#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

New Trends in Diagnosis and Treatment of Systemic Sclerosis


Authors: R. Bečvář;  J. Štork;  P. Jansa
Authors‘ workplace: Revmatologický ústav, Praha, ředitel prof. MUDr. Karel Pavelka, DrSc.
Published in: Vnitř Lék 2006; 52(7-8): 712-717
Category: 130th Internal Medicine Day - Rheumatology in clinical practice

Overview

Systemic sclerosis (SSc) is a generalised connective tissue disease of unknown origin, which clinically shows by skin thickening and sclerosis of different extent (scleroderma) and by typical involvement of visceral organs. At the same time fibrotic and sclerotic changes occur in the blood vesel walls. SSc usually involves females at young and middle age. Myalgias, arthralgias and arthritis are nonspecific, tendon friction rubs in fingers are more typical for this diagnosis. Gastrointestinal involvement starts early in the oropharyngeal part, esophagus and proceeds into the distal parts. Fibrotic changes lead to slow transit dysmotility and pseudoobstruction and/or dilation of the bowels. The main symptoms are dysphagia, pyrosis, malabsorption and constipation. SSc produces two major patterns of abnormality within the lungs - a fibrosing alveolitis or a primary pulmonary vascular disease. More frequently an insterstitial process develops which can be followed by pulmonary arterial hypertension. Cardiac involvement can also have different forms. Myocardial fibrosis usually appears at first in the conduction system by arrhythmias and various conduction blocks while pericarditis is mostly asymptomatic. Renal manifestation of SSc is observed in 8 - 10 % patients. The most severe form - scleroderma renal crisis is characterised by the new onset of accelerated hypertension and rapidly progressive oliguric renal failure. No therapies have been proven to modify the course of SSc. Some of the drugs can affect only the skin changes. Majority of the currently applied agents have only a symptomatic effect.

Key words:
systemic sclerosis - Raynaud‘s phenomenon - fibrosis - sclerosis - alveolitis - hypertension


Sources

1. Medsger TA. Comment on scleroderma criteria cooperative study. In: Black CM, Myers AR (eds). Systemic sclerosis (Scleroderma). New York: Glower Med 1985: 235-249.

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

3. Furst DE, Clements PJ. Pathogenesis of scleroderma. In: Clements PJ, Furst DE. Systemic sclerosis. Baltimore: Williams&Wilkins 1996: 275-286.

4. Roberts AB, Sporn MB, Assoian RK et al. Transforming growth factor type beta: rapid induction of fibrosis and angiogenesis in vivo and stimulation of collagen formation in vitro. Proc Natl Acad Sci 1986; 83: 4167-4171.

5. Wigley FM, Wise RA, Mikdashi J et al. The postocclusive hyperemic response in patients with systemic sclerosis. Arthritis Rheum 1990; 33: 1620-1625.

6. Maricq HR. Widefield capillary microscopy. Technique and rating scale for abnormalities seen in scleroderma and related disorders. Arthritis Rheum 1981; 24: 1159-1165.

7. Rodnan GP, Lipinski E, Luksick J. Skin thickness and collagen content in progressive systemic sclerosis and localized scleroderma. Arthritis Rheum 1979; 22: 130-140.

8. Jiranek GC, Bredfelt JE. Organ involvement: Gut and hepatic manifestations. In: Clements PJ, Furst DE Systemic sclerosis. Baltimore: Williams&Wilkins 1996: 453-482.

9. Zatloukal P, Bečvář R, Štork J et al. Plicní postižení u progresivní systémové sklerózy. Čes Revmatol 1999; 7: 16-21.

10. Jansa P, Aschermann M, Ambrož D et al. Plicní arteriální hypertenze u systémových onemocnění pojiva. Rheumatologia 2006; 20: 13-18.

11. Shulman LE, Kurban AK, Harvey ABL. Tendon friction rubs in progressive sclerosis (scleroderma). Arthritis Rheum 1961; 4: 438-445.

12. Follansbee WP, Miller TR, Curtis EI et al. A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma). J Rheumatol 1990; 17: 656-662.

13. Steen VD. Organ involvement: renal. In: Clements PJ, Furst DE. Systemic sclerosis. Baltimore: Williams&Wilkins 1996: 425-440.

14. Lukáč J. Systémová skleróza. Púchov: Lubor Šeba 1998: 135-136.

15. Degiannis D, Seibold JR, Czarnecki M. Soluble and cellular markers of immune activation in patients with systemic sclerosis. Clin Immunol Immunopathol 1990; 56: 259-266.

16. Greaves M, Malia RG, Milford WA et al. Elevated von Willebrand factor antigen in systemic sclerosis: relationship to visceral disease. Br J Rheumatol 1988; 27: 281-285.

17. Kadono T, Kikuchi K, Sato S et al. Elevated plasma endothelin levels in systemic sclerosis. Arch Dermatol Res 1995; 287: 439-442.

18. Tan EM, Rodnan GP. Profile of antinuclear antibodies in progressive systemic sclerosis (PSS). Arthritis Rheum 1975; 18: 430-442.

19. Weiner ES, Hildebrandt S, Senecal JL. Prognostic significance of anticentromere antibodies and anti-topoisomerase I antibodies in Raynaud's disease. Arthritis Rheum 1991; 34: 68-75.

20. Brennan P, Silman A, Black C et al. Reliability of skin involvement measures in scleroderma. Br J Rheumatol 1992; 31: 457-460.

21. Clements PJ, Bečvář R, Drosos AA et al. Assessment of gastrointestinal involvement. Clin Exp Rheumatol 2003; 21 (Suppl 29): S15-S18.

22. Reynolds HY, Newbal L. Analysis of protein and respiratory cells obtained from human lungs by bronchial lavage. J Lab Clin Med 1974; 84: 559-730.

23. Subcommittee for Scleroderma Criteria of the American Rheumatism Association. Diagnostic and Therapeutic Criteria Committee: Preliminary Criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23: 581-590.

24. Munves EF, Schur PH. Antibodies to Sm and RNP. Prognosticators of disease involvement. Arthritis Rheum 1983; 26: 848-859.

25. Kahaleh MB, LeRoy EC. Interleukin-2 in scleroderma: correlation of serum levels with extent of skin involvement and disease duration. Ann Intern Med 1989; 110: 446-450.

26. Bečvář R, Štork J, Pešáková V et al. Clinical correlations of potential activity markers in systemic sclerosis. Ann NY Acad Sci 2005; 1051: 404-412.

27. Štork J, Bečvář R. Hodnocení závažnosti a aktivity systémové sklerodermie. Čes Slov Derm 2004; 70: 213-217.

28. Lukáč J. Systémová skleróza. Púchov: Lubor Šeba 1998: 256-316.

29. Bečvář R, Kozák T, Forejtová Š et al. Autologní transplantace kmenových buněk u nemocné systémovou sklerodermií s refrakterním intersticiálním plicním procesem. Čes Revmatol 2005; 13: 73-76.

Labels
Diabetology Endocrinology Internal medicine
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#