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Interdisziplinäre klinische Rheumatologie pp 801–816Cite as

Systemische Sklerose (M 34)

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Die systemische Sklerose (SSc) ist eine Multiorganerkrankung mit Entzündung und Fibrose, die regelmäßig die Haut und die Blutgefäße (Arterien, kleine Blutgefäße), oft auch die Lunge, den Magen-Darm-Trakt (Ösophagus, Magen, Duodenum, Jejunum und Ileum), seltener auch die Niere, das Herz, die exokrinen Drüsen und das Bewegungssystem betrifft. Im Vordergrund der klinischen Erscheinungen stehen die Sklerodermie (Dermatofibrose) unterschiedlicher Intensität und Ausdehnung (Akrosklerose, limitierte Sklerodermie, diffuse Sklerodermie) und die Durchblutungsstörungen, insbesondere der Finger und Zehen (Raynaud-Phänomen, Fingerkuppenulzera) mit progredientem Substanzverlust an den Akren.

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Literatur

  • Abu-Shakra M, Lee P (1995) Mortality in systemic sclerosis: A comparison with the general population. J Rheumatol 22: 2100–2102

    PubMed  CAS  Google Scholar 

  • Akeson A, Gustafson T, Wollheim F, Brismar J (1987) Esophageal dysfunction and radionuclide transit in progressive systemic sclerosis. Scand J Rheumatol 16: 291–299

    Article  Google Scholar 

  • Baroni SS, Santillo M, Bevilacqua F, Luchetti M, Spadoni T, Mancini M, Fraticelli P, Sambo P, Funaro A, Kazlauskas A, Avvedimento EV, Gabrielli A (2006) Stimulatory autoantibodies to the PDGF receptor in systemic sclerosis. N Engl J Med 354: 2667–2676

    Article  PubMed  CAS  Google Scholar 

  • Bovenzi M, Barbone F, Betta A, Tommasini M, Versini W (1995) Scleroderma and occupational exposure. Scand J Work Environ Health 21: 289–292

    PubMed  CAS  Google Scholar 

  • Calore EE, Cavaliere MJ, Perez NM, Takayasu V, Wakamatsu A, Kiss MH (1995) Skeletal muscle pathology in systemic sclerosis. J Rheumatol 22: 2246–2249

    PubMed  CAS  Google Scholar 

  • Clements PJ (1995) Measuring disease activity and severity in scleroderma. Curr Opin Rheumatol 7: 517–521

    PubMed  CAS  Google Scholar 

  • Clements PJ, Furst DE, Wong WK, Mayes M, White B, Wigley F, Weisman MH, Barr W, Moreland LW, Medsger TA jr, Steen V, Martin RW, Collier D, Weinstein A, Lally E, Varga J, Weiner S, Andrews B, Abeles M, Seibold JR (1999) High-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis: analysis of a two-year, doubleblind, randomized, controlled clinical trial. Arthritis Rheum 42: 1194–1203

    Article  PubMed  CAS  Google Scholar 

  • Clements PJ, Lachenbruch PA, Cheng Ng S, Simmons M, Sterz M, Furst DE (1990) Skin score. A semiquantitative measure of cutaneous involvement that improves prediction of prognosis in systemic sclerosis. Arthritis Rheum 33: 1256–1263

    PubMed  CAS  Google Scholar 

  • Clements PJ, Lachenbruch PA, Seibold JR et al. (1993a) Skin thickness score in systemic sclerosis: An assessment of interobserver variabillty in 3 independent studies. J Rheumatol 20: 1892–1896

    PubMed  CAS  Google Scholar 

  • Clements PJ, Lachenbruch PA, Sterz M et al. (1993b) Cyclosporine in systemic sclerosis. Results of a forty-eight-week open safety study in ten patients. Arthritis Rheum 36: 75–83

    Article  PubMed  CAS  Google Scholar 

  • Crepy MN, Conso F (1996) Sclérodermie et facteurs professionels (ou environnementaux) — Sklerodermie und berufliche (oder Umwelt-) Faktoren. Zbl Arbeitsmed 46: 155

    Google Scholar 

  • Czirjak L, Nagy Z, Szegedi G (1993) Survival analysis of 118 patients with systemics clerosis. J Intern Med 234: 335–337

    PubMed  CAS  Google Scholar 

  • Czirjak L, Szegdi G (1986) Nifedipine treatment for progressive systemic sclerosis. Arthritis Rheum 29: 1053–1054

    Article  PubMed  CAS  Google Scholar 

  • D’Angelo WA, Fries JF, Masi AT, Shulman LE (1969) Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med 46: 429–440

    Article  Google Scholar 

  • Denton CP, Bickerstaff MC,, Shiwen X et al. (1995) Serial circulating adhesion molecule levels reflect disease severity in systemics sclerosis. Br J Rheumatol 34: 1048–1054

    Article  PubMed  CAS  Google Scholar 

  • Denton CP, Black CM (2003) Pulmonary hypertension in systemic sclerosis. Rheum Dis Clin. Noth Am 29: 335–341

    Article  Google Scholar 

  • Fox RI, Kangh (1992) Genetic and environmental factors in systemic sclerosis. Curr Opin Rheumatol 4: 857–861

    PubMed  CAS  Google Scholar 

  • Furst DE, Clements PJ (1997) Hypothesis for the pathogenesis of systemic sclerosis. J Rheumatol 24: 53–57 (Abstract)

    Google Scholar 

  • Furst DE, Clements PJ, Graze P, Gale R, Roberts N (1979) A syndrome resembling progressive systemic sclerosis after Bone marrow transplantation. Arthritis Rheum 22: 904–910

    Article  PubMed  CAS  Google Scholar 

  • Genth E (1994 a) Autoantikörper-definierte Varianten der systemischen Sklerose — klinische und immungenetische Aspekte. Nieren Hochdruckkrankh 23: 596–604

    Google Scholar 

  • Genth E (1994 b) Autoantikörper in der Diagnostik systemischer rheumatischer Erkrankungen. Kassenarzt 37: 37–43

    Google Scholar 

  • Genth E (1996) Serologisch-immunologische Erkenntnishilfen in der Diagnostik autoimmuner rheumatischer Erkrankungen. In Gerok W et al. (Hrsg) Klinik der Gegenwart. Urban & Schwarzenberg, München, X pp XIV, 7: 1-XIV, 7: 22

    Google Scholar 

  • Genth E, Kaufmann S, Mierau R (1993) Das Anti-(Aminoacyl-tRNA-) Synthetase-Syndrom (Jo-1-Syndrom). Ein eigenständiges Autoantikörper-assoziiertes Krankheitsbild mit Myositis, fibrosierender Alveolitis und Polyarthritis. Akt Rheumatol 18: 113–119

    Article  Google Scholar 

  • Genth E, Mierau R (1993) Skerodermie-assoziierte Autoantikörper. Z Rheumatol 52: 323

    Google Scholar 

  • Genth E, Mierau R, Genetzky P et al. (1990) Immunogeneticassociations of scleroderma-related antinuclear antibodies. Arthritis Rheum 33: 657–665

    Article  PubMed  CAS  Google Scholar 

  • Goulding R (1994) Toxic oil syndrome — Erythro-myalgia syndrome. In: Kochen W, Steinharth (eds) L-tryptophan: Current prospects in medicine and drug safety. De Gruyter, Berlin, pp 284–292

    Google Scholar 

  • Haustein U, Ziegler V (1986) Sklerodermie und Sklerodermie-ähnliche Erkrankungen durch Umweltsubstanzen. Dematosen 34: 61–67

    CAS  Google Scholar 

  • Herrick AL, Illingworth K, Blann A, Hay CRM, Hollis S, Jayson MIV (1996) Von Willebrand factor, thrombomodulin, thromboxane, b-thromboglobulin and markers of fibrinolysis in primary Raynaud’s phenomenon and systemic sclerosis. Ann Rheum Dis 55: 122–127

    Article  PubMed  CAS  Google Scholar 

  • Kahaleh B, Matucci-Cerinic M (1995) Raynaud’s phenomenon and scleroderma. Arthritis Rheum 38: 1–4

    Article  PubMed  CAS  Google Scholar 

  • Krieg T, Meurer M (1988) Continuing medical education. J Am Acad Dermatol 18: 457–481

    Article  PubMed  CAS  Google Scholar 

  • Kuwana M, Kaburaki J, Okano Y, Tojo T, Homma M (1994) Clinical and prognostic associations based on serum anti nuclear antibodies in Japanese patients with systemic sclerosis. Arthritis Rheum 37: 75–83

    Article  PubMed  CAS  Google Scholar 

  • Lee P, Langevitz P, Alderdice CA et al. (1992) Mortality in systemic sclerosis (scleroderma). Q J Med 82: 139–148

    PubMed  CAS  Google Scholar 

  • LeRoy EC (1992) Raynaud’s phenomenon, scleroderma, overlap syndromes, and other fibrosing syndromes. Curr Opin Rheumatol 4: 821–824

    PubMed  CAS  Google Scholar 

  • LeRoy EC (1996) Systemic sclerosis. A vascular perspective. Rheum Dis Clin North Am 22/4: 675–694

    Article  Google Scholar 

  • LeRoy EC, Black C, Fleischmajer R et al. (1988) Scleroderma (systemic sclerosis): Classification subsets and pathogenesis. J Rheumatol 15: 202–205

    PubMed  CAS  Google Scholar 

  • LeRoy EC, Maricq HR, Kahaleh MB (1980) Undifferentiated connective tissue syndromes. Arthritis Rheum 23: 341–343

    Article  PubMed  CAS  Google Scholar 

  • Masi AT, Rodnan GP, Medsger T et al. (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23: 581–590 (Abstract)

    Article  Google Scholar 

  • Medsger TA (1988) Classification of systemic sclerosis. In: Jayson MIV, Black CM (eds) Systemic sclerosis: scleroderma. Wiley, Chichester New York, pp 1–6

    Google Scholar 

  • Medsger T (1993) Arthritis and allied conditions. (Eds: McCarthy DJ, Koopman WJ) Philadelphia, Lea & Febiger

    Google Scholar 

  • Meyrick Thomas RH, Rademaker M, Grimes SM et al. (1987) Nifedipine in the treatment of Raynaud’s phenomenon in patients with systemic sclerosis. Br J Dermatol 117: 237–241

    Article  PubMed  CAS  Google Scholar 

  • Morita A, Sakakibara S, Sakakibara N, Yamauchi R, Tsuji T (1995) Successful treatment of systemic sclerosis with topical PUVA. J Rheumatol 22: 2361–2365 (Abstract)

    PubMed  CAS  Google Scholar 

  • Nash RA, McSweeney PA, Storb R et al. (1997) Development of a protocol for allogeneic marrow transplantation for severe systemic sclerosis: Paradigm for autoimmune disease. J Rheumatol 24: 72–78 (Abstract)

    Google Scholar 

  • Oelzner O, Hein G, Uhlemann C, Wollina U (1995) Therapie der systemischen Sklerose, Qualitätssicherung in der Rheumatologie. (Eds: Genth E et al. und Kommission Qualitätssicherung der Deutschen Gesellschaft für Rheumatologie). Steinkopff, Darmstadt

    Google Scholar 

  • Pereira S, Black C, Welch K et al. (1987) Autoantibodies and immunogenetics in 30 patients with systemic sderosis and their families. J Rheumatol 14: 760–765

    PubMed  CAS  Google Scholar 

  • Poormoghim H, Lucas M, Fertig N, Medsger TA, Jr (2000) Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. Arthritis Rheum 43: 444–451

    Article  PubMed  CAS  Google Scholar 

  • Pope J (1993) Treatment of systemic sclerosis. Curr Opin Rheumatol 14: 792–801

    Google Scholar 

  • Reveille JD (1995) Molecular genetics of systemic sclerosis. Curr Opin Rheumato 17: 522–528

    Article  Google Scholar 

  • Reveille JD, Durban E, MacLeod-StClair M et al. (1992 a) Association of amino acid sequences in the HLA-DQB1 first domain with the antitopoisomerase I autoantibody reponse in scleroderma (progressive systemic sclerosis). J Clin Invest 89: 973–980

    Article  Google Scholar 

  • Reveille JD, Owerbach D, Goldstein R, Moreda R, Isern RA, Arnett FC (1992 b) Association of polar amino acids at position 26 of the HLA-DQB 1 first domain with the anticentromere autoantibody response in systemic sclerosis (scleroderma). J Clin Invest 90: 1208–1213

    Article  Google Scholar 

  • Sakkas LI (2005) New developments in the pathogenesis of systemic sclerosis. Autoimmunity 38: 113–116

    Article  PubMed  CAS  Google Scholar 

  • Sattar MA, Guindi RT, Sugathan TN (1990) Penicillamine in systemic sclerosis: A reappraisal. Clin Rheumatol 9: 517–522

    Article  PubMed  CAS  Google Scholar 

  • Scheja A, Akesson A, Niewierowicz I, Wallin L, Wildt M, Wollheim FA (1996) Computer based quantitative analysis of capillary abnormalities in systemic sclerosis and its relation to plasma concentration of von Willebrand factor. Ann Rheum Dis 55: 52–56

    Article  PubMed  CAS  Google Scholar 

  • Seibold JR (1994) Systemic sclerosis — clinical features. In: Klippel JH, Dieppe PA (eds) Rheumatology. Mosby, St. Louis

    Google Scholar 

  • Sgonc R, Gruschwitz MS, Boeck G, Sepp N, Gruber J, Wick G (2000) Endothelial cell apoptosis in systemic sclerosis is induced by antibody-dependent cell-mediated cytotoxicity via CD95. Arthritis Rheum 43: 2550–2562

    Article  PubMed  CAS  Google Scholar 

  • Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR (1972) Mixed connective tissue disease. An apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA) Am J Med 52: 148–159

    Article  PubMed  CAS  Google Scholar 

  • Silman AJ (1991) Scleroderma and survival. Ann Rheum Dis 50: 267–269

    Article  PubMed  CAS  Google Scholar 

  • Silman AJ (1997) Scleroderma — Demographics and survival. J Rheumatol 24: 58–61 (Abstract)

    Google Scholar 

  • Silman AJ, Jones S (1992) What is the contribution of occupational environmental factors to the occurrence of scleroderma in men? Ann Rheum Dis 51: 1322–1324

    Article  PubMed  CAS  Google Scholar 

  • Silman AJ, Newman J (1994) Genetic and environmental factors in scleroderma. Curr Opin Rheumatol 6: 607–611

    Article  PubMed  CAS  Google Scholar 

  • Silver RM (1992) Eosinophilia-myalgia syndrome, toxic-oil syndrome, and diffuse fasciitis with eosinophilia. Curr Opin Rheumatol 4: 851–856

    PubMed  CAS  Google Scholar 

  • Smith EA, LeRoy EC (1994) Systemic sclerosis: etiology and pathogenesis. In: Klippel JH, Dieppe PA (eds) Rheumatology. Mosby, St. Louis

    Google Scholar 

  • Spiera RF, Gibofsky A, Spierah (1994) Siliconegel filled breast implants and connective tissue disease: An overview. J Rheumatol 21: 239–245

    PubMed  CAS  Google Scholar 

  • Steen VD (1996) Scleroderma renal crisis. Rheum Dis Xlin North Am 22: 861–878

    Article  CAS  Google Scholar 

  • Steen VD, Medsger TA (1997) The palpable tendon friction rub: An important physical examination finding in patients with systemic sclerosis. Arthritis Rheum 40: 1146–1151 (Abstract)

    Article  PubMed  CAS  Google Scholar 

  • Steen VD, Owens GR, Redmond C, Rodnan GP, Medsger TA (1985) The effect of D-penicillamine on pulmonary findings in systemic sclerosis. Arthritis Rheum 28: 882–888

    Article  PubMed  CAS  Google Scholar 

  • Steen VD, Costantino JP, Shapiro AP, Medsger TA (1990) Outcome of renal crisis in systemic sclerosis: Relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med 113: 352–357

    PubMed  CAS  Google Scholar 

  • Steen VD, Graham G, Conte C, Owens G. Medsger TA (1992) Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 35: 765–770

    Article  PubMed  CAS  Google Scholar 

  • Steen VD, Conte C, Owens GR, Medsger TA (1994 a) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37: 1283–1289

    Article  PubMed  CAS  Google Scholar 

  • Steen VD, Lanz JK, Conte C, Owens GR, Medsger TA (1994 b) Therapy for severe interstitial lung disease in systemic sclerosis. Arthritis Rheum 37: 1290–1296

    Article  PubMed  CAS  Google Scholar 

  • Stephens CO, Briggs DC, Whyte J et al. (1994) Familial scleroderma — Evidence for environmental vs. genetic trigge. Br J Rheumatol 33: 1131–1135

    Article  PubMed  CAS  Google Scholar 

  • Stupi AM, Steen VD, Owens GR, Barnes EL, Rodnan GP, Medsger TA (1986) Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum 29: 515–524

    Article  PubMed  CAS  Google Scholar 

  • Swartz RD, Crofford LJ, Phan SH, Ike RW Su LD (2003) Nephrogenic fibrosing dermopathy: a novel cutaneous fibrosing disorder in patients with renal failure. Am. J Med. 114: 563–572 Tashkin DP, Elashoff R, Clements PJ et al. (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354: 2655–2666

    Article  PubMed  Google Scholar 

  • van den Hoogen FHJ, Boerbooms AMT, Swaak AJG, Rasker JJ, van Lier HJJ, van de Putte LBA (1996) Comparison of methotrexate with placebo in the treatment of systemic sderosis: A 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol 35: 364–372

    Article  PubMed  Google Scholar 

  • White B (1996). Immunopathogenesis of systemic sclerosis. Rheum Dis Clin North Am 22: 695–708

    Article  PubMed  CAS  Google Scholar 

  • Wigley FM, Flavahan NA (1996) Raynaud’s phenomenon. Rheum Dis Clin North Am 22: 765–781

    Article  PubMed  CAS  Google Scholar 

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  1. Rheumaklinik Aachen, Burtscheider Markt 24, 52066, Aachen

    E. Genth Prof. Dr.

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Editors and Affiliations

  1. Rheumatologikum, Rathenaustraße 13/14, 30159, Hannover

    Henning Zeidler

  2. Klinik für Orthopädie und orthopädische Rheumatologie, HELIOS Klinikum Berlin-Buch, Schwanebecker Chaussee 50, 13125, Berlin

    Josef Zacher

  3. Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie, Universitätsklinikum Charité Med. Fakultät, Humboldt-Universität zu Berlin, Charitéplatz 1, 10117, Berlin

    Falk Hiepe

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Genth, E. (2008). Systemische Sklerose (M 34). In: Zeidler, H., Zacher, J., Hiepe, F. (eds) Interdisziplinäre klinische Rheumatologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-34105-5_60

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