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Abstract
Sarcoidosis is a systemic granulomatous disease of unknown etiology that mainly develops between 25 and 40 years of age and primarily affects the lungs and the lymphatic system. Generally accepted pathogenic hypothesis is that certain environmental agents could promote sarcoidosis in genetically susceptible hosts. The characteristic lesion of sarcoidosis is a noncaseating granuloma that consist of epithelioid cells and T lymphocytes. Diverse clinical courses are observed according to the epidemiologic factors, involved organs, and disease severity. Spontaneous remissions occur in approximately two thirds of patients, but the course is chronic or deteriorative in 10 to 30%. Mortality, ranging between 1 and 5%, is mostly related to progressive respiratory insufficiency, or central nervous system or myocardial involvement. The mainstay of treatment for sarcoidosis is systemic corticosteroid administration. However, the indication, optimal dose, and duration of corticosteroid therapy continue to be controversial.
Figures and Tables
Figure 1
Chest radiographic staging of sarcoidosis.
(A) Stage I. The posteroanterior film shows bilateral hilar lymphadenopathy with clear lung fields.
(B) Stage II. The posteroanterior film shows bilateral hilar lymphadenopathy associated with parenchymal infiltration.
(C) StageIII.The posteroanterior film shows parenchymal infiltration without hilar lymphadenopathy, but no obvious fibrotic changes.
(D) StageIV. The posteroanterior film shows predominantly upper lobe fibrotic abnormalities with hilar retraction, coarse fibrotic masses (mostly on the left), and bibasal compensatory emphysema (secondary to the loss of upper lobe volume).
References
1. Hutchinson J. Hutchinson J, editor. Case of livid papillary psoriasis. Illustrations of clinical surgery. 1877. vol.1. London: J&A Churchill;42–43.
2. Boeck C. Multiple benign sarkoid of the skin. Journal of Cutaneous and Genitourinary Disease. 1899. 17:543–550.
3. Hillerdal G, Nou E, Osterman K, Schmekel B. Sarcoidosis: epidemiology and prognosis. A 15-year European study. Am Rev Respir Dis. 1984. 130:29–32.
4. Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med. 1997. 336:1224.
5. Rybicki BA, Major M, Popovich J Jr, Maliarik MJ, Iannuzzi MC. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol. 1997. 145:234–241.
6. Henke CE, Henke G, Elveback LR, Beard CM, Ballard DJ, Kurland LT. The epidemiology of sarcoidosis in Rochester, Minnesota: a population-based study of incidence and survival. Am J Epidemiol. 1986. 123:840–845.
7. James DG, Hosoda Y. James DG, editor. Epidemiology. Sarcoidosis and other granulomatous disorders. 1994. New York: Marcel Dekker;729–743.
8. Teirstein AS, Lesser M. Fanburg BL, editor. Worldwide distribution and epidemiology of sarcoidosis. Sarcoidosis and other granulomatous diseases of the lung. 1983. New York: Marcel Dekker;101–134.
9. Hosoda Y, Yamaguchi M, Hiraga Y. Global epidemiology of sarcoidosis. What story do prevalence and incidence tell us? Clin Chest Med. 1997. 18:681–694.
10. Hosoda Y. Grassi C, Rizzato G, Pozzi E, editors. Epidemiology of sarcoidosis: State-of-the-art. Proceedings of the 11th World Congress on sarcoidosis and other granulomatous disorders. 1988. Amsterdam: Elsevier Science;279–290.
11. Bresnitz EA, Strom BL. Epidemiology of sarcoidosis. Epidemiol Rev. 1983. 5:124–156.
12. Scientific committee in korean academy of tuberculosis and respiratory disease. National survey of sarcoidosis in korea. Tuberc Respir Dis. 1992. 39:453–473.
13. Kim DS, Ahn JJ. Sarcoidosis in korea. Tuberc Respir Dis. 2000. 49:274–280.
14. Verleden GM, du Bois RM, Bouros D, Drent M, Millar A, Muller-Quernheim J, Semenzato G, Johnson S, Sourvino G, Olivier D, Pietinalho A, Xaubet A. Genetic predisposition and pathogenetic mechanisms of interstitial lung diseases of unknown origin. Eur Respir J Suppl. 2001. 32:17s–29s.
15. du Bois RM, Goh N, McGrath D, Cullinan P. Is there a role for microorganisms in the pathogenesis of sarcoidosis? J Intern Med. 2003. 253:4–17.
16. Conron M, Du Bois RM. Immunological mechanisms in sarcoidosis. Clin Exp Allergy. 2001. 31:543–554.
17. Boros DL. Experimental granulomatosis. Clin Dermatol. 1986. 4:10–21.
18. Hunninghake GW, Crystal RG. Pulmonary sarcoidosis: a disorder mediated by excess helper T-lymphocyte activity at sites of disease activity. N Engl J Med. 1981. 305:429–434.
19. Semenzato G, Pezzutto A, Chilosi M, Pizzolo G. Redistribution of T lymphocytes in the lymph nodes of patients with sarcoidosis. N Engl J Med. 1982. 306:48–49.
20. Agostini C, Trentin L, Zambello R, Bulian P, Siviero F, Masciarelli M, Festi G, Cipriani A, Semenzato G. CD8 alveolitis in sarcoidosis: incidence, phenotypic characteristics, and clinical features. Am J Med. 1993. 95:466–472.
21. Konishi K, Moller DR, Saltini C, Kirby M, Crystal RG. Spontaneous expression of the interleukin 2 receptor gene and presence of functional interleukin 2 receptors on T lymphocytes in the blood of individuals with active pulmonary sarcoidosis. J Clin Invest. 1988. 82:775–781.
22. Robinson BW, McLemore TL, Crystal RG. Gamma interferon is spontaneously released by alveolar macrophages and lung T lymphocytes in patients with pulmonary sarcoidosis. J Clin Invest. 1985. 75:1488–1495.
23. Baughman RP, Strohofer SA, Buchsbaum J, Lower EE. Release of tumor necrosis factor by alveolar macrophages of patients with sarcoidosis. J Lab Clin Med. 1990. 115:36–42.
24. Moller DR, Forman JD, Liu MC, Noble PW, Greenlee BM, Vyas P, Holden DA, Forrester JM, Lazarus A, Wysocka M, Trinchieri G, Karp C. Enhanced expression of IL-12 associated with Th1 cytokine profiles in active pulmonary sarcoidosis. J Immunol. 1996. 156:4952–4960.
25. Agostini C, Trentin L, Facco M, Sancetta R, Cerutti A, Tassinari C, Cimarosto L, Adami F, Cipriani A, Zambello R, Semenzato G. Role of IL-15, IL-2, and their receptors in the development of T cell alveolitis in pulmonary sarcoidosis. J Immunol. 1996. 157:910–918.
26. Kreipe H, Radzun HJ, Heidorn K, Barth J, Kiemle-Kallee J, Petermann W, Gerdes J, Parwaresch MR. Proliferation, macrophage colony-stimulating factor, and macrophage colonystimulating factor-receptor expression of alveolar macrophages in active sarcoidosis. Lab Invest. 1990. 62:697–703.
27. Rosen Y. Dail DH, Hammer SP, editors. Sarcoidosis. Pulmonary Pathology. 1994. 2nd ed. New York: Springer-Verlag;13–645.
28. Colby TV, Carrington CB. Thurlbeck WM, Churg AM, editors. Interstitial lung diseases. Pathology of the lung. 1995. New York: Thieme Medical Publishers;589–737.
29. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med. 1999. 160:736–755.
30. Bein ME, Putman CE, McLoud TC, Mink JH. A reevaluation of intrathoracic lymphadenopathy in sarcoidosis. AJR Am J Roentgenol. 1978. 131:409–415.
31. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol. 1985. 144:513–528.
32. Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H Jr, Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ, McLennan G, Moller DR, Newman LS, Rabin DL, Rose C, Rybicki B, Weinberger SE, Terrin ML, Knatterud GL, Cherniak R. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med. 2001. 164:1885–1889.
33. Neville E, Walker AN, James DG. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. Q J Med. 1983. 52:525–533.
34. Romer FK. Presentation of sarcoidosis and outcome of pulmonary changes. Dan Med Bull. 1982. 29:27–32.
35. Reich JM, Johnson RE. Course and prognosis of sarcoidosis in a nonreferral setting. Analysis of 86 patients observed for 10 years. Am J Med. 1985. 78:61–67.
36. Siltzbach LE. Sarcoidosis: clinical features and management. Med Clin North Am. 1967. 51:483–502.
37. Gottlieb JE, Israel HL, Steiner RM, Triolo J, Patrick H. Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy. Chest. 1997. 111:623–631.
38. Hunninghake GW, Gilbert S, Pueringer R, Dayton C, Floerchinger C, Helmers R, Merchant R, Wilson J, Galvin J, Schwartz D. Outcome of the treatment for sarcoidosis. Am J Respir Crit Care Med. 1994. 149:893–898.
39. Chappell AG, Cheung WY, Hutchings HA. Sarcoidosis: a long-term follow up study. Sarcoidosis Vasc Diffuse Lung Dis. 2000. 17:167–173.
40. Pietinalho A, Ohmichi M, Lofroos AB, Hiraga Y, Selroos O. The prognosis of pulmonary sarcoidosis in Finland and Hokkaido, Japan. A comparative five-year study of biopsyproven cases. Sarcoidosis Vasc Diffuse Lung Dis. 2000. 17:158–166.
41. Lynch JP 3rd, Sharma OP, Baughman RP. Extrapulmonary sarcoidosis. Semin Respir Infect. 1998. 13:229–254.
42. Yamaguchi M, Hosoda Y, Sasaki R, Aoki K. Epidemiological study on sarcoidosis in Japan. Recent trends in incidence and prevalence rates and changes in epidemiological features. Sarcoidosis. 1989. 6:138–146.
43. Iwai K, Sekiguti M, Hosoda Y, DeRemee RA, Tazelaar HD, Sharma OP, Maheshwari A, Noguchi TI. Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis. 1994. 11:26–31.
44. James DG, Epstein WL. James DG, editor. Cutaneous sarcoidosis. Sarcoidosis and other granulomatous disorders. 1994. New York: Marcel Dekker;311–322.
45. Gilman MJ, Wang KP. Transbronchial lung biopsy in sarcoidosis. An approach to determine the optimal number of biopsies. Am Rev Respir Dis. 1980. 122:721–724.
46. Costabel U. Sensitivity and specificity of BAL findings in sarcoidosis. Sarcoidosis. 1992. 9:211–214.
47. Scadding JG. Prognosis of intrathoracic sarcoidosis in England. A review of 136 cases after five years' observation. Br Med J. 1961. 2:1165–1172.
48. Siltzbach LE, James DG, Neville E, Turiaf J, Battesti JP, Sharma OP, Hosoda Y, Mikami R, Odaka M. Course and prognosis of sarcoidosis around the world. Am J Med. 1974. 57:847–852.
49. Lynch JP 3rd, Kazerooni EA, Gay SE. Pulmonary sarcoidosis. Clin Chest Med. 1997. 18:755–785.
50. Gibson GJ, Prescott RJ, Muers MF, Middleton WG, Mitchell DN, Connolly CK, Harrison BD. British Thoracic Society Sarcoidosis study: effects of long term corticosteroid treatment. Thorax. 1996. 51:238–247.
51. Johns CJ, Schonfeld SA, Scott PP, Zachary JB, MacGregor MI. Longitudinal study of chronic sarcoidosis with low-dose maintenance corticosteroid therapy. Outcome and complications. Ann N Y Acad Sci. 1986. 465:702–712.
52. Yamamoto M, Saito N, Tachibana T. Chretien J, Marsac J, Saltiel JC, editors. Effects of an 18 month corticosteroid therapy to stage I and stage II sarcoidosis patients (control trial). Sarcoidosis and other granulomatous disorders. 1980. Paris: Pergamon Press;470–474.
53. Gideon NM, Mannino DM. Sarcoidosis mortality in the United States 1979-1991: an analysis of multiple-cause mortality data. Am J Med. 1996. 100:423–427.
54. Perry A, Vuitch F. Causes of death in patients with sar-coidosis. A morphologic study of 38 autopsies with clinico-pathologic correlations. Arch Pathol Lab Med. 1995. 119:167–172.
55. Sharma OP. Pulmonary sarcoidosis and corticosteroids. Am Rev Respir Dis. 1993. 147:1598–1600.
56. Takada K, Ina Y, Noda M, Sato T, Yamamoto M, Morishita M. The clinical course and prognosis of patients with severe, moderate or mild sarcoidosis. J Clin Epidemiol. 1993. 46:359–366.
57. Viskum K, Vestbo J. Vital prognosis in intrathoracic sarcoidosis with special reference to pulmonary function and radiological stage. Eur Respir J. 1993. 6:349–353.
58. Vestbo J, Viskum K. Respiratory symptoms at presentation and long-term vital prognosis in patients with pulmonary sarcoidosis. Sarcoidosis. 1994. 11:123–125.
59. Huang CT, Heurich AE, Sutton AL, Lyons HA. Mortality in sarcoidosis. A changing pattern of the causes of death. Eur J Respir Dis. 1981. 62:231–238.
60. Baughman RP, Winget DB, Bowen EH, Lower EE. Predicting respiratory failure in sarcoidosis patients. Sarcoidosis Vasc Diffuse Lung Dis. 1997. 14:154–158.
61. Löfgren S. Erythema nodosum: studies on etiology and pathogenesis in 185 adult cases. Acta Med Scand. 1946. 124:1–197.
62. James DG, Neville E, Siltzbach LE. A worldwide review of sarcoidosis. Ann N Y Acad Sci. 1976. 278:321–334.
63. Glennas A, Kvien TK, Melby K, Refvem OK, Andrup O, Karstensen B, Thoen JE. Acute sarcoid arthritis: occurrence, seasonal onset, clinical features and outcome. Br J Rheumatol. 1995. 34:45–50.
64. Berlin M, Fogdell-Hahn A, Olerup O, Eklund A, Grunewald J. HLA-DR predicts the prognosis in Scandinavian patients with pulmonary sarcoidosis. Am J Respir Crit Care Med. 1997. 156:1601–1605.
65. Gran JT, Bohmer E. Acute sarcoid arthritis: a favourable outcome? A retrospective survey of 49 patients with review of the literature. Scand J Rheumatol. 1996. 25:70–73.
66. Rizzato G, Montemurro L. James DG, editor. The locomotor system. The locomotor system. 1994. New York: Marcel Dekker;349–373.
67. Ward K, O'onnor C, Odlum C, Fitzgerald MX. Prognostic value of bronchoalveolar lavage in sarcoidosis: the critical influence of disease presentation. Thorax. 1989. 44:6–12.
68. Lofgren S. The concept of erythema nodosum revised. Scand J Respir Dis. 1967. 48:348–353.
69. James D. Sarcoidosis of the skin. Semin Respir Med. 1992. 13:422–441.
70. Chapelon C, Ziza JM, Piette JC, Levy Y, Raguin G, Wechsler B, Bitker MO, Bletry O, Laplane D, Bousser MG, et al. Neurosarcoidosis: signs, course and treatment in 35 confirmed cases. Medicine (Baltimore). 1990. 69:261–276.
71. Rizzato G, Colombo P. Nephrolithiasis as a presenting feature of chronic sarcoidosis: a prospective study. Sarcoidosis Vasc Diffuse Lung Dis. 1996. 13:167–172.
72. Israel HL, Karlin P, Menduke H, DeLisser OG. Factors affecting outcome of sarcoidosis. Influence of race, extrathoracic involvement, and initial radiologic lung lesions. Ann N Y Acad Sci. 1986. 465:609–618.
73. Adams J. Hypercalcemia and hypercalcuria. Semin Respir Med. 1992. 13:402–410.
74. Rizzato G, Fraioli P, Montemurro L. Nephrolithiasis as a presenting feature of chronic sarcoidosis. Thorax. 1995. 50:555–559.
75. Johnson D, Yamakido M, O S. Musculoskeletal involvement in sarcoidosis. Semin Respir Med. 1992. 13:415–419.
76. Rohatgi P. Osseous sarcoidosis. Semin Respir Med. 1992. 13:468–488.
77. Sartoris DJ, Resnick D, Resnik C, Yaghmai I. Musculoskeletal manifestations of sarcoidosis. Semin Roentgenol. 1985. 20:376–386.
78. Scott TF. Neurosarcoidosis: progress and clinical aspects. Neurology. 1993. 43:8–12.
79. Agbogu BN, Stern BJ, Sewell C, Yang G. Therapeutic considerations in patients with refractory neurosarcoidosis. Arch Neurol. 1995. 52:875–879.
80. Baughman RP, Lower EE, du Bois RM. Sarcoidosis. Lancet. 2003. 361:1111–1118.
81. Baughman RP. Therapeutic options for sarcoidosis: new and old. Curr Opin Pulm Med. 2002. 8:464–469.
82. Paramothayan S, Jones PW. Corticosteroid therapy in pulmonary sarcoidosis: a systematic review. JAMA. 2002. 287:1301–1307.
83. Judson MA. An approach to the treatment of pulmonary sarcoidosis with corticosteroids: the six phases of treatment. Chest. 1999. 115:1158–1165.
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