Tuberc Respir Dis.  2011 Sep;71(3):163-171.

Interstitial Lung Disease

• Chung MP

Abstract

Recently published articles on interstitial lung disease (ILD) have focused on the accurate diagnosis of idiopathic pulmonary fibrosis (IPF), serum biomarkers, acute exacerbation of IPF, the prognostic factors of ILD and the trial of new treatment. In particular, reports on the serum biomarkers such as CC-chemokine ligand 18, surfactant protein, circulating fibrocytes, and acute exacerbation of IPF are sufficient to be mentioned here. Pirfenidone therapy is the most important trial for the treatment of IPF. Other newer treatment trials such as interferon-gamma, sildenafil and imatinib have been reported to be unsuccessful. On the other hand, the sirolimus trial for lymphangioleiomyomatosis is promising. Combined pulmonary fibrosis and emphysema and IgG4-related disease are established to be the new disease entities of ILD.

Keyword

Lung Disease, Interstitial; Idiopathic Pulmonary Fibrosis; Pirfenidone

MeSH Terms

Benzamides
Biomarkers
Emphysema
Hand
Idiopathic Pulmonary Fibrosis
Interferon-gamma
Lung Diseases, Interstitial
Lymphangioleiomyomatosis
Piperazines
Pulmonary Fibrosis
Purines
Pyridones
Pyrimidines
Sirolimus
Sulfones
Imatinib Mesylate
Sildenafil Citrate
Benzamides
Interferon-gamma
Piperazines
Purines
Pyridones
Pyrimidines
Sirolimus
Sulfones

Reference

1. Crosby LM, Waters CM. Epithelial repair mechanisms in the lung. Am J Physiol Lung Cell Mol Physiol. 2010. 298:L715–L731.

2. Lee JS, Collard HR, Raghu G, Sweet MP, Hays SR, Campos GM, et al. Does chronic microaspiration cause idiopathic pulmonary fibrosis? Am J Med. 2010. 123:304–311.

3. Rottoli P, Bargagli E, Landi C, Magi B. Proteomic analy sis in interstitial lung diseases: a review. Curr Opin Pulm Med. 2009. 15:470–478.

4. Verma S, Slutsky AS. Idiopathic pulmonary fibrosis--new insights. N Engl J Med. 2007. 356:1370–1372.

5. Borchers AT, Chang C, Keen CL, Gershwin ME. Idiopathic pulmonary fibrosis-an epidemiological and pathological review. Clin Rev Allergy Immunol. 2011. 40:117–134.

6. Bradley B, Branley HM, Egan JJ, Greaves MS, Hansell DM, Harrison NK, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax. 2008. 63:Suppl 5. v1–v58.

7. Dempsey OJ, Kerr KM, Remmen H, Denison AR. How to investigate a patient with suspected interstitial lung disease. BMJ. 2010. 340:c2843.

8. Raghu G, Nicholson AG, Lynch D. The classification, natural history and radiological/histological appearance of idiopathic pulmonary fibrosis and the other idiopathic interstitial pneumonias. Eur Respir Rev. 2008. 17:108–115.

9. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011. 183:431–440.

10. Galvin JR, Frazier AA, Franks TJ. Collaborative radiologic and histopathologic assessment of fibrotic lung disease. Radiology. 2010. 255:692–706.

11. Wells AU. The clinical utility of bronchoalveolar lavage in diffuse parenchymal lung disease. Eur Respir Rev. 2010. 19:237–241.

12. Behr J, Ryu JH. Pulmonary hypertension in interstitial lung disease. Eur Respir J. 2008. 31:1357–1367.

13. Hyzy R, Huang S, Myers J, Flaherty K, Martinez F. Acute exacerbation of idiopathic pulmonary fibrosis. Chest. 2007. 132:1652–1658.

14. Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King TE Jr, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007. 176:636–643.

15. Schwartz DA. Genetic analysis of sporadic and familial interstitial pneumonia. Proc Am Thorac Soc. 2008. 5:343–347.

16. Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 2010. 138:251–256.

17. Castelino FV, Varga J. Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management. Arthritis Res Ther. 2010. 12:213.

18. Antoniou KM, Margaritopoulos G, Economidou F, Siafakas NM. Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement. Eur Respir J. 2009. 33:882–896.

19. Fischer A, Brown KK, Frankel SK. Treatment of connective tissue disease-associated interstitial lung disease. Clin Pulm Med. 2009. 16:74–80.

20. McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest. 2008. 133:507–516.

21. Johnson SR, Cordier JF, Lazor R, Cottin V, Costabel U, Harari S, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010. 35:14–26.

22. Huizar I, Kavuru MS. Alveolar proteinosis syndrome: pathogenesis, diagnosis, and management. Curr Opin Pulm Med. 2009. 15:491–498.

23. Chung MP, Yi CA, Lee HY, Han J, Lee KS. Imaging of pulmonary vasculitis. Radiology. 2010. 255:322–341.

24. Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med. 2011. 183:573–581.

25. Morgenthau AS, Iannuzzi MC. Recent advances in sarcoidosis. Chest. 2011. 139:174–182.

26. Maher TM. The diagnosis of idiopathic pulmonary fibrosis and its complications. Expert Opin Med Diagn. 2008. 2:1317–1331.

27. Fell CD, Martinez FJ, Liu LX, Murray S, Han MK, Kazerooni EA, et al. Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2010. 181:832–837.

28. Ohshimo S, Bonella F, Cui A, Beume M, Kohno N, Guzman J, et al. Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009. 179:1043–1047.

29. Sverzellati N, Wells AU, Tomassetti S, Desai SR, Copley SJ, Aziz ZA, et al. Biopsy-proved idiopathic pulmonary fibrosis: spectrum of nondiagnostic thin-section CT diagnoses. Radiology. 2010. 254:957–964.

30. Song JW, Do KH, Kim MY, Jang SJ, Colby TV, Kim DS. Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia. Chest. 2009. 136:23–30.

31. van Moorsel CH, van Oosterhout MF, Barlo NP, de Jong PA, van der Vis JJ, Ruven HJ, et al. Surfactant protein C mutations are the basis of a significant portion of adult familial pulmonary fibrosis in a Dutch cohort. Am J Respir Crit Care Med. 2010. 182:1419–1425.

32. van den Blink B, Wijsenbeek MS, Hoogsteden HC. Serum biomarkers in idiopathic pulmonary fibrosis. Pulm Pharmacol Ther. 2010. 23:515–520.

33. Thomeer M, Grutters JC, Wuyts WA, Willems S, Demedts MG. Clinical use of biomarkers of survival in pulmonary fibrosis. Respir Res. 2010. 11:89.

34. Prasse A, Probst C, Bargagli E, Zissel G, Toews GB, Flaherty KR, et al. Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009. 179:717–723.

35. Kinder BW, Brown KK, McCormack FX, Ix JH, Kervitsky A, Schwarz MI, et al. Serum surfactant protein-A is a strong predictor of early mortality in idiopathic pulmonary fibrosis. Chest. 2009. 135:1557–1563.

36. Moeller A, Gilpin SE, Ask K, Cox G, Cook D, Gauldie J, et al. Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009. 179:588–594.

37. Moore BB. Fibrocytes as potential biomarkers in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009. 179:524–525.

38. Collard HR, Calfee CS, Wolters PJ, Song JW, Hong SB, Brady S, et al. Plasma biomarker profiles in acute exacerbation of idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol. 2010. 299:L3–L7.

39. Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011. 37:356–363.

40. Konishi K, Gibson KF, Lindell KO, Richards TJ, Zhang Y, Dhir R, et al. Gene expression profiles of acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2009. 180:167–175.

41. Suda T, Kaida Y, Nakamura Y, Enomoto N, Fujisawa T, Imokawa S, et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 2009. 103:846–853.

42. Huie TJ, Olson AL, Cosgrove GP, Janssen WJ, Lara AR, Lynch DA, et al. A detailed evaluation of acute respiratory decline in patients with fibrotic lung disease: aetiology and outcomes. Respirology. 2010. 15:909–917.

43. Kondoh Y, Taniguchi H, Kataoka K, Kato K, Suzuki R, Ogura T, et al. Prognostic factors in rapidly progressive interstitial pneumonia. Respirology. 2010. 15:257–264.

44. Park IN, Jegal Y, Kim DS, Do KH, Yoo B, Shim TS, et al. Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J. 2009. 33:68–76.

45. Akira M, Inoue Y, Arai T, Okuma T, Kawata Y. Long-term follow-up high-resolution CT findings in non-specific interstitial pneumonia. Thorax. 2011. 66:61–65.

46. Lee JW, Lee KS, Lee HY, Chung MP, Yi CA, Kim TS, et al. Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. AJR Am J Roentgenol. 2010. 195:916–922.

47. Ozawa Y, Suda T, Naito T, Enomoto N, Hashimoto D, Fujisawa T, et al. Cumulative incidence of and predictive factors for lung cancer in IPF. Respirology. 2009. 14:723–728.

48. Olson AL, Swigris JJ, Sprunger DB, Fischer A, Fernandez-Perez ER, Solomon J, et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med. 2011. 183:372–378.

49. Mukae H, Ishimoto H, Sakamoto N, Hara S, Kakugawa T, Nakayama S, et al. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis. Chest. 2009. 136:1341–1347.

50. Taniguchi H, Ebina M, Kondoh Y, Ogura T, Azuma A, Suga M, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010. 35:821–829.

51. Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, et al. Idiopathic Pulmonary Fibrosis Clinical Research Network. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010. 363:620–628.

52. King TE Jr, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet. 2009. 374:222–228.

53. Daniels CE, Lasky JA, Limper AH, Mieras K, Gabor E, Schroeder DR, et al. Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med. 2010. 181:604–610.

54. Klingsberg RC, Mutsaers SE, Lasky JA. Current clinical trials for the treatment of idiopathic pulmonary fibrosis. Respirology. 2010. 15:19–31.

55. Thabut G, Christie JD, Ravaud P, Castier Y, Dauriat G, Jebrak G, et al. Survival after bilateral versus single-lung transplantation for idiopathic pulmonary fibrosis. Ann Intern Med. 2009. 151:767–774.

56. Bissler JJ, McCormack FX, Young LR, Elwing JM, Chuck G, Leonard JM, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med. 2008. 358:140–151.

57. Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005. 26:586–593.

58. Mejía M, Carrillo G, Rojas-Serrano J, Estrada A, Suárez T, Alonso D, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009. 136:10–15.

59. Cottin V, Le Pavec J, Prévot G, Mal H, Humbert M, Simonneau G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J. 2010. 35:105–111.

60. Shigemitsu H, Koss MN. IgG4-related interstitial lung disease: a new and evolving concept. Curr Opin Pulm Med. 2009. 15:513–516.

61. Shrestha B, Sekiguchi H, Colby TV, Graziano P, Aubry MC, Smyrk TC, et al. Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis: report of 6 and 12 cases with similar histopathology. Am J Surg Pathol. 2009. 33:1450–1462.

62. Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Kobayashi T, et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009. 251:260–270.

63. Scientific Committee of the Korean Academy of Tuberculosis and Respiratory Diseases. 2008 National survey of idiopathic interstitial pneumonia in Korea. Tuberc Respir Dis. 2009. 66:141–151.

64. Kim C, Jeong SH, Shim JJ, Cha SI, Son C, Chug MP, et al. Clinical features of pulmonary langerhans cell histiocytosis in Korea. Tuberc Respir Dis. 2009. 66:98–103.

65. Byun MK, Kim DS, Kim YW, Chung MP, Shim JJ, Cha SI, et al. Clinical features and outcomes of idiopathic pulmonary alveolar proteinosis in Korean population. J Korean Med Sci. 2010. 25:393–398.

66. Park HY, Nam HS, Chung MP, Jeong SH, Kim YJ, Cha SI, et al. A nationwide survey of lymphangioleiomyomatosis in Korea. J Korean Med Sci. 2010. 25:1182–1186.