Skip to main content
NCBI home page
Elsevier - PMC COVID-19 Collection logoLink to Elsevier - PMC COVID-19 Collection
. 2009 Jan 6;43(2):92–100. [Article in Spanish] doi: 10.1157/13098420

Epidemiología de la sarcoidosis

Epidemiology of Sarcoidosis

Estrella Fernández Fabrellas 1,⁎
PMCID: PMC7130812  PMID: 17288898

Abstract

La sarcoidosis es una enfermedad multisistémica que afecta frecuentemente al pulmón. Su incidencia y prevalencia han sido ampliamente estudiadas, pero la falta de estandarización del diagnóstico, los diferentes métodos de detección de casos y la escasa sensibilidad y especificidad de las pruebas diagnósticas explican los datos discordantes. El pronóstico es generalmente favorable. Gran parte de las personas afectadas no manifestarán nunca síntomas y muchas tienen remisión espontánea. El curso es crónico en el 10-30% de los casos, con un deterioro permanente de la función pulmonar. La enfermedad es el resultado de la acción de un agente externo que desencadena la respuesta inmunitaria característica en individuos genéticamente susceptibles. Se han implicado factores ambientales, ocupacionales y genéticos, pero las investigaciones están todavía en los inicios. Estudios de casos y controles, así como los avances en biología molecular, ayudarán a definir los factores de susceptibilidad genética y a entender los distintos fenotipos de la sarcoidosis.

Palabras clave: Sarcoidosis, Epidemiología, Etiología

Bibliografía

  • 1.Satement on sarcoidosis Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (WASOG) adopted by the ATS Board Directors and by the ERS Executive Committee. Am J Respir Crit Care Med. 1999;160:736–755. doi: 10.1164/ajrccm.160.2.ats4-99. [DOI] [PubMed] [Google Scholar]
  • 2.Rybicki B.A., Major M., Popovich J., Maliarik M.J., Iannuzzi M.C. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol. 1997;145:234–241. doi: 10.1093/oxfordjournals.aje.a009096. [DOI] [PubMed] [Google Scholar]
  • 3.Rossman M.D., Thompson B.W., Frederik M., Cizman B., Magira E., Monos D. Association of HLA class II alleles with sarcoidosis: results by race and sex of the ACCESS study. Am J Respir Crit Care. Med. 2001;163:A959. [Google Scholar]
  • 4.Du Bois R.M., Goh N., McGrath D., Cullinan P. Is there a role for microorganisms in the pathogenesis of sarcoidosis? J Intern Med. 2003;253:4–17. doi: 10.1046/j.1365-2796.2003.01073.x. [DOI] [PubMed] [Google Scholar]
  • 5.Yamamoto M., Sharma O.P., Hosoda Y. Special report: the 1991 descriptive definition of sarcoidosis. Sarcoidosis. 1992;9(Suppl 1):33–34. [Google Scholar]
  • 6.Newman L.S. Aetiologies of sarcoidosis. Eur Respir Mon. 2005;32:23–48. [Google Scholar]
  • 7.Henke C.E., Henke G., Elveback R., Beard C.M., Ballard D.J., Kurland L.T. The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival. Am J Epidemiol. 1986;123:840–845. doi: 10.1093/oxfordjournals.aje.a114313. [DOI] [PubMed] [Google Scholar]
  • 8.Alcoba Leza M., Pérez-Simón M.R., Guerra Laso J.M., Carro Fernández J.A., Alonso Fernández M.A., Blanco Iglesias B. Sarcoidosis en un área sanitaria de León. Epidemiología y características clínicas. An Med Interna. 2003;20:617–620. [PubMed] [Google Scholar]
  • 9.Mana J., Badrinas F., Morera J., Fite E., Manresa F., Fernández-Nogues F. Sarcoidosis in Spain. Sarcoidosis. 1992;9:118–122. [PubMed] [Google Scholar]
  • 10.Xaubet A., Ancochea J., Morell F., Rodríguez-Arias J.M., Villena V., Blanquer R. Report on the incidence of interstitial lung diseases 35. ases in Spain. Sarcoidosis Vasc Diffuse Lung Dis. 2004;21:64–70. [PubMed] [Google Scholar]
  • 11.Hosoda Y., Yamaguchi M., Hiraga Y. Global epidemiology of sarcoidosis. In: Sharma O.M.P., editor. Clinics in chest medicine: sarcoidosis. W.B. Saunders Company; Philadelphia: 1997. pp. 681–694. [DOI] [PubMed] [Google Scholar]
  • 12.ACCESS Research Group Design of A Case Control Etiologic Study of Sarcoidosis (ACCESS)J Clin Epidemiol. 1999;52(12):1173–1186. doi: 10.1016/s0895-4356(99)00142-0. [DOI] [PubMed] [Google Scholar]
  • 13.Terstein A.S., Judson M.A., Baughman R.P., Rossman M.D., Yeager H., Moller D.R. The spectrum of biopsy sites for the diagnosis of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2005;22:139–146. [PubMed] [Google Scholar]
  • 14.Freemer M., King T.E., The ACCESS Study Characterization of sarcoidosis in the United States. Am J Respir Crit Care Med. 2001;164:1754–1755. doi: 10.1164/ajrccm.164.10.2109111b. [DOI] [PubMed] [Google Scholar]
  • 15.Yeager H., Rossman M.D., Baughman R.P., Teirstein A.S., Judson M.A., Rabin D.L. Pulmonary and psychosocial findings at enrollment in ACCESS study. Sarcoidosis Vasc Diffuse Lung Dis. 2005;22:147–153. [PubMed] [Google Scholar]
  • 16.Judson M.A., Baughman R.P., Thompson B.W., Teirstein A.S., Terrin M.L., Rossman M.D. Two years prognosis of sarcoidosis: the ACCESS experience. Sarcoidosis Vasc Diffuse Dis. 2003;20:204–211. [PubMed] [Google Scholar]
  • 17.Baughman R., Teirstein A., Judson M., Rossman M.D., Yeager H., Bresnitz E.A. Clinical characteristics of patients in A Case Control Study of Sarcoidosis. Am J Respir Crit Care Med. 2001;164:1885–1889. doi: 10.1164/ajrccm.164.10.2104046. [DOI] [PubMed] [Google Scholar]
  • 18.Keller A.Z. Anatomic sites, age attributes and rates of sarcoidosis in U.S. veterans. Am Rev Respir Dis. 1973;107:615–620. doi: 10.1164/arrd.1973.107.4.615. [DOI] [PubMed] [Google Scholar]
  • 19.Rybicki B.A., Maliarik M.J., Major M., Popovich J., Iannuzzi M.C. Epidemiology, demographics and genetics of sarcoidosis. Semin. Respir Infect. 1998;13:166–173. [PubMed] [Google Scholar]
  • 20.Chapelon-Abric C. Epidemiology of sarcoidosis and its genetic and environmental risk factors. Rev Med Intern. 2004;25:494–500. doi: 10.1016/j.revmed.2004.01.011. [DOI] [PubMed] [Google Scholar]
  • 21.Byg K.E., Milman N., Hansen S. Sarcoidosis in Denmark 1980-1994. A registry-based incidence study compromising 5536 patients. Sarcoidosis Vasc Diffuse Lung Dis. 2003;20:46–52. [PubMed] [Google Scholar]
  • 22.Hoffmann A.L., Milman N., Byg K.E. Childhood sarcoidosis in Denmark 1979-1994: incidence, clinical features and laboratory results at presentation in 48 children. Acta Paediatr. 2004;93:30–36. [PubMed] [Google Scholar]
  • 23.Rybicki B.A., Iannuzzi M.C., Frederik M.M., Thompson B.W., Rossman M.D., Bresnitz E.A. Familial aggregation of sarcoidosis. A Case-Control Etiologic Study of Sarcoidosis (ACCESS) Am J Respir Crit Care Med. 2001;164:2085–2091. doi: 10.1164/ajrccm.164.11.2106001. [DOI] [PubMed] [Google Scholar]
  • 24.Spagnolo P., Renzoni E.A., Wells A.U., Sato H., Grutters J.C., Sestini P. C-C chemokine receptor 2 and sarcoidosis: association with Löfgren's syndrome. Am J Respir Crit Care Med. 2003;168:1162–1166. doi: 10.1164/rccm.200303-456OC. [DOI] [PubMed] [Google Scholar]
  • 25.Grunewald J., Wahlstrom J., Berlin M., Wigzell H., Eklund A., Olerup O. Lung restricted T cell receptor AV2S3+ CD4+ T cell expansions in sarcoidosis patients with shared HLA-DR beta chain conformation. Thorax. 2002;57:348–352. doi: 10.1136/thorax.57.4.348. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 26.Badrinas F., Morera J., Fité E., Plasencia A. Seasonal clustering of sarcoidosis. Lancet. 1989;2:455–456. doi: 10.1016/s0140-6736(89)90638-7. [DOI] [PubMed] [Google Scholar]
  • 27.Panayeas S., Theodorakopoulos P., Bouras A., Constantopoulos S. Seasonal occurrence of sarcoidosis in Greece. Lancet. 1991;338:510–511. doi: 10.1016/0140-6736(91)90581-9. [DOI] [PubMed] [Google Scholar]
  • 28.Wilsher M.L. Seasonal clustering of sarcoidosis presenting with erythema nodosum. Eur Respir J. 1998;12:1197–1199. doi: 10.1183/09031936.98.12051197. [DOI] [PubMed] [Google Scholar]
  • 29.Milton D.R., Barnard J., Frederik M. A case control etiologic study of sarcoidosis. Environmental and occupational risk factors. Am J Respir Crit Care Med. 2004;170:1324–1330. doi: 10.1164/rccm.200402-249OC. [DOI] [PubMed] [Google Scholar]
  • 30.Pérez Arellano J.L., Ramos Guevara R.M., López Ochoa F.J., Alcázar Montero M.C., De León Gómez B, Flores M.T. Sarcoidosis. Estudio retrospectivo de 30 casos en Salamanca y comparación con otras series nacionales. Rev Clin Esp. 1991;189:159–162. [PubMed] [Google Scholar]
  • 31.Hills S.E., Parkes S.A., Baker S.B.C. Epidemiology of sarcoidosis in the Isle of Man: evidence for space-time clustering. Thorax. 1987;42:427–430. doi: 10.1136/thx.42.6.427. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 32.Chappell A.G., Cheung W.Y., Hutchings H.A. Sarcoidosis: a long-term follow up study. Sarcoidosis Vasc Diffuse Lung Dis. 2000;17:167–173. [PubMed] [Google Scholar]
  • 33.Thomeer M.J., Vansteenkiste J., Verbeken E.K., Demedts M. Interstitial lung diseases: characteristics at diagnosis and mortality risk assessment. Respir Med. 2004;98:567–573. doi: 10.1016/j.rmed.2003.10.015. [DOI] [PubMed] [Google Scholar]
  • 34.Sulica R., Teirstein A.S., Kakarla S., Nemani N., Behnegar A., Padilla M.L. Distinctive clinical, radiographic and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest. 2005;128:1483–1489. doi: 10.1378/chest.128.3.1483. [DOI] [PubMed] [Google Scholar]
  • 35.Askling J., Grunewald J., Eklund A., Hillerdal G., Ekbom A. Increased risk for cancer following sarcoidosis. Am J Respir Crit Care Med. 1999;160:1668–1672. doi: 10.1164/ajrccm.160.5.9904045. [DOI] [PubMed] [Google Scholar]
  • 36.Romer F.K., Hommelgaard P., Schou G. Sarcoidosis and cancer re-visited: a long-term follow-up study of 555 Danish sarcoidosis patients. Eur Respir J. 1998;12:906–912. doi: 10.1183/09031936.98.12040906. [DOI] [PubMed] [Google Scholar]
  • 37.Foley P.J., Lympany P.A., Puscinska E., Zielinski J., Welsh K.I., Du Bois R.M. Analysis of MHC encoded antigen-processing genes TAP1 and TAP2 polymorphisms in sarcoidosis. Am J Respir Crit Care Med. 1999;160:1009–1014. doi: 10.1164/ajrccm.160.3.9810032. [DOI] [PubMed] [Google Scholar]
  • 38.Iannuzzi M.C., Maliarik M.J., Poisson L.M., Rybicki B.A. Sarcoidosis susceptibility and resistance HLA-DQB1 alleles in African Americans. Am J Respir Crit Care Med. 2003;167:1225–1231. doi: 10.1164/rccm.200209-1097OC. [DOI] [PubMed] [Google Scholar]
  • 39.Schurmann M., Reichel P., Muller-Myhsok B., Schlaak M., Muller-Quernheim J., Schwinger E. Results from a genome-wide search for predisposing genes in sarcoidosis. Am J Respir Crit Care Med. 2001;164:840–846. doi: 10.1164/ajrccm.164.5.2007056. [DOI] [PubMed] [Google Scholar]
  • 40.Rybicki B.A., Maliarik J., Poisson L.M., Sheffer R., Chen K.M., Major M. The major histocompatibility complex gene region and sarcoidosis susceptibility in African Americans. Am J Respir Crit Care Med. 2003;167:444–449. doi: 10.1164/rccm.2112060. [DOI] [PubMed] [Google Scholar]
  • 41.Rabin D.L., Thompson B., Brown K.M., Judson M.A., Huang X., Lac-kland D.T. Sarcoidosis: social predictors of severity at presentation. Eur Respir J. 2004;24:601–608. doi: 10.1183/09031936.04.00070503. [DOI] [PubMed] [Google Scholar]
  • 42.Olden K., White S.L. Health-related disparities: influence of environmental factors. Med Clin North Am. 2005;89:721–738. doi: 10.1016/j.mcna.2005.02.001. [DOI] [PubMed] [Google Scholar]
  • 43.Semenzato G., Bortoli M., Brunetta E., Agostini C. Immunology and pathophysiology of sarcoidosis. Eur Respir Mon. 2005;32:49–63. [Google Scholar]
  • 44.Gorham E.D., Garland C.F., Garland F.C., Kaiser K., Travis W.D., Centeno J.A. Trends and occupational associations in incidence of hospitalized pulmonary sarcoidosis and other lung diseases in Navy personnel. A 27-year historical prospective study, 1975-2001. Chest. 2004;126:1431–1438. doi: 10.1378/chest.126.5.1431. [DOI] [PubMed] [Google Scholar]
  • 45.Barnard J., Cecile R., Newman L., Canner M., Martyny J., McCam-mon C. Job and industry classifications associated with sarcoidosis in a case-control etiologic study of sarcoidosis (ACCESS) J Occup Environ Med. 2005;47:226–234. doi: 10.1097/01.jom.0000155711.88781.91. [DOI] [PubMed] [Google Scholar]
  • 46.Kajdasz D.K., Judson M.A., Mohr L.C., Lackland D.T. Geographic variation in sarcoidosis in South Carolina: its relation to socioeconomic status and health care indicators. Am J Epidemiol. 1999;150:271–278. doi: 10.1093/oxfordjournals.aje.a009998. [DOI] [PubMed] [Google Scholar]
  • 47.Kucera G.P., Rybicki B.A., Kirkey K.L., Coon S.W., Major M.L., Maliarik M.J. Occupational risk factors for sarcoidosis in African-American siblings. Chest. 2003;123:1527–1535. doi: 10.1378/chest.123.5.1527. [DOI] [PubMed] [Google Scholar]
  • 48.Brown S.T., Brett I, Almenoff P.L., Lesser M., Terrin M., Teirstein A.S. Recovery of cell wall-deficient organisms from blood does not distinguish between patients with sarcoidosis and control subjects. Chest. 2003;123:413–417. doi: 10.1378/chest.123.2.413. [DOI] [PubMed] [Google Scholar]
  • 49.Saboor S.A., Johnson N.M., McFadden J. Detection of mycobacte-rial DNA in sarcoidosis and tuberculosis with polymerase chain reaction. Lancet. 1992;339:1012–1015. doi: 10.1016/0140-6736(92)90535-b. [DOI] [PubMed] [Google Scholar]
  • 50.Bocart D., Lecossier D., De Lassence A., Valeyre D., Battesti J.P., Hance A.J. A search for mycobacterial DNA in granulomatous tissues from patients with sarcoidosis using the polymerase chain reaction. Am Rev Respir Dis. 1992;145:1142–1148. doi: 10.1164/ajrccm/145.5.1142. [DOI] [PubMed] [Google Scholar]
  • 51.Fite E., Fernández-Figueras M.T., Prats R., Vaquero M., Morera J. High prevalence of Mycobacterium tuberculosis DNA in biopsies from sarcoidosis patients from Catalonia, Spain. Respiration. 2006;73:20–26. doi: 10.1159/000087688. [DOI] [PubMed] [Google Scholar]
  • 52.Song Z., Marzilli L., Greenlee B.M., Chen E.S., Silver R.F., Askin F.B. Mycobacterial catalase-peroxidase is a tissue antigen and target of the adaptive immune response in systemic sarcoidosis. J Exp Med. 2005;201:755–767. doi: 10.1084/jem.20040429. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 53.Padilla M.L., Schilero G.J., Teirstein A.S. Donor-acquired sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19:18–24. [PubMed] [Google Scholar]
  • 54.Müller C.B.J. Sarcoidosis recurrence following lung transplantation. Transplantation. 1996;16:1117–1119. doi: 10.1097/00007890-199604150-00024. [DOI] [PubMed] [Google Scholar]
  • 55.Ikonomopoulos J.A., Gorgoulis V.G., Kastrinakis N.G., Galanos A.A., Karameris A., Kittas C. Experimental inoculation of laboratory animals with samples collected from sarcoidal patients and molecular diagnosis evaluation of the results. In Vivo. 2000;14:761–765. [PubMed] [Google Scholar]
  • 56.Vidal S., De la Horra C., Martín J., Montes-Cano M.A., Rodríguez E., Respaldiza N. Pneumocystis jiroveci colonisation in patients with interstitial lung disease. Clin Microbiol Infect. 2006;12:231–235. doi: 10.1111/j.1469-0691.2005.01337.x. [DOI] [PubMed] [Google Scholar]
  • 57.Gazouli M., Ikonomopoulos J., Koundourakis A., Bartos M., Pavlik I., Overduin P. Characterization of Mycobacterium tuberculosis complex isolates from Greek patients with sarcoidosis by spoligotyping. J Clin Microbol. 2005;43:4858–4861. doi: 10.1128/JCM.43.9.4858-4861.2005. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 58.McGrath D.S., Daniil Z., Foley P., Du Bois J.L., Lympany P.A., Cullinan P. Epidemiology of familial sarcoidosis in the UK. Thorax. 2000;55:751–754. doi: 10.1136/thorax.55.9.751. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 59.Harrington D., Major M., Popovich J., Maliarik M., Iannuzzi M. Familial sarcoidosis: analysis of 61 families. Am Rev Respir Dis. 1993;147:A91. [Google Scholar]
  • 60.Thomeer M., Demedts M., Wuyts W. Epidemiology of sarcoidosis. Eur Respir Mon. 2005;32:13–22. [Google Scholar]
  • 61.Rybicki B.A., Kirkey K.L., Major M., Maliarik M.J., Popovich J., Chase G.A. Familial risk ratio of sarcoidosis in African-American sibs and parents. Am J Epidemiol. 2001;153:188–193. doi: 10.1093/aje/153.2.188. [DOI] [PubMed] [Google Scholar]
  • 62.Pérez Arellano J.L. Lavado broncoalveolar en la enfermedad pulmonar intersticial. Últimas noticias. Arch Bronconeumol. 2002;38:57–59. doi: 10.1016/s0300-2896(02)75151-x. [DOI] [PubMed] [Google Scholar]
  • 63.Çelik G., Sen E., Ülger A.F., Özdemir-Kumbasar Ö., Alper D., Elhan A.H. Antígenos leucocitarios humanos A y B en pacientes turcos con sarcoidosis. Arch Bronconeumol. 2004;40:449–452. doi: 10.1016/s1579-2129(06)60354-6. [DOI] [PubMed] [Google Scholar]
  • 64.Du Bois R.M., Beirne P.A., Anevlavis S.E. Genetics of sarcoidosis. Eur Respir Mon. 2005;32:64–81. [Google Scholar]
  • 65.Rossman M.D., Thompson B.T., Frederik M., Maliarik M., Iannuzzi M.C., Rybicki B.A. HLA-DRB1*1101: a significant risk factor for sarcoidosis in Blacks and Whites. Am J Hum Genet. 2003;73:720–735. doi: 10.1086/378097. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 66.Rybicki B.A., Walewski J.L., Maliarik M.J., Kian H., Iannuzzi M.C., The ACCESS Research Group The BTNL2 gene and sarcoidosis susceptibility in African Americans and Whites. Am J Hum Genet. 2005;77:491–499. doi: 10.1086/444435. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 67.Rybicki B.A., Hirst K., Iyengar S.K., Barnard J.G., Judson M.A., Rose C.S. A sarcoidosis genetic linkage consortium: the Sarcoidosis Genetic Analysis (SAGA) study. Sarcoidosis Vasc Diffuse Lung Dis. 2005;22:83–86. [PubMed] [Google Scholar]
  • 68.Iannuzzi M.C., Iyengar S.K., Gray-McGuire C., Elston R.C., Baughman R.P., Donohue J.F. Genome-wide search for sarcoidosis susceptibility genes in African Americans. Genes Immun. 2005;6:509–518. doi: 10.1038/sj.gene.6364235. [DOI] [PubMed] [Google Scholar]

Articles from Archivos De Bronconeumologia are provided here courtesy of Elsevier

RESOURCES