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Systemische Sklerodermie

Aktuelle Klassifikation und Diagnostik der Organbeteiligung

Systemic sclerosis

Current classification and diagnosis of organ involvement

Der Hautarzt volume 66, pages 599–603 (2015)Cite this article

Zusammenfassung

Die systemische Sklerodermie (SSc) ist eine chronisch entzündliche Autoimmunerkrankung mit unbekannter Ätiologie, die zu Ablagerung von Kollagen und Extrazellulärmatrixproteinen in der Haut und den betroffenen inneren Organen führt. Die Diagnose der SSc wird anhand von klinischen, serologischen und apparativen Untersuchungen gestellt. Im Jahr 2013 wurden neue Kriterien zur Klassifikation der SSc veröffentlicht, die auch frühe Formen der SSc berücksichtigen. Eine eingehende klinische und apparative Untersuchung ist in den oligosymptomatischen Anfangsstadien und im weiteren Verlauf der SSc entscheidend, um rechtzeitig beginnende Organbeteiligungen diagnostizieren und behandeln zu können.

Abstract

Systemic sclerosis (SSc) is a rare, chronic inflammatory autoimmune disease with unknown etiology, which leads to deposition of collagen and extracellular matrix proteins in the skin and affected internal organs. The diagnosis of SSc is based on clinical, serological, and paraclinical examinations. In 2013 new criteria for the classification of systemic sclerosis, which also take early forms of SSc into consideration, were developed. A complete clinical and paraclinical examination is important for the oligosymptomatic early stages and the subsequent disease course of SSc in order to diagnose and timely treat a developing organ involvement.

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Literatur

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

    CAS  PubMed  Article  Google Scholar 

  2. Masi AT, Subcommittee For Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 23(5):581–590

    Article  Google Scholar 

  3. LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205

    CAS  PubMed  Google Scholar 

  4. Aringer M, Müller-Ladner U, Burkhardt et al (2015) Gemeinsame deutschsprachige Nomenklatur für die systemische Sklerose. Z Rheumatol 74(2):100–103

    CAS  PubMed  Article  Google Scholar 

  5. van den Hoogen F, Khanna D, Fransen J et al (2013) 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747

    PubMed Central  PubMed  Article  Google Scholar 

  6. Moinzadeh P, Aberer E, Ahmadi-Simab K et al (2015) Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. Ann Rheum Dis 74(4):730–737

    CAS  PubMed Central  PubMed  Article  Google Scholar 

  7. 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(8):1185–1192

    CAS  PubMed Central  PubMed  Article  Google Scholar 

  8. Koenig M, Joyal F, Fritzler MJ et al (2008) Autoantibodies and Microvascular Damage Are Independent Predictive Factors for the Progression of Raynaud’s Phenomenon to Systemic Sclerosis. Arthritis Rheum 58(12):3902–3912

    PubMed  Article  Google Scholar 

  9. Czirják L, Kumánovics G, Varjú C et al (2008) Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis 67(1):59–63

    PubMed  Article  Google Scholar 

  10. Moinzadeh P, Fonseca C, Hellmich et al (2014) Association of anti-RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 16(1):R53

    PubMed Central  PubMed  Article  Google Scholar 

  11. Steen VD (2003) Scleroderma renal crisis. Rheum Dis Clin North Am 29(2):315–333

    PubMed  Article  Google Scholar 

  12. Mierau R, Moinzadeh P, Riemekasten G et al (2011) Frequency of disease-associated and other nuclear autoantibodies in patients of the German network for systemic scleroderma: correlation with characteristic clinical features. Arthritis Res Ther 13(5):R172

    CAS  PubMed Central  PubMed  Article  Google Scholar 

  13. Kuwana M, Kaburaki J, Okana Y, Tojo T, Homma M (1994) Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis. Arthritis Rheum 37(1):75–83

    CAS  PubMed  Article  Google Scholar 

  14. Hunzelmann N, Genth E, Krieg T et al (2008) Organ-specific diagnosis in patients with systemic sclerosis: recommendations of the German Network for Systemic Sclerosis (DNSS). Z Rheumatol 67(4):334–336

    CAS  PubMed  Article  Google Scholar 

  15. Scheja A, Akesson A (1997) Comparison of high frequency (20 MHz) ultrasound and palpation for the assessment of skin involvement in systemic sclerosis (scleroderma). Clin Exp Rheumatol 15(3):283–288

    CAS  PubMed  Google Scholar 

  16. Belz D, Hunzelmann N, Moinzadeh P (2014) Digital ulcers in systemic scleroderma. Hautarzt 65(11):944–948

    CAS  PubMed  Article  Google Scholar 

  17. Schlager O, Gschwandtner ME, Herberg et al (2010) Correlation of infrared thermography and skin perfusion in Raynaud patients and in healthy controls. Microvasc Res 80(1):54–57

    PubMed  Article  Google Scholar 

  18. Rossi D, Russo A, Manna E, et al (2013) The role of nail-videocapillaroscopy in early diagnosis of scleroderma. Autoimmun Rev 12(8):821–825

    PubMed  Article  Google Scholar 

  19. Mimura Y, Ihn H, Jinnin M (2005) Clinical and laboratory features of scleroderma patients developing skeletal myopathy. Clin Rheumatol 24(2):99–102

    PubMed  Article  Google Scholar 

  20. Latsi PI, Wells AU (2003) Evaluation and management of alveolitis and interstitial lung disease in scleroderma. Curr Opin Rheumatol 15(6):748–755

    PubMed  Article  Google Scholar 

  21. Proudman SM, Stevens WM, Sahhar J et al (2007) Pulmonary arteriel hypertension in systemic sclerosis: the need for early detection and treatment. Intern Med J 37(7):485–494

    CAS  PubMed  Article  Google Scholar 

  22. Khanna D, Nagaraja V, Gladue H et al (2013) Measuring response in the gastrointestinal tract in systemic sclerosis. Curr Opin Rheumatol 25(6):700–706

    PubMed  Article  Google Scholar 

  23. Kaye SA, Lim SG, Taylor M et al (1995) Small bowel bacterial overgrowth in systemic sclerosis: detection using direct and indirect methods and treatment outcome. Br J Rheumatol 34(3):265

    CAS  PubMed  Article  Google Scholar 

  24. Champion HC (2008) The Heart in Scleroderma. Rheum Dis Clin North Am 34(1):181–190

    PubMed Central  PubMed  Article  Google Scholar 

  25. Steen VD, Mayes MD, Merkel PA (2003) Assessment of kidney involvement. Clin Exp Rheumatol 21(3):S29–S31

    Google Scholar 

  26. Domsic RT, Rodriguez-Reyna T, Lucas M et al (2011) Skin thickness progression rate: a predictor of mortality and early internal organ involvement in diffuse scleroderma. Ann Rheum Dis 70(1):104–109

    PubMed  Article  Google Scholar 

  27. Tyndall AJ, Bannert B, Vonk M et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815

    PubMed  Article  Google Scholar 

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Affiliations

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

    O.D. Persa, P Moinzadeh & N. Hunzelmann

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  1. O.D. Persa
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  2. P Moinzadeh
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  3. N. Hunzelmann
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Correspondence to O.D. Persa.

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O.-D. Persa, P. Moinzadeh und N. Hunzelmann geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Persa, O., Moinzadeh, P. & Hunzelmann, N. Systemische Sklerodermie. Hautarzt 66, 599–603 (2015). https://doi.org/10.1007/s00105-015-3653-6

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  • DOI: https://doi.org/10.1007/s00105-015-3653-6

Schlüsselwörter

  • Systemische Sklerose
  • Hautfibrose
  • Lungenfibrose
  • Pulmonale Hypertonie
  • Kollagen

Keywords

  • Systemic scleroderma
  • Skin fibrosis
  • Lung fibrosis
  • Pulmonary hypertension
  • Collagen