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

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

  • 1Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.


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.


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

MeSH Terms

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


  • 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.


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