Idiopathic pulmonary fibrosis (IPF) is a chronic fatal pulmonary disease characterized by complex illness condition. There is no effective treatment at present except lung transplantation. The comprehensive evaluation is helpful for the management of patients with IPF in hierarchical stages. Therefore, it is very important to evaluate IPF by various independent factors. At present, the commonly used methods for clinical evaluation on IPF include assessment of health-related quality of life, assessment of physiological function, assessment of imaging, assessment of laboratory examination, and multi-dimensional assessment system. However, there are different advantages and disadvantages on diverse evaluation methods for the evaluation of IPF.
Humans ; Idiopathic Pulmonary Fibrosis/diagnosis* ; Quality of Life

Humans ; Diagnosis, Differential ; Idiopathic Pulmonary Fibrosis/pathology* ; Lung/pathology*

Interstitial lung disease (ILD) is a rare condition characterized by extensive inflammation and fibrosis mainly involving the pulmonary interstitium or alveoli. Usually, patients with ILD clinically present with chronic cough and exertional dyspnea. ILD is classified into subtypes based on clinical characteristics, detailed history obtained from patients, and radiological, and/or histopathological features. The most common type of idiopathic interstitial pneumonia is idiopathic pulmonary fibrosis (IPF). IPF is a chronic progressive fibrosing ILD and is associated with poor prognosis. An exclusive diagnosis of IPF requires no known condition causing ILD and typical radiological and/or histopathological features of lung fibrosis. Fibrosis observed in this condition is attributable to repetitive epithelial injury with consequent abnormal wound healing in genetically susceptible and elderly individuals. Currently, pirfenidone and nintedanib are useful disease-modifying agents available to treat IPF. In this article, we review the concept, diagnosis, clinical course, and treatment of ILD.
Aged ; Cough ; Diagnosis ; Dyspnea ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Inflammation ; Lung ; Lung Diseases, Interstitial ; Prognosis ; Wound Healing

BACKGROUND: There have been several studies showing that the angiotensin II and angiotensin converting enzyme(ACE) contributes to the apoptosis of alveolar epithelial cells in idiopathic interstitial pneumonia and the activation of fibroblasts during the process of pulmonary fibrosis. These results suggest that the pulmonary fibrosis can be inhibited by the angiotensin II receptor antagonist(AGIIRA). This study was performed to identify the therapeutic effect of AGIIRA in idiopathic pulmonary fibrosis(IPF). METHOD: Thirteen patients with IPF, who were diagnosed with an open lung biopsy(6 patients) and furfilling the ATS criteria(7 patients) between March 1999 and October 2001 at the Gachon medical center, were enrolled in this study. Of these patients, eight patients were treated with a regimen including AGIIRA(AT group), and five were treated without AGIIRA(NT group). The pulmonary function tests and dyspnea(ATS scale) were measured at diagnosis and 1 year after treatment. All the data was collected to analyze the therapeutic effect of AGIIRA on the patients with IPF. RESULTS: The AT group contained 8 patients(M:F=4:4) and the NT group contained 5 patients (M:F=3:2). There was no significant difference in the serum angiotensin II level between the two groups(202.5+/-58.5 vs 163.7+/-47.3pg/ml, p>0.05). The AT group showed an upward trend in TLC(+3%), FVC(+4%), FEV1(+3%) and DLco(+2%) compared to the NT group(TLC(-14%), FVC(-3%), FEV1(-4%) except for DLco(+5%)). The dyspnea score in the AT group improved significantly but not in the NT group. CONCLUSION: These results suggest that the angiotensin II receptor antagonist may have an effect on stabilizing IPF.
Angiotensin II* ; Angiotensins* ; Apoptosis ; Diagnosis ; Dyspnea ; Epithelial Cells ; Fibroblasts ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis* ; Lung ; Pulmonary Fibrosis ; Receptors, Angiotensin* ; Respiratory Function Tests

The specific diagnosis in diffuse interstitial lung disease may be obtained through open lung biopsy. Diffuse interstitial lung disease is often associated with lung cancer. We report one case of lung adenocarcinoma with idiopathic pulmonary fibrosis in whom previous open lung biopsy had been performed. We need general concepts about sites of open lung biopsy in these patients. Therefore, we report this case and document other references.
Adenocarcinoma* ; Biopsy* ; Diagnosis ; Humans ; Idiopathic Pulmonary Fibrosis* ; Lung Diseases, Interstitial ; Lung Neoplasms ; Lung*

Microscopic polyangiitis is a systemic small-vessel vasculitis that is associated primarily with necrotizing glomerulonephritis and pulmonary capillaritis. A recurrent and diffuse alveolar hemorrhage due to pulmonary capi llaritis is the main clinical manifestation of lung involvement. Recently, an interstitial lung disease that mimics idiopathic pulmonary fibrosis was reported to be rarely associated with microscopic polyangiitis. Here we report two patients with microscopic polyangiitis who showed a honeycomb lung at the time of the initial diagnosis with a brief review of relevant literature.
Diagnosis ; Glomerulonephritis ; Hemorrhage ; Humans ; Idiopathic Pulmonary Fibrosis ; Lung Diseases, Interstitial ; Lung* ; Microscopic Polyangiitis* ; Vasculitis

OBJECTIVETo develop a life quality scale suitable for idiopathic pulmonary fibrosis (IPF) patients, objectively reflecting its changes.

METHODSAuthors first put forward a theoretical structure model of a scale according to patient-reported outcome (PRO) scale formulation principle by combining basic theories of Chinese medicine (CM). Then authors developed an initial scale on the basis of various life quality scales for respiratory disease patients by using structural decision making. Totally 34 patients with confirmed diagnosis of IPF were tested by questionnaire. Items were screened using expert importance scoring method, factor analysis, correlation coefficient method, Cronbach's alpha coefficient method. IPF patient reported outcomes (IPF PRO, IP) were finally defined.

RESULTSA new IP scale was developed covering three areas and 38 items. Pearson correlation coefficient for correlation analysis of clinical symptom scores in ST-George Respiratory Questionnaire and IP scale was 0.828 (P < 0.01). Pearson correlation coefficient for correlation analysis of activity ability scores was 0.929 (P < 0.01). Pearson correlation coefficient for correlation analysis of total scores was 0.862 (P < 0.01). By reliability of IP scale itself (reliability) analysis, Cronbach's alpha coefficient was 0.713. By using factor analysis method for data analysis, KMO statistics was 0.902.

CONCLUSIONIP scale fully reflected the connotation of IPF patients' quality of life, so it could be used as CM clinical therapeutic effect evaluation tool.

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease characterized by progressive lung fibrogenesis and histological features of usual interstitial pneumonia. IPF has a poor prognosis and presents a spectrum of disease courses ranging from slow evolving disease to rapid deterioration; thus, a differential diagnosis remains challenging. Several biomarkers have been identified to achieve a differential diagnosis; however, comprehensive reviews are lacking. This review summarizes over 100 biomarkers which can be divided into six categories according to their functions: differentially expressed biomarkers in the IPF compared to healthy controls; biomarkers distinguishing IPF from other types of interstitial lung disease; biomarkers differentiating acute exacerbation of IPF from stable disease; biomarkers predicting disease progression; biomarkers related to disease severity; and biomarkers related to treatment. Specimen used for the diagnosis of IPF included serum, bronchoalveolar lavage fluid, lung tissue, and sputum. IPF-specific biomarkers are of great clinical value for the differential diagnosis of IPF. Currently, the physiological measurements used to evaluate the occurrence of acute exacerbation, disease progression, and disease severity have limitations. Combining physiological measurements with biomarkers may increase the accuracy and sensitivity of diagnosis and disease evaluation of IPF. Most biomarkers described in this review are not routinely used in clinical practice. Future large-scale multicenter studies are required to design and validate suitable biomarker panels that have diagnostic utility for IPF.
Humans ; Idiopathic Pulmonary Fibrosis/diagnosis* ; Biomarkers ; Lung Diseases, Interstitial ; Lung ; Bronchoalveolar Lavage Fluid ; Disease Progression ; 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 nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.
Azathioprine ; Bronchiectasis ; Cough ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Dyspnea ; Female ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Immunosuppressive Agents ; Inflammation ; Lung ; Lung Diseases, Interstitial ; Pathology ; Prognosis ; Survival Rate ; Traction