Tuberc Respir Dis.  2009 Oct;67(4):275-280.

Clinical Year in Review of Interstitial Lung Diseases: Focused on Idiopathic Interstitial Pneumonia

Affiliations
  • 1Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea. wylee@wonju.yonsei.ac.kr

Abstract

Interstitial lung disease (ILD) is a group of diseases characterized by pulmonary interstitial inflammation. Finally the inflammation results in pulmonary fibrosis and impairment of oxygen transportation. The causes of idiopathic interstitial pneumonia (IIP) are unknown. Diagnosis of IIP is not easy, especially distinguising between nonspecific interstitial pneumonia and usual interstitial pneumonia (UIP). First line treatments of IIP include corticosteroids and immune modulators, which have limited effect. Currently, several drugs are being researched to prevent and treat fibrosis. Newer drugs that may useful to treat pulmonary fibrosis include endothelin receptor antagonist, recombinant soluble TNF receptor antagonist, and cotrimoxazole. The causes of IIP are largely unknown, treatment is not specific, and prognosis is poor. Recent studies are underway to investigate the pathogenesis and treatment of IIP and pulmonary fibrosis. As the pathogenesis of IIP is elucidated, better treatments will emerge.

Keyword

Idiopathic interstitial pneumonia; Nonspecific interstitial pneumonia; Usual interstitial pneumonia; Pulmonary fibrosis

MeSH Terms

Adrenal Cortex Hormones
Fibrosis
Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis
Inflammation
Lung
Lung Diseases, Interstitial
Oxygen
Prognosis
Pulmonary Fibrosis
Receptors, Endothelin
Receptors, Tumor Necrosis Factor
Transportation
Trimethoprim, Sulfamethoxazole Drug Combination
Adrenal Cortex Hormones
Oxygen
Receptors, Endothelin
Receptors, Tumor Necrosis Factor

Figure

  • Figure 1 DPLDs consist of disorders of known causes (e.g., collagen vascular disease or environmental or drug-related causes) and disorders of unknown causes. The latter include IIPs, granulomatous lung disorders, and other forms of interstitial lung disease.The most important distinction among the IIPs is that between IPF and NSIP. Other IIPs include DIP, RBILD, AIP, COP, and LIP. AIP: acute interstitial pneumonia; COP: formerly known as Bronchiolitis obliterans organizing pneumonia (BOOP); COP: cryptogenic organizing pneumonia; DIP: desquamative interstitial pneumonia; DLPD: diffuse parenchymal lung disease; IIP: idiopathic interstitial pneumonia; IPF: idiopathic pulmonary fibrosis; LIP: lymphocytic interstitial pneumonia; NSIP: nonspecific interstitial pneumonia; RBILD: respiratory bronchiolitis-associated interstitial lung disease; UIP: usual interstitial pneumonia. The ATS/ERS Classification of Diffuse Parenchymal Lung Disease 2002.

  • Figure 2 A new model for the pathogenesis of idiopathic pulmonary fibrosis: injury activates multiple inflammatory, cell signalling and repair pathways. Activation of these cascades causes an imbalance in pro- and antifibrotic mediators. In turn, these mediators activate multiple cell types, causing changes in cellular functioning and cell-cell interactions that ultimately result in progressive fibrosis. Th: T-helper cell; CTGF: connective tissue growth factor; TGF-b: transforming growth factor-b; PDGF: platelet-derived growth factor; FXa: factor Xa; PG: prostaglandin; IFN-c: interferon-c; EMT: epithelial-mesenchymal transition7.


Reference

1. Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002. 19:794–796.
2. Morgenthau AS, Padilla ML. Spectrum of fibrosing diffuse parenchymal lung disease. Mt Sinai J Med. 2009. 76:2–23.
3. Silva CI, Muller NL, Hansell DM, Lee KS, Nicholson AG, Wells AU. Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. Radiology. 2008. 247:251–259.
4. Travis WD, Hunninghake G, King TE Jr, Lynch DA, Colby TV, Galvin JR, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008. 177:1338–1347.
5. 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.
6. Pinheiro GA, Antao VC, Wood JM, Wassell JT. Occupational risks for idiopathic pulmonary fibrosis mortality in the United States. Int J Occup Environ Health. 2008. 14:117–123.
7. Maher TM, Wells AU, Laurent GJ. Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms? Eur Respir J. 2007. 30:835–839.
8. Bringardner BD, Baran CP, Eubank TD, Marsh CB. The role of inflammation in the pathogenesis of idiopathic pulmonary fibrosis. Antioxid Redox Signal. 2008. 10:287–301.
9. Rogliani P, Mura M, Assunta Porretta M, Saltini C. New perspectives in the treatment of idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2008. 2:75–93.
10. Kim R, Meyer KC. Therapies for interstitial lung disease: past, present and future. Ther Adv Respir Dis. 2008. 2:319–338.
11. King TE Jr, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008. 177:75–81.
12. Raghu G, Brown KK, Costabel U, Cottin V, du Bois RM, Lasky JA, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med. 2008. 178:948–955.
13. Varney VA, Parnell HM, Salisbury DT, Ratnatheepan S, Tayar RB. A double blind randomised placebo controlled pilot study of oral co-trimoxazole in advanced fibrotic lung disease. Pulm Pharmacol Ther. 2008. 21:178–187.
14. Nishiyama O, Kondoh Y, Kimura T, Kato K, Kataoka K, Ogawa T, et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology. 2008. 13:394–399.
15. Holland AE, Hill CJ, Conron M, Munro P, McDonald CF. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax. 2008. 63:549–554.
16. Agarwal R, Jindal SK. Acute exacerbation of idiopathic pulmonary fibrosis: a systematic review. Eur J Intern Med. 2008. 19:227–235.
17. Akira M, Kozuka T, Yamamoto S, Sakatani M. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008. 178:372–378.
18. Antoniou KM, Hansell DM, Rubens MB, Marten K, Desai SR, Siafakas NM, et al. Idiopathic pulmonary fibrosis: outcome in relation to smoking status. Am J Respir Crit Care Med. 2008. 177:190–194.
19. Ryu YJ, Chung MP, Han J, Kim TS, Lee KS, Chun EM, et al. Bronchoalveolar lavage in fibrotic idiopathic interstitial pneumonias. Respir Med. 2007. 101:655–660.
20. Kinder BW, Brown KK, Schwarz MI, Ix JH, Kervitsky A, King TE Jr. Baseline BAL neutrophilia predicts early mortality in idiopathic pulmonary fibrosis. Chest. 2008. 133:226–232.
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