitat Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE et al (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354:2655–2666 CrossRefPubMed Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE et al (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354:2655–2666 CrossRefPubMed
27.
Zurück zum Zitat Tashkin DP, Roth MD, Clements PJ et al (2016) Mycophenylate versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 4:208 CrossRef Tashkin DP, Roth MD, Clements PJ et al (2016) Mycophenylate versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 4:208 CrossRef
28.
Zurück zum Zitat Tingey T, Shu J, Smuczek J, Pope J (2013) Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res 65:1460–1471 CrossRef Tingey T, Shu J, Smuczek J, Pope J (2013) Meta-analysis of healing and prevention of digital ulcers in systemic sclerosis. Arthritis Care Res 65:1460–1471 CrossRef
29.
Zurück zum Zitat Van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z et al (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311:2490–2498 CrossRefPubMed Van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z et al (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311:2490–2498 CrossRefPubMed
30.
Zurück zum Zitat 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 CrossRefPubMed 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 CrossRefPubMed
Metadaten
Titel
Aktuelle Therapie der systemischen Sklerodermie
verfasst von
Prof. Dr. Nicolas Hunzelmann
Publikationsdatum
08.04.2019
Verlag
Springer Vienna
Erschienen in
hautnah / Ausgabe 3/2019
Print ISSN: 1866-2250
Elektronische ISSN: 2192-6484
DOI
https://doi.org/10.1007/s12326-019-0326-8