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>Revistas >Revista Cubana de Reumatología >Año 2014, No. S1


Reyes LGA, Guibert TZM, López CG, Hernández DC, Macías ÁR, Martínez LJP, Álvarez VRR
Esclerosis sistémica. Evaluación clínico-epidemiológica de una serie de casos en dos centros de referencia en Cuba
Rev Cub de Reu 2014; 16 (S1)

Idioma: Español
Referencias bibliográficas: 80
Paginas: 346-355
Archivo PDF: 579.75 Kb.


Texto completo




RESUMEN

Introducción: La esclerosis sistémica es una enfermedad grave y potencialmente mortal causante de marcada fibrosis de la piel y múltiples órganos viscerales vitales. Esta entidad presenta diferentes grados de severidad y progresión, con mortalidad incrementada.
Objetivos: Determinar las características socio demográficas, clínico-epidemiológicas, y manejo terapéutico de una serie de 34 pacientes cubanos atendidos en centros de referencia de reumatología.
Método: Se recogieron variables clínicas, órganos y sistemas afectados, test de laboratorio e inmunología, endoscopía gastroduodenal, ecografía cardíaca, cateterismo, tomografía axial computarizada de alta resolución, pruebas funcionales respiratorias, y otros estudios, terapéutica seguida, complicaciones y desenlace.
Resultados: presentaron formas difusas de la enfermedad 20 (58.8 %), formas limitadas 11 (32.3 %), 2 síndromes de CREST (5.88 %) y una sobreposición. El tiempo de evolución promedio fue de 7.4 años DS/5.3. La participación músculo-esquelética (ME) en 16 (47 %), retracciones articulares, úlceras digitales y resorción de falanges en 4 (12.5 %).
La participación gastrointestinal (56.2 %) y 16 /19 (85 %) desarrollaron disfagia que fue severa en 5(31.2 %). Insuficiencia respiratoria 9 (26.4 %), en tanto la afectación cardiaca 8 (23 %), y 6 (17.6 %) presentaron afectación renal. El 68 % de esta serie presentó fenómeno de Raynaud. La terapéutica con antifibróticos del tipo D’Pencilamina 19 (55.8 %), procinéticos 16 (47.4 %) y gastroprotectores 8 (23.5 %). esteroides 9 (26.4 %), inhibidores de la enzima convertidora de la angiotensina (IECA) 7 (20.5 %), sildelnafil 3 (8.82 %) entre otras drogas.
Conclusiones: La afectación de órganos vitales resulta similar en población cubana al compararse con las series publicadas, con mayor repercusión gastrointestinal y respiratoria. Evolución general tórpida.


Palabras clave: esclerosis sistémica, esclerodermia, epidemiología.


REFERENCIAS

  1. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 2003; 29(2):239-54.

  2. Loannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger Jr TA, Lucas M, Michet CJ, Moutsopoulos HM. Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med. 2005;118(1):2-10.

  3. Rodríguez Hernández JF, Iglesias Sánchez JL, Dueñas Miranda MC, Díaz Domínguez MA, Diez Cabrea M, Esclerosis Sistémica: Aspectos epidemiológicos clínicos en la provincia de Pinar del Rio. Rev Cub Reumatol. 2000;2(1):19-26.

  4. Al Aranji G, White D, Solanki K. Scleroderma renal crisis following silicone breast implant ruptura: a case report and review of the literatura. Clin Exp Rheumatol. 2014;32(2):262-6.

  5. Sekanecz E, Szamosi S, Horvath A, Nemeth A,Juhász B. Szántó J, Szekanecz Z. Malignancies associated with systemic sclerosis. Autoimmun Rev. 2012;(12):852-5.

  6. Bernarsky S, Ramsey-Goldman R, Labrecque J, Joseph L, Boivinc JF, Petrid M, et al. Cancer risk in systemic lupus: an update international multicentre cohort study. J Autoimmun. 2013;42:130-5. doi:10.1016/j.jaut.2012.12.009

  7. Khanna D, Melikterminas E. Gastrointestinal involvement in systemic sclerosis. Indian Journal of Rheumatology. 2008;3(1):3-20.

  8. Steen VD,Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43(11):2437-44.

  9. Van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci Cerinic M et al. Classification criteria for systemic sclerosis: an American College of rheumatology / European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747-55.

  10. Shu E, Kanoh H, Seishima M. Scleroderma renal crisis following pericardial effusion in a Japanese female. J Dermatol. 2014;41(9):824-6.

  11. Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L. Scleroderma renal crisis. J Rheumatol. 2014;41(6):1040-8.

  12. Lambova S. Cardiac manifestations in systemic sclerosis. World J Cardiol. 2014;26;6(9):993-1005.

  13. Reyes Llerena Gil A, Guibert Toledano M, Solier L, López Mantecón A M, Delgado Ferreira I, Almendariz Díaz M de la C, López Aguilera I R, Macías R. Manifestaciones gastrointestinales severas en pacientes con Esclerosis Sistémica. Reporte de casos y revisión de la literatura. Rev Cubana de Reumatol. 2007;9(10):15-7.

  14. Masi A T. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis & Rheumatism. 1980;23(5):581-90.

  15. Hoeper MM, Bogaard HJ, Condiffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB. Definitions and diagnosis of pulmonary hypertension. J AM Coll Cardiol. 2013;62 Suppl. 25: S42-50.

  16. Zafer Mengeloglu, Tekin Tas, Esra Kocoglu, Gülali Aktas, and Seyda Karabörk. Determination of Anti-nuclear Antibody Pattern Distribution and Clinical Relationship Pak J Med Sci. 2014;30(2):380-3.

  17. Afsar I, Sener AG, Vural A, Hizli N, Türker M. Evaluation of immunoblotting test results in patients with positive antinuclear antibody. Türk Mikrobiyol Cem Derg. 2007;37(1):39-42.

  18. Fritzler MJ, Wiik A, Fritzler ML, Barr SG. The use and abuse of commercial kits used to detect autoantibodies. Arthritis Res Ther. 2003;5(4):192-201.

  19. Muro Y. Antinuclear antibodies. Autoimmunity. 2005;38(1):3-9.

  20. LeRoy EC,Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger Jr TA., Wollheim F. Scleroderma (Systemic Sclerosis): Classifications subsets and pathogenesis (Ed). J Rheumatol. 1988;15(2):202-5.

  21. Denton CP, Black CM, Korn JH, De Crombrugghe, B.Meeting report : Systemic Sclerosis. Currents pathogenetic concepts and future prospects for targeted therapy. Lancet. 1996;347:1453-58.

  22. Le Roy EC. Sentinel signs and simptoms of systemic sclerosis. Current Op Rheumatol. 1989;1(4):499-504.

  23. Maurer B, Graf N, Michel BA, Müller-Ladner U, Czirják L, Denton CP, et al. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database. Ann Rheum Dis. 2014 Jun 30. doi:10.1136/annrheumdis-2014-205226.

  24. Chung L.Therapeutic options for digital ulcers in patients with systemic sclerosis. J Dutsch Dermatol Ges. 2007;5(6):460-5.

  25. Neto F, Nieves M, de Queiroz V ‘’Doencas difusas do tecido conjuntivo’’. Esclerose Sistémica. En Reumatología. Lisboa: Ed. LIDEL: 2002.

  26. Zinmerman AF, Pizzichini MM. Update on ethiopatogenesis of systemic sclerosis. Rev Bras Rheumatol. 2013;53(6):516-24.

  27. Chung L, Fiorentino I. Digital ulcers in patients with systemic sclerosis. Arthritis Rheum. 2007;57(7):1280-6.

  28. Blocka KLN, Basset LW, Furst de. The arthropathy of advanced progressive systemic sclerosis: a radiographic survey. Arthritis Rheum. 1981;24(7):874-84.

  29. James R. Seibold. Textbook of rheumatology. Systemic Sclerosis.7ma ed. Madrid: Ed. Elsevier; 2006.

  30. Velayos E E, Masi A T, Stevens M B, Shulman L E. Medsger T A.jr: The 'CREST' syndrome. Comparison with systemic sclerosis. The Pediatric Rheumatology European Society/ American College of rheumatology / European League against rheumatism provisional classification criteria for juvenile systemic sclerosis. Archives of internal medicine. 1979;139(11):1240-4.

  31. Avouac J, Walker UA, Hachulla E, Riemekasten G, Cuomo G, Carreira PE, et al. Joint and tendon involvement predict

  32. disease progression in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis. 2014 Aug 27. doi:10.1136/annrheumdis-2014-205295

  33. Jaovisidha K, Csuka ME, Almagro UA, Soergel KH. Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature. Semin Arthritis Rheum. 2005;34 (4):689-702.

  34. Marie I. Gastrointestinal involvement in systemic sclerosis. Press Med. 2006;35(12):1952-65.

  35. Ntoumazios S K, Voulgari P V, Potsis K, Koutis E, Tsifetaki N, Assimakopoulos D A. Esophageal involvement in scleroderma: gastroesophageal reflux, the common problem. Semin Arthritis Rheum. 2006;36(3):173-81.

  36. Kirby DF, Chatterjee S. Evaluation and management of gastrointestinal manifestations in scleroderma. Curr Opin Rheumatol. 2014;26(6):621-9.

  37. Savarino E, Furnari M, de Bortoli N, Martinucci I, Bodini G, Ghio M, Savarino V. Gastrointestinal involvement in systemic sclerosis. Presse Med. 2006(35)12:1952-65.

  38. Meijs J, Pors D, Vliet Vlieland TP, Huizinga TW, Schouffoer AA. Translation, cross-cultural adaptation, and validation of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument (SCTC GIT) 2.0 into Dutch. Clin Exp Rheumatol. 2014 Jun 30. PMID:24984228.

  39. Kowal-Belecka O, Landewé R, Avouac J, Chwiesko,S, Miniati I, Czirjak L. et al EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR).Ann Rheum Dis. 2009;68(5):620-28.

  40. Winstone TA, Assayag D, Wilcox PG, Dunne JV, Haque CJ, Leipsic J, Collard HR, Ryerson CJ. Predictors of mortality and progression in scleroderma-associated interstitial lung disease: a systematic review. Chest. 2014;146(2):422-36 doi:10.1378/chest.13-2626.

  41. Szamosi S, Szekancecz Z, Szucs G. Gastrointestinal manifestations in Hungarian scleroderma patients. Rheumatol Int. 2006;26(12):1120-24.

  42. Szamosi S, Marodi L, Czirjak L. Ellenes Z, Szules G. Juvenile systemic sclerosis: a follow-up study of eight patients. An N Y Acad Sci. 2005;1051(1):229-34.

  43. Hawley DP, Baildam EM, Amin TS, Cruikshank MK, Davidson JE, Dixon J, et al. Access to care for children and young people diagnosed with localized scleroderma or juvenile SSc in the UK. Rheumatology (Oxford). 2012;51(7):1235-9.

  44. Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, Lehman TJ, Olivera SK. Et al. Juvenil Scleroderma. Working Group of the pediatric rheumatology European Society. University of Padua, Italy: Arthritis and Rheumatism 2006 ;54(12):3971-8.

  45. Jara L J, Barrera A. Afección Renal en la Esclerosis Sistémica. Reumatología Clínica. 2006; 2 Suppl 3:20-3.

  46. Wells AU, Denton CP. Interstitial lung disease in connective tissue disease-mechanisms and management. New York: Ed. Nature Publishing Group; 2014.

  47. Y, Matsushita T, Hasegawa M, Ueda-Hayakawa I, Sato S, Takehara K, Fujimoto M. High incidence of pulmonary arterial hypertension in systemic sclerosis patients with anti-centriole autoantibodies. Mod Rheumatol. 2013 Oct 25. PubMed: PMID:24252018

  48. Moazedi-Fuerst FC, Kielhauser SM, Brickmann SM, Hermann J, Lutfi A, Meilinger M, Brezinschek HP, Graninger WB. Rituximab for systemic sclerosis: arrest of pulmonary disease progression in five cases Results of a lower dosage and shorter interval regimen. Scand J Rheumatol. 2014;43(3): 257-8.

  49. Galie-Galie N, Torbicki A, Barst R. Dartevelle P, Haworth, S., Higenbottam, T, Simonneau, Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The task Force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology. Eur Heart J. 2004;25(24):2243-78.

  50. Distler O, F Behrens, D Hoscher, I. Faeldvary, A. Zink, P Nash, et al. Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis. J Rheumatol. 2006;33(9):1726-8.

  51. Montisci R, Vacca A, Garau PP, Colonna P, Ruscazio M, Passiu G, Mathieu A. Detection of early impairment of coronary flow reserve in patients with systemic sclerosis. Ann Rheum Dis. 2003;62(9):890-3.

  52. Ishida R, Murata Y, Sawada Y. Thallium-201 myocardial SPECT in patients with collagen disease. Nucl Med Commun. 2000;21(8):729-34.

  53. Steen V D, Medsger T A, Osial T A, Zieger G L, Shapiro A P, Rodnan G P et al. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med. 1984;76:779-86.

  54. Steen VD. Kidney involvement in systemic sclerosis. Presse Med. 2014; 43(10):305-14. Doi:10.1016/j.lpm.2014.02.031

  55. Shu E, Kanoh H, Seishima M. Scleroderma renal crisis following pericardial effusion in a Japanese female. J Dermatol. 2014;41(9):824-6.

  56. Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L. Scleroderma renal crisis. J Rheumatol. 2014;41(6):1040-8.

  57. Alias A, Rodríquez EJ, Baterman HE, Sterrett AG, Valeriano-Marcet J. Rheumatology and oncology: an update review of rheumatic manifestations of malignancy and antineoplastic therapy. Bull N Y Hosp Jt, Dis. 2012;70(2):109-14.

  58. Quintana Duque M A, Iglesias Gamarra A. Restrepo Suárez JF. Tratamiento de la Esclerosis Sistémica: evidencia actual. Actualización. Rev Colomb de Reumatología. 2005;12(4): 350-61.

  59. Zandman-Goddard G, Twezer-Zaks N, Shoenfeld Y. New therapeutic strategies for systemic sclerosis--a critical analysis of the literature. Clin Dev Immunol. 2005;12(3):165-73.

  60. Cipriani P, Ruscitti P, Carubbi F, Liakouli V, Giacomelli R. Methotrexate: an old new drug in autoimmune disease. Expert Rev Clin Immunol. 2014;22:1-12.

  61. Xuan J, Shen L, Zhang C, Ambrus JL. Biologics in Systemic Sclerosis. Curr Pharm Biotechnol. 2014;15(6):549-57.

  62. Jimenez SA, Piera-Velazquez S. Potential role of human-specific genes, human-specific microRNAs and human-specific non-coding regulatory RNAs in the pathogenesis of Systemic Sclerosis and Sjögren's Syndrome. Autoimmunity reviews. 2013;12(11):1046-51.

  63. Ferri C, Sebastiani M, Lo Monaco A, Iudici M, Giuggioli D, Furini F, Manfredi A, Cuomo G, Spinella A, Colaci M, Govoni M, Valentini G. Systemic sclerosis evolution of disease pathomorphosis and survival. Our experience on Italian patients' population and review of the literature. Autoimmun Rev. 2014;13(10):1026-34.

  64. Ishak R, Abbas O. Penicillamine revisited: historic overview and review of the clinical uses and cutaneous adverse effects. Am J Clin Dermatol. 2013;14(3):223-33.

  65. Nagaraja V, Denton CP, Khanna D. Old medications and new targeted therapies in systemic sclerosis. Rheumatology (Oxford). 2014 Jul:285. Doi:10.1093/rheumatology/keu285

  66. Shalev T, Haviv Y, Segal E, Ehrenfeld M, Pauzner R, Levy Y, Langevitz P, Shoenfeld Y. Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases. Clin Exp Rheumatol. 2006; 24(4):380-6.

  67. Nipkour M, Baron M. Mortality in systemic : lesson learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26(2):131-37.

  68. Clements J P, Furst D E, Wong W K, Mayes, M., White, B., Wigley, F., ... & Seibold, J. RHigh doses versus low doses D’Penicillamine in early diffuse systemic sclerosis. Arthritis Rheum. 1999;42(6):1194-203.

  69. Lam G K, Hummers L K, Woods A, Wigley F M. Efficacy and safety of Etanercept in the treatment of systemic sclerosis associated joint diseases. J Rheumatol. 2007;34(7):1636-37.

  70. Giorgetti F, Minnucci M L, Santori P, Ercoli L, Rossi M, Del Papa M, Morico G, Squadroni P, Olivieri A, Centurión R. Autologous peripheral stem cell transplantation in a patient with diffuse systemic sclerosis: our experience. Rheumatism. 2004;56;(1):51-6.

  71. Plastiras SC, Tzivras M, Vlachoyiannopoulos PG: Severe gastrointestinal involvement in systemic sclerosis Clin Rheumatol. 2007;26(6):1025-26.

  72. García de la Peña-Lefebre P, Rodríguez Rubio S, Valero Expósito M, Carmona L, Gami Gutiérrez J, Díaz-Miguel C. et al. Long term experience of bosentan for treating ulcers and healed ulcers in sistemic sclerosis patients. Rheumatology (Oxford). 2008;47(4):464-6.

  73. Barst R J, Langleben D, Badesh D, Frost A, Lawrence E C, Shapiro S, Naeije R, Galie N. Treatment of pulmonary arterial hypertension with the sele-endothelin-A receptor antagonist sitaxsentan. J Am Cardiol. 2006;16;47(10):2049-56.

  74. Driscoll J A, Chakinola M M. Medical Therapy for pulmonary hypertension . Expert Opin Pharmacother. 2008;9(1):65-81.

  75. McLaughlin VV, Gaine SP, Howard LS, Leuchte HH, Mathier MA, Mehta S, Sitbon O. Treatment goals of

  76. pulmonary hypertension. J Am Coll Cardiol. 2013; 62 Suppl 25:S73-81.

  77. Garg N, Sharma M K, Sinha N. Role of oral sildenafil in severe pulmonary hypertension : efficacy and dose response relationship. Int J Cardiol. 2007;120(3):306-13.

  78. Becvar R, Store J, Jansa P. et al. New Trends in diagnosis and treatment of systemic sclerosis. Vnitr Lek. 2006;52(7-8):712-7.

  79. Ross N, Poulalhon N, Farge D, Madelaine I, Mauviel A, Verrecchia F. In vitro evidence for a direct antifibrotic role of the immunosuppressive drug mycophenolate mofetil. J Pharmacol Exp Ther. 2007;321;(2):583-9.

  80. Nihtyanova S I, Brough G M, Black C M, Denton C P. Mycophenolate mofetil in diffuse cutaneous systemic sclerosis: retrospective análisis. Rheumatology (Oxford). 2007;46;(3):442-5.

  81. Taylor Jiménez B, Guerra Castro M, Hernández Pía N, Lima Cuellar E, Rodríguez Soria N, Álvarez Escobar M C, Oxigenación Hiperbárica: una opción terapéutica en Esclerosis Sistémica Progresiva. Rev Cubana de Reumatol. 2005;7(7-8):34-40.

  82. Herink AK, Köthe L, Girndt M, Keysser G. Treatment options of acral ulcers in MCTD. Med Klin (Munich). 2010;105(11):837-40.



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