Sindrom CREST

  • Joko Anggoro Fakultas Kedokteran Universitas Mataram

Abstract

Sindrom CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, dan telangiectasia) syndrome adalah anggota kelompok heterogen skleroderma. Frekuensi di Amerika Serikat, insidensi sklerosis sistemik berkisar antara 2,7-19,3 kasus baru per juta orang dewasa per tahun. Peningkatan yang tampak dalam insidensi dan prevalensi selama 50 tahun terakhir kemungkinan besar karena klasifikasi yang lebih baik, diagnosis yang lebih dini, dan survival yang lebih baik. Beberapa penelitian antibodi serum menunjukkan bahwa sindrom CREST mungkin mencakup sekitar 22-25% dari semua kasus sklerosis sistemik; tetapi, penelitian epidemiologis yang mencari secara spesifik pada sindrom CREST masih belum ada. Naskah ini mengupas lebih dalam tentang sindrom CREST mulai aspek klasifikasi penyakit ini dalam skleroderma, patogenesis, gambaran klinis, prognosis, dan terapi yang terbaru. Katakunci Sindrom CREST, patogenesis, klinis, terapi

References

1. Horner KL, Raugi GJ, Wemple MA. CREST syndrome. eMedicine Dermatology. 2008;Available from: http://www.emedicine.medscape.com.

2. Steen VD, Medsger TA. Changes in Causes of Death in Systemic Sclerosis, 1972–2002. Annals of the Rheumatic Diseases. 2007;66(7):940–944.

3. Simeon CP, Armadans L, Fonollosa V, Solans R, Selva A, Villar M, et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis.
Rheumatology. 2003;42(1):71–75.

4. Guern VL, Mahr A, Mouthon L, Jeaneret D, Carzon M. Prevalence of systemic sclerosis in a French multi-ethnic county. Rheumatology. 2004;43(9):1129–1137.

5. Allcock RJ, Forrest I, Corris PA, Crook PR, Griffiths ID. A study of the prevalence of systemic sclerosis in northeast England. Rheumatology.
2004;43:596–602.

6. Hunzelmann N, Genth E, Krieg T, Lehmacher W, Melchers I, Meurer M, et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology. 2008;47(8):1185–1192.

7. Tager RE, Tikly M. Clinical and laboratory manifestation of systemic sclerosis (scleroderma) in Black South Africans. Rheumatology. 1999;38:397–400.

8. Wollheim FA. Clasification of systemic sclerosis. Vision and reality. Rheumatology. 2005;44:1212–1216.

9. Khan AI, Susa J, Ansari MQ. Systemic Sclerosis (Scleroderma). Labmedicine. 2005;36(11):723–726.

10. Denton CP, Black CM. Scleroderma (Systemic Sclerosis): Fitzpatrick’s Dermatology in General Medicine. McGraw-Hill. 2008;7:1553–1562.

11. Loubiere LS, Lambert NC, Madeleine MM, Porter AJ, Mullarkey ME, Pang JM. HLA allelic variants encoding DR11 in diffuse and limited systemic sclerosis in Caucasian women. Rheumatology. 2005;44:318–322.

12. Bovenzi M, Barbone F, Pisa FE, Betta A, Romeo L, Tonello A, et al. A case-control study of occupational exposures and systemic sclerosis. International Archives of Occupational and Environmental Heal th. 2004;77(1):10–16.

13. Tangri N, Young BM. Soft-tissue infection and underlying calcinosis of CREST syndrome. Canadian Medical Association Journal. 2006;175(9):1060–
1061.

14. Herrick AL. Review: Pathogenesis of Raynaud’s phenomenon. Rheumatology. 2005;44:587–596.

15. Sunderkotter C, Riemekasten G. Pathophysiology and clinical consequences of Raynaud’s phenome non related to systemic sclerosis. Rheumatology. 2006;45:iii33–iii35.

16. Sheehan NJ. Dysphagia and other manifestations of oesophageal involvement in the musculoskeletal diseases. Rheumatology. 2008;47(6):746–752.

17. Pitrez EH, Bredemeier M, Xavier RM, Capobianco KG, Restelli VG, Vieira MV, et al. Oesophageal dysmotility in systemic sclerosis: comparison of HRCT and scintigraphy. The British Journal of Radiology. 2006;79:719–724.

18. Jones J, Brenner C, Chinn R, Bunjer CB. Pictorial review: Radiological associations with dermatolo gical disease. The British Journal of Radiology.
2005;78:662–671.

19. Hoeper MM. Pulmonary hypertension in collagen vascular disease. European Respiratory Journal. 2002;19(3):571–576.

20. Roman MJ, Salmon JE. Cardiovascular Manifestations of Rheumatologic Diseases. Circulation. 2007;116(20):2346–2355.

21. Marie I, Levesque H, Tranvouez JL, Francois A, Riachi G, Cailleux Nea. Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome.
Rheumatology. 2001;40:102–106.

22. Avouac J, Sordet C, Depinay C, Ardizonne M, Vacher-Lavenu MC, Sibilia J, et al. Systemic sclerosis associated Sjogren’s syndrome and relationship
to the limited cutaneous subtype: Results of a prospective study of sicca syndrome in 133 consecutive patients. Arthritis & Rheumatism. 2006;54(7):2243–2249.

23. Nakamura T, Higashi S, Tomoda K, Tsukano M, Sugi K. Primary biliary cirrhosis (PBC)–CREST overlap syndrome with coexistence of Sjogren’s ¨
syndrome and thyroid dysfunction. Clinical Rheumatology. 2007;26(4):596–600.

24. Szucs G, Szekanecz Z, Zilahi E, Kapitany A, Barath S, Szamosi S. Systemic sclerosis–rheumatoid arthritis overlap syndrome: a unique combination of features suggests a distinct genetic, serological and clinical entity. Rheumatology. 2007;46:989–993.

25. Hill CL, Nguyen AM, Roder D, Roberts-Thomson P. Risk of cancer in patients with scleroderma: a population based cohort study. Annals of the
Rheumatic Diseases. 2003;62(8):728–731.

26. Hesselstrand R, Scheja A, Shen GQ, Wiik A, Akesson A. The association of antinuclear antibodies with organ involvement and survival in systemic
sclerosis. Rheumatology. 2003;42(4):534–540.

27. Hu PQ, Fertig N, Medsger TA, Wright TM. Molecular Recognition Patterns of Serum Anti-DNA Topoisomerase I Antibody in Systemic Sclerosis. The
Journal of Immunology. 2004;173(4):2834–2841.

28. Hugle T, van Laar JL. Review: Stem cell transplantation for rheumatic autoimmune diseases. Arthritis Research & Therapy. 2008;10:1–10.

29. Cukierman T, Elinav E, Korem M, Chajek-Shaul T. CONCISE REPORT: Low dose warfarin treatment for calcinosis in patients with systemic sclerosis (abstract). Annals of the Rheumatic Diseases. 2004;63(10):1341–1343.

30. Riemekasten G, Sunderkotter C. Vasoactive therapies in systemic sclerosis. Rheumatology. 2006;45(3):iii49–iii51.

31. Bogoch ER, Gross DK. Surgery of the hand in patients with systemic sclerosis: outcomes and considerations (abstract). Journal of Rheumatology.
2005;32:642–648.

32. Melone CP, McLoughlin JC, Beldner S. Surgical management of the hand in scleroderma. Current Opinion in Rheumatology. 1999;11(6):514–520.
Published
2017-11-14
Section
Literature Review