Diffuse interstitial lung diseases (DILDs) are a heterogeneous group of non-neoplastic, noninfectious disorders resulting from damage to the lung parenchyma, and present with similar clinical features. There has been difficulty in the classification and diagnosis of DILDs because they contain more than 200 diseases. The American Thoracic Society and European Respiratory Society (ATS/ERS) expert meeting have proposed to classify DILDs into 4 categories, (1) known cases, (2) granulomatous diseases, (3) idiopathic interstitial pneumonias (IIPs), and (4) other forms. Among them, IIPs also comprise several different clinicopathological entities; however, it was previously thought to be a single disease, idiopathic pulmonary fibrosis (IPF), and resulted in a lot of confusion. Therefore, the ATS/ERS consensus classification provided a standardized nomenclature and diagnostic criteria of IIPs. IIPs are now classified into seven clinicopathologic entities; IPF, nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), and lymphoid interstitial pneumonia (LIP). Because of these complexities, it is not easy to make the diagnosis of the patients with DILDs. This review will present a brief overview of DILDs and summary of diagnostic approaches with highlights on several specific items. An important thing to emphasize is the diagnosis of DILDs should be multi-disciplinary and the final diagnosis should be rendered only after reviewing all of the clinical, radiological, and pathological data of the patient. The more detailed description and discussion on some common diseases of DILDs will be followed by different authors.
Consensus ; Cryptogenic Organizing Pneumonia ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Diseases, Interstitial

Idiopathic interstitial pneumonias (IIP), a heterogeneous group of diffuse parenchymal lung diseases, include seven clinicopathologic entities: idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis (RB)-associated interstitial lung disease (ILD), desquamative interstitial pneumonia (DIP), and lymphoid interstitial pneumonia (LIP). Each of these entities has a typical histologic pattern that correlates well with imaging features. Thus, imaging plays an essential role in classifying and differentiating this group of diseases. The characteristic HRCT findings of IPF are reticular opacity with honeycombing and traction bronchiectasis in a predominantly basal and peripheral distribution. NSIP manifests as basal ground-glass opacity and reticular opacity. Honeycombing is rare. COP is characterized by patchy peripheral or peribronchovascular consolidation. AIP appears as extensive, mixed ground-glass opacity and consolidation. RB-ILD and DIP are smoking-related diseases associated with CT features of poorly defined centrilobular nodules and ground-glass opacity. LIP is a rare disease characterized by ground-glass opacity sometimes associated with perivascular cysts. Although some of idiopathic interstitial pneumonias may show diagnostic CT features, the final diagnosis of IIPs is usually made by means of evaluation of all the combined clinical, radiologic, and pathologic findings.
Bronchiectasis ; Bronchiolitis ; Cryptogenic Organizing Pneumonia ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Lip ; Lung Diseases, Interstitial ; Rare Diseases ; Traction

Usual interstitial pneumonia/ Idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, Cryptogenic organizing pneumonia(bronchiolitis obliterans organizing pneumonia : BOOP), Acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, Desquamative interstitial pneumonia, Lymphoid interstitial pneumonia.
Idiopathic Interstitial Pneumonias* ; Idiopathic Pulmonary Fibrosis ; Lung Diseases, Interstitial ; Pneumonia

Diffuse interstitial lung diseases (DILD) are a group of chronic disorders showing varying degrees of active inflammation and established fibrosis mainly involving the interstitium of the lungs. DILD can be classified into two groups in an etiologic aspect; idiopathic and DILD with known-cause, such as connective tissue diseases associated with DILD. Although there had been complexity and variability in the classification of idiopathic interstitial pneumonia (IIP), an international standard was established for the classification of IIPs including seven clinicalradiologic-pathologic entities; idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), and lymphoid interstitial pneumonia (LIP). The prognosis of fibrotic NSIP and IPF is much poorer compared to those of other spectrum of IIPs, such as COP, RB-ILD, DIP, and LIP. Therefore, fibrotic NSIP and IPF can be considered separately as a group of fibrotic IIPs. Trying to predict the prognosis of IIPs, there has been an effort to differentiate inflammationpredominant lesions from fibrosis-predominant lesions in patients with fibrotic IIPs. Radiologic features of low fibrotic scores at high-resolution CT and early enhancement patterns at dynamic enhancement of MRI can be useful prognostic determinants for the prediction of disease improvement in patients with fibrotic IIPs.
Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Inflammation ; Lip ; Lung ; Lung Diseases, Interstitial ; Prognosis

Alveolitis, Extrinsic Allergic ; diagnosis ; pathology ; Biopsy ; Chronic Disease ; Cryptogenic Organizing Pneumonia ; diagnosis ; Diagnosis, Differential ; Granuloma, Respiratory Tract ; diagnosis ; Humans ; Idiopathic Interstitial Pneumonias ; diagnosis ; Idiopathic Pulmonary Fibrosis ; diagnosis

Idiopathic interstitial pneumonia (IIP) is a histologically identifiable pulmonary disease without a known cause that usually infiltrates the lung interstitium. IIP is largely classified into idiopathic pulmonary fibrosis, idiopathic non-specific interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease (ILD), cryptogenic organizing pneumonia, desquamative interstitial pneumonia, and acute interstitial pneumonia. Each of these diseases has a different prognosis and requires specific treatment, and a multidisciplinary approach that combines chest high-resolution computed tomography (HRCT), histological findings, and clinical findings is necessary for their diagnosis. Diagnosis of IIP is made based on clinical presentation, chest HRCT findings, results of pulmonary function tests, and histological findings. For histological diagnosis, video-assisted thoracoscopic biopsy and transbronchial lung biopsy are used. In order to identify ILD associated with connective tissue disease, autoimmune antibody tests may also be necessary. Many biomarkers associated with disease prognosis have been recently discovered, and future research on their clinical significance is necessary. The diagnosis of ILD is difficult because patterns of ILD are both complicated and variable. Therefore, as with other diseases, accurate history taking and meticulous physical examination are crucial.
Biomarkers ; Biopsy ; Classification ; Connective Tissue Diseases ; Cryptogenic Organizing Pneumonia ; Diagnosis ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Diseases ; Lung Diseases, Interstitial ; Physical Examination ; Prognosis ; Respiratory Function Tests ; Thorax

No abstract available.
Idiopathic Interstitial Pneumonias*

No abstract available.
Idiopathic Interstitial Pneumonias*

Idiopathic pulmonary fibrosis (IPF) is strongly associated with lung cancer compared with the general population. However, other types of idiopathic interstitial pneumonia (IIP) are rarely associated with lung cancer. We describe a case of a primary lung cancer associated with IIP other than IPF, which was considered to be nonspecific interstitial pneumonia (NSIP), and NSIP disappeared spontaneously after treating the primary lung cancer.
Adenocarcinoma* ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Lung Diseases, Interstitial* ; Lung Neoplasms ; Lung*

Idiopathic interstitial pneumonias are at present classified as one of four types: usual, nonspecific, acute, or desquamative. The acute form has the worst prognosis, followed by the usual and the nonspecific form; it is in desquamative cases that prognosis is best. At high-resolution CT, usual interstitial pneumonia, the most frequent type, manifests as patchy subpleural areas of ground-glass attenuation, irregular linear opacity, and honeycombing, which the nonspecific type, the second most frequent, appears as subpleural patchy areas of ground-glass attenuation with associated areas of irregular linear opacity. Acute interstitial pneumonia demonstrates extensive bilateral airspace consolidation and patchy or diffuse bilateral areas of ground-glass attenuation in middle and lower lung zones.
Idiopathic Interstitial Pneumonias* ; Idiopathic Pulmonary Fibrosis ; Lung ; Lung Diseases ; Lung Diseases, Interstitial ; Prognosis