Enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. Revisión narrativa de la literatura
DOI:
https://doi.org/10.17533/udea.iatreia.v30n3a03Palabras clave:
Azatioprina, Ciclofosfamida, enfermedad pulmonar intersticial, esclerosis sistémica, Micofenolato Mofetil, Rituximab, tomografía, trasplante de médula óseaResumen
Introducción: la esclerosis sistémica es una enfermedad autoinmune crónica, caracterizada por la tríada de vasculopatía de pequeños vasos, activación del sistema inmune y aumento de los depósitos de matriz extracelular tanto en la piel como en los órganos internos. En los últimos años, el compromiso pulmonar ha cobrado gran importancia, pasando a ser la primera causa de muerte, dada la disminución de la mortalidad por crisis renales con el advenimiento de los inhibidores de la enzima convertidora de angiotensina. La afección pulmonar puede ocurrir como hipertensión o enfermedad intersticial difusa; esta última es más común en pacientes con la variedad generalizada, en los primeros tres años de la enfermedad. Tiene mal pronóstico si no se inicia el tratamiento, cuya meta es detener el deterioro de la función pulmonar. Entre los tratamientos disponibles, la ciclofosfamida cuenta con la mejor evidencia, pero se están estudiando, con resultados preliminares satisfactorios, otros tratamientos como micofenolato mofetil, rituximab, trasplante autólogo de células hematopoyéticas y trasplante de pulmón.
Objetivo: describir, de acuerdo con lo reportado en la literatura, la epidemiología, la fisiopatología, los métodos diagnósticos y el tratamiento de la enfermedad pulmonar intersticial en pacientes con esclerosis sistémica.
Métodos: revisión estructurada, no sistemática de la literatura, enfocada en los aspectos de interés, mencionados y que incluyó 52 artículos.
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(1.) Tyndall A, Tyrrell Kennedy A, Allanore Y, Khanna D. Clinical and experimental scleroderma 2014. Clin Exp Rheumatol. 2014 Nov-Dec;32(6 Suppl 86):S-3.
(2.) Silver KC, Silver RM. Management of Systemic-Sclerosis-Associated Interstitial Lung Disease. Rheum Dis Clin North Am. 2015 Aug;41(3):439-57. DOI 10.1016/j.rdc.2015.04.006.
(3.) Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007 Jul;66(7):940-4.
(4.) Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000 Nov;43(11):2437-44.
(5.) Scussel-Lonzetti L, Joyal F, Raynauld JP, Roussin A, Rich E, Goulet JR, et al. Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine (Bal- timore). 2002 Mar;81(2):154-67.
(6.) Walker UA, Tyndall A, Czirják L, Denton C, Farge-Bancel D, Kowal-Bielecka O, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis. 2007 Jun;66(6):754-63.
(7.) Bussone G, Mouthon L. Interstitial lung disease in systemic sclerosis. Autoimmun Rev. 2011 Mar;10(5):248- 55. DOI 10.1016/j.autrev.2010.09.012.
(8.) Herzog EL, Mathur A, Tager AM, Feghali-Bostwick C, Schneider F, Varga J. Review: interstitial lung disease associated with systemic sclerosis and idiopathic pulmonary fibrosis: how similar and distinct? Arthritis Rheumatol. 2014 Aug;66(8):1967-78. DOI 10.1002/art.38702.
(9.) Wuttge DM, Andreasson A, Tufvesson E, Johansson ÅC, Scheja A, Hellmark T, et al. CD81 and CD48 show different expression on blood eosinophils in systemic sclerosis: new markers for disease and pulmonary inflammation? Scand J Rheumatol. 2016;45(2):107-13. DOI 10.3109/03009742.2015.1054877.
(10.) Solomon JJ, Olson AL, Fischer A, Bull T, Brown KK, Raghu G. Scleroderma lung disease. Eur Respir Rev. 2013 Mar;22(127):6-19. DOI 10.1183/09059180.00005512.
(11.) Kundu S, Mitra S, Ganguly J, Mukherjee S, Ray S, Mitra R. Spectrum of diffuse parenchymal lung diseases with special reference to idiopathic pulmonary fibrosis and connective tissue disease: An eastern India experience. Lung India. 2014 Oct;31(4):354-60. DOI 10.4103/0970-2113.142115.
(12.) Murray LA, Rubinowitz A, Herzog EL. Interstitial lung disease: is interstitial lung disease the same as scleroderma lung disease? Curr Opin Rheumatol. 2012 Nov;24(6):656-62.
(13.) Hant FN, Herpel LB, Silver RM. Pulmonary manifestations of scleroderma and mixed connective tissue disease. Clin Chest Med. 2010 Sep;31(3):433-49. DOI 10.1016/j.ccm.2010.05.004.
(14.) Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Pölzleitner D, Burghuber OC, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology. 1990 Sep;176(3):755-9.
(15.) D’Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969 Mar;46(3):428-40.
(16.) Behr J, Furst DE. Pulmonary function tests. Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v65-7. DOI 10.1093/rheumatology/ken313.
(17.) Legnani D, Rizzi M, Sarzi-Puttini P, Cristiano A, La Spina T, Frassanito F, et al. Diffusing Pulmonary Capacity Measured During Effort: A Possible Early Marker of Pulmonary Involvement In Systemic Sclerosis. Isr Med Assoc J. 2015 Dec;17(12):739-43.
(18.) Garin MC, Highland KB, Silver RM, Strange C. Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J Rheumatol. 2009 Feb;36(2):330-6. DOI 10.3899/jrheum.080447.
(19.) Rizzi M, Sarzi-Puttini P, Airoldi A, Antivalle M, Battellino M, Atzeni F. Performance capacity evaluated using the 6-minute walk test: 5-year results in patients with diffuse systemic sclerosis and initial interstitial lung disease. Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S142-7.
(20.) Vandecasteele E, De Pauw M, De Keyser F, Decuman S, Deschepper E, Piette Y, et al. Six-minute walk test in systemic sclerosis: A systematic review and meta-analysis. Int J Cardiol. 2016 Jun;212:265-73. DOI 10.1016/j.ijcard.2016.03.084.
(21.) Diot E, Boissinot E, Asquier E, Guilmot JL, Lemarié E, Valat C, et al. Relationship between abnormalities on high-resolution CT and pulmonary function in systemic sclerosis. Chest. 1998 Dec;114(6):1623-9.
(22.) Remy-Jardin M, Remy J, Wallaert B, Bataille D, Hatron PY. Pulmonary involvement in progressive systemic sclerosis: sequential evaluation with CT, pulmonary function tests, and bronchoalveolar lavage. Radiology. 1993 Aug;188(2):499-506.
(23.) Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008 Jun;177(11):1248-54. DOI 10.1164/rccm.200706-877OC.
(24.) Salaffi F, Carotti M, Di Donato E, Di Carlo M, Cecca- relli L, Giuseppetti G. Computer-Aided Tomographic Analysis of Interstitial Lung Disease (ILD) in Patients with Systemic Sclerosis (SSc). Correlation with Pul- monary Physiologic Tests and Patient-Centred Mea- sures of Perceived Dyspnea and Functional Disability. PLoS One. 2016 Mar;11(3):e0149240. DOI 10.1371/journal.pone.0149240.
(25.) Moazedi-Fuerst FC, Kielhauser S, Brickmann K, Tripolt N, Meilinger M, Lufti A, et al. Sonographic assessment of interstitial lung disease in patients with rheumatoid arthritis, systemic sclerosis and systemic lupus erythematosus. Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S87-91.
(26.) Moazedi-Fuerst FC, Zechner PM, Tripolt NJ, Kielhauser SM, Brickmann K, Scheidl S, et al. Pulmonary echography in systemic sclerosis. Clin Rheumatol. 2012 Nov;31(11):1621-5. DOI 10.1007/s10067-012-2055-8.
(27.) Silver RM, Bogatkevich G, Tourkina E, Nietert PJ, Hoffman S. Racial differences between blacks and whites with systemic sclerosis. Curr Opin Rheumatol. 2012 Nov;24(6):642-8.
(28.) Christmann RB, Wells AU, Capelozzi VL, Silver RM. Gastroesophageal reflux incites interstitial lung disease in systemic sclerosis: clinical, radiologic, histopathologic, and treatment evidence. Semin Arthritis Rheum. 2010 Dec;40(3):241-9. DOI 10.1016/j.semar-thrit.2010.03.002.
(29.) Hsu E, Shi H, Jordan RM, Lyons-Weiler J, Pilewski JM, Feghali-Bostwick CA. Lung tissues in patients with systemic sclerosis have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension. Arthritis Rheum. 2011 Mar;63(3):783-94. DOI 10.1002/art.30159.
(30.) van Bon L, Affandi AJ, Broen J, Christmann RB, Marijnissen RJ, Stawski L, et al. Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis. N Engl J Med. 2014 Jan;370(5):433-43. DOI 10.1056/NE-JMoa1114576.
(31.) Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al. Cyclophosphamide versus 31. Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006 Jun;354(25):2655-66.
(32.) Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, et al. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med. 2007 Nov;176(10):1026-34.
(33.) Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, et al. A multicenter, prospective, rando- mized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. 2006 Dec;54(12):3962-70.
(34.) Nihtyanova SI, Brough GM, Black CM, Denton CP. Mycophenolate mofetil in diffuse cutaneous systemic sclerosis--a retrospective analysis. Rheumatology (Oxford). 2007 Mar;46(3):442-5.
(35.) Swigris JJ, Olson AL, Fischer A, Lynch DA, Cosgrove GP, Frankel SK, et al. Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest. 2006 Jul;130(1):30-6.
(36.) anopoulos ST, Bournia VK, Trakada G, Giavri I, Kostopoulos C, Sfikakis PP. Mycophenolate versus cyclophosphamide for progressive interstitial lung disease associated with systemic sclerosis: a 2-year case control study. Lung. 2013 Oct;191(5):483-9. DOI 10.1007/s00408-013-9499-8.
(37.) Zamora AC, Wolters PJ, Collard HR, Connolly MK, Elicker BM, Webb WR, et al. Use of mycophenolate mofetil to treat scleroderma-associated interstitial lung disease. Respir Med. 2008 Jan;102(1):150-5.
(38.) Derk CT, Grace E, Shenin M, Naik M, Schulz S, Xiong W. A prospective open-label study of mycophenolate mofetil for the treatment of diffuse systemic sclerosis. Rheumatology (Oxford). 2009 Dec;48(12):1595-9. DOI 10.1093/rheumatology/kep295.
(39.) Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, Selva-O’Callaghan A, Solans-Laqué R, Vilardell-Tarrés M. Effect of mycophenolate sodium in scleroderma-related interstitial lung disease. Clin Rheumatol. 2011 Nov;30(11):1393-8. DOI 10.1007/s10067-011-1823-1.
(40.) Henes JC, Horger M, Amberger C, Schmalzing M, Fierlbeck G, Kanz L, et al. Enteric-coated mycophenolate sodium for progressive systemic sclerosis—a prospective open-label study with CT histography for monitoring of pulmonary fibrosis. Clin Rheumatol. 2013 May;32(5):673-8. DOI 10.1007/s10067-012-2155-5.
(41.) Owen C, Ngian GS, Elford K, Moore O, Stevens W, Nikpour M, et al. Mycophenolate mofetil is an effective and safe option for the management of systemic sclerosis-associated interstitial lung disease: results from the Australian Scleroderma Cohort Study. Clin Exp Rheumatol. 2016 Sep-Oct;34 Suppl 100(5):170-176.
(42.) Launay D, Buchdahl AL, Berezné A, Hatron PY, Hachulla E, Mouthon L. Mycophenolate mofetil following cyclophosphamide in worsening systemic sclerosis-associated interstitial lung disease. J Scleroderma Relat Disord. 2016 Apr;1(2):234-40. DOI 10.5301/jsrd.5000205.
(43.) ClinicalTrials.gov [Internet]. Bethesda: NLM; 2009 [cited 2015 Oct 20]. Comparison of Therapeutic Regimens for Scleroderma Interstitial Lung Disease (The Scleroderma Lung Study II) (SLSII). Available from: https://clinicaltrials.gov/ct2/show/NCT00883129
(44.) American College of Rheumatology [Internet]. Atlanta: Wiley; 2016 [cited 2016 Apr 22]. Abstracts tagged “mycophenolate mofetil”. Available from: http://acrabstracts.org/tag/mycophenolate-mofetil/
(45.) Nadashkevich O, Davis P, Fritzler M, Kovalenko W. A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol. 2006 Mar;25(2):205-12.
(46.) Walker KM, Pope J; participating members of the Scleroderma Clinical Trials Consortium (SCTC; Canadian Scleroderma Research Group (CSRG). Treatment of systemic sclerosis complications: what to use when first-line treatment fails—a consensus of systemic sclerosis experts. Semin Ar- thritis Rheum. 2012 Aug;42(1):42-55. DOI 10.1016/j.emarthrit.2012.01.003.
(47.) Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Kazantzi A, Sirinian C, et al. Experience with ri- tuximab in scleroderma: results from a 1-year, proof- of-principle study. Rheumatology (Oxford). 2010 Feb;49(2):271-80. DOI 10.1093/rheumatology/kep093.
(48.) Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomi- sed phase 2 trial. Lancet. 2011 Aug;378(9790):498-506. DOI 10.1016/S0140-6736(11)60982-3.
(49.) van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophospha- mide in diffuse cutaneous systemic sclerosis: a ran- domized clinical trial. JAMA. 2014 Jun;311(24):2490-8. DOI 10.1001/jama.2014.6368.
(50.) ClinicalTrials.gov [Internet]. Bethesda: NLM; 2009 [cited 2015 Oct 20]. Scleroderma Treatment With Autologous Transplant (STAT) Study (STAT). Available from: https://clinicaltrials.gov/ct2/show/NCT01413100
(51.) ClinicalTrials.gov [Internet]. Bethesda: NLM; 2009 [cited 2015 Apr 22]. Scleroderma: Cyclophosphamide or Transplantation (SCOT). Available from: https://clinicaltrials.gov/ct2/show/NCT00114530
(52.) Khan IY, Singer LG, de Perrot M, Granton JT, Keshavjee S, Chau C, et al. Survival after lung transplantation in systemic sclerosis. A systematic review. Respir Med. 2013 Dec;107(12):2081-7. DOI 10.1016/j.rmed.2013.09.015.
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