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INTRODUCTION@#The current gold standard for diagnosing interstitial lung disease (ILD) involves an ILD clinic evaluation, followed by discussion in a multidisciplinary meeting (MDM). However, there is a paucity of data on the impact of ILD MDMs on the diagnosis and management of ILDs in Southeast Asia. We studied the clinical impact of the ILD service on the diagnosis and management of ILDs at a university-affiliated tertiary hospital in Singapore.@*METHODS@#A single-centre retrospective review was done on 97 consecutive patients referred for evaluation to the ILD service from March 2016 to August 2017.@*RESULTS@#Mean age of the patients was 67 ± 11 years. Gender distribution was almost equal (52% male), with a majority of never-smokers (63%). Mean forced vital capacity (FVC) was 1.81 ± 0.66 L (66% ± 20% predicted). The three commonest referral diagnoses were ILD of uncertain classification (n = 38, 39%), connective tissue disease-associated ILD (CTD-ILD) (n = 24, 25%) and idiopathic pulmonary fibrosis (IPF) (n = 16, 17%). Following evaluation by the ILD service, there was a change of diagnosis in 60 (62%) patients and a change of management in 71 (73%) patients. The majority of consensus MDM diagnoses were IPF (n = 35, 36%), CTD-ILD (n = 30, 30%) and others (n = 15, 15%). There was a significant prognostic separation between the IPF and non-IPF diagnoses made following evaluation by the ILD service.@*CONCLUSION@#The ILD service allowed for more precise subtyping of various ILDs. This is particularly useful for IPF patients, who can benefit from antifibrotic therapies.
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Objective: To explore the influencing factors related to the quality of life of the patients with idiopathic interstitial pneumonia (IIP), and to provide the theoretical basis for improving the quality of life, preventing other complications, and improving the prognosis of the patients with IIP. Methods: St George' s Respiratory Questionnaire (SGRQ) was used to evaluate the quality of life of 300 hospitalization patients with IIP who met the inclusion criteria in the study. The differences of quality of life among different groups of people were compared. A general linear regression model was used to screen the independent influencing factors that may affect the scores of quality life of all dimensions. Results: Drinking, forced vital capacity (FVC) and 6 min walk test (6MWT) were the independent influencing factors of the symptom score of the IIP patients (P<0. 05); nation, family per capita monthly income, carbon monoxide diffusion capacity (DLCO), and 6MWT were the independent influencing factors of activity limit score of the IIP patients (P<0. 05); nation, family per capita monthly income, FVC, and 6MWT were the independent influencing factors of life impact score of the IIP patients (P<0. 05); nation, family per capita monthly income, FVC, and 6MWT were the independent influencing factors of the scores of quality of life of the IIP patients (P<0. 05). Conclusion: The activity limit of IIP patients is more obvious. Drinking, nation and family per capita monthly income, FVC, 6MWT and DLOC are the factors affecting the quality of life of the IIP patients in different dimensions.
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Abstract The diagnosis of idiopathic interstitial pneumonias (IIPs) involves a multidisciplinary scenario in which the radiologist assumes a key role. The latest (2013) update of the IIP classification by the American Thoracic Society/European Respiratory Society proposed some important changes to the original classification of 2002. The novelties include the addition of a new disease (idiopathic pleuroparenchymal fibroelastosis) and the subdivision of the IIPs into four main groups: chronic fibrosing IIPs (idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia); smoking-related IIPs (desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease); acute or subacute IIPs (cryptogenic organizing pneumonia and acute interstitial pneumonia); rare IIPs (lymphoid interstitial pneumonia and idiopathic pleuroparenchymal fibroelastosis); and the so-called "unclassifiable" IIPs. In this study, we review the main clinical, tomographic, and pathological characteristics of each IIP.
Resumo O diagnóstico das pneumonias intersticiais idiopáticas (PIIs) envolve um cenário multidisciplinar no qual o radiologista assume papel fundamental. A última atualização (2013) da classificação das PIIs pela American Thoracic Society/European Respiratory Society propõe algumas mudanças importantes em relação à classificação original de 2002. Dentre as novidades, destacam-se o acréscimo de uma nova doença (fibroelastose pleuroparenquimatosa idiopática) e a subdivisão das PIIs em quatro grupos principais: PIIs crônicas fibrosantes (fibrose pulmonar idiopática e pneumonia intersticial não específica); PIIs relacionadas ao tabagismo (pneumonia intersticial descamativa e bronquiolite respiratória com doença intersticial pulmonar); PIIs agudas/subagudas (pneumonia em organização e pneumonia intersticial aguda); PIIs raras (pneumonia intersticial linfocítica e fibroelastose pleuroparenquimatosa idiopática); além das ditas "inclassificáveis". Foram revisadas, de forma didática neste estudo, as principais características clínicas, tomográficas e patológicas de cada uma das PIIs.
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Objective To investigate the short-term effects of glucocorticoid therapy on patients with idiopathic interstitial pneumonia using high resolution CT semi quantitative scores. Methods According to the different HRCT scores will be January 2015 -2017 year in January in our hospital for diagnosis and treatment of idiopathic interstitial pneumonia patients with 60 cases of groups: control group with HRCT score < 20%, the observation group with HRCT score more than 20%; the patients in the two groups of experimental data were recorded in detail and analyzed and discussed the high resolution CT semi quantitative score judgment of idiopathic interstitial pneumonia patients treated with glucocorticoid efficacy. Results The observation group (HRCT score≥20%) accuracy is higher than that of the control group (HRCT score<20%), the difference between groups was statistically significant (P<0.05). Conclusion The treatment of idiopathic interstitial pneumonia in patients with glucocorticoid selection of high resolution CT;semi quantitative score to determine significant short-term effect, HRCT score of the best critical value was 20%, the diagnostic accordance rate can obtain excellent.
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Objective To study and analyze the short-term effect of glucocorticoid in patients with idiopathic interstitial pneumonia by analyzing the semi quantitative scores of high resolution CT.Methods 50 cases of idiopathic interstitial pneumonia treated in our hospital from May 2015 to October 2016 were selected as the research object.Clinical data were analyzed, including smoking, signs of hospitalization and dyspnea score.High resolution CT level for the right diaphragmatic top tube, bifurcation, left atrium roof, in 3 aspects from the calculation of cellular disease and shadow grid as a percentage of average, the critical point of high resolution CT scores were 10%, 20%, 30%, 40% and 50%.The accuracy, sensitivity and specificity of glucocorticoids were analyzed.The chi square test was used to examine the enumeration data.Results The accuracy was 72% when the high resolution CT score was 20% critical point.In patients treated with corticosteroids, the high resolution CT score was greater than or equal to 20% for patients and 25 for patients with a score of less than 20% for 25 patients.20% or more patients died during hospitalization, 10 cases, the mortality rate was 40%, and the effective rate of glucocorticoid treatment was 8%.Less than 20% of the patients died during hospitalization, 1 cases, the mortality rate was 4%, and the effective rate of glucocorticoid treatment was 50%.Conclusion High resolution CT semi quantitative score judgment of idiopathic interstitial pneumonia patients treated with glucocorticoid efficacy of high accuracy, high resolution 20% scores higher accuracy, and the diagnosis is well, with further clinical promotion and application significance.
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Objective To evaluate the short-term effect of glucocorticoid therapy in patients with idiopathic interstitial pneumonia by high resolution CT semi quantitative score. Methods 32 cases of idiopathic interstitial pneumonia were treated with conventional treatment, combined with glucocorticoids, the use of high resolution CT semi quantitative score, the short-term efficacy of its accurate judgement. Results The analysis shows that high resolution CT semi quantitative score below 20％ idiopathic interstitial pneumonia patients with hospital mortality period is only 5.26％, and the high resolution CT semi quantitative score during the high fatality rate of 38.46％ hospitalized patients with 20.00％ or more, the difference was statistically significant (P<0.05). Conclusion The application of high resolution CT semi quantitative score of received corticosteroid therapy for idiopathic interstitial pneumonia patients with curative effect to give accurate judgment to the doctor for the future development of drug treatment and guarantee its prognosis are of positive significance.
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The most common and most lethal type of idiopathic interstitial pneumonia (IIP) is idiopathic pulmonary fibrosis (IPF), which accounts for 55% of lung diseases classified as IIPs. Diagnosis of IPF requires precision and a multidisciplinary approach .Indeed, an early and accurate diagnosis of IPF is critical for a better outcome, especially with the advent of new specific treatments for this disease. The previous guidelines using major and minor criteria for the clinical (i.e. non-pathological) diagnosis of IPF have been discarded, as it is now clear that, in an appropriate clinical setting, the presence of a classical UIP pattern on the HRCT scan is sufficient for a diagnosis of IPF to be made. In the presence of the four classical features, that together accurately identify a Usual interstitial pneumonia (UIP) pattern, a definitive diagnosis of IPF can be made. Guidelines emphasizes the importance of multidisciplinary discussion between clinicians, radiologists and pathologists to improve diagnostic confidence. The course of disease in IPF is unpredictable, but the importance of an early diagnosis is clear, as individuals with less severe lung function abnormalities have a better prognosis.
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Usual interstitial pneumonia (UIP) is one type of idiopathic interstitial pneumonia, characterized by its poor prognosis and gradual deterioration of clinical course. So it is important to distinguish UIP from other interstitial pneumonia. Defi nitive histological diagnosis of UIP requires lung biopsy. The criteria for diagnosis of UIP in the absence of a surgical lung biopsy were recently defi ned. We report a case of 75-year-old man who was diagnosed as bronchopneumonia with chief complaint of fever, dyspnea on fi rst visit, then fi nally diagnosed as UIP through the remaining of chest abnormality after treatment.
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Aged , Humans , Biopsy , Bronchopneumonia , Dyspnea , Fever , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Lung , Lung Diseases, Interstitial , Prognosis , ThoraxABSTRACT
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.
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Humans , Connective Tissue Diseases , Cryptogenic Organizing Pneumonia , Fibrosis , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Inflammation , Lip , Lung , Lung Diseases, Interstitial , PrognosisABSTRACT
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.
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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 CombinationABSTRACT
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.
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Humans , Consensus , Cryptogenic Organizing Pneumonia , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Lung , Lung Diseases, InterstitialABSTRACT
PURPOSE: Histologic classification plays a key role in the classification of idiopathic interstitial pneumonia (IIP) into clinically meaningful categories in terms of natural history, prognosis and treatment. The implications of histological diagnosis, clinical features and prognosis in children has not been described. This study aimed to analyze the clinical features of IIP in children. METHODS: A total of 15 patients with IIP were recruited, who had a surgical lung biopsy. The age, sex, symptoms, initial oxygen saturation, radiologic findings and clinical courses were retrospectively investigated. RESULTS: The median age at diagnosis was 3 years. Cough (93.3%), tachypnea (86.7%) and dyspnea (80%) were the most common symptoms, and laboratory findings were nonspecific. Acute interstitial pneumonia (n=6), chronic pneumonitis of infancy (n=4) nonspecific interstitial pneumonia (n=3) were relatively common. All patients received corticosteroid therapy and the mortality rate was 26.7% (n=4). CONCLUSION: IIP has diverse clinical features according to subtypes. Knowledge of the underlying histopathology will allow the prediction of more accurate prognosis, the decision of appropricate therapy, and the clinical investigation of novel therapeutic agents in patients with IIP.
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Child , Humans , Biopsy , Classification , Cough , Diagnosis , Dyspnea , Idiopathic Interstitial Pneumonias , Lung , Lung Diseases, Interstitial , Mortality , Natural History , Oxygen , Pneumonia , Prognosis , Retrospective Studies , TachypneaABSTRACT
BACKGROUND: Although corticosteroid and cytotoxic agent such as cyclophosphamide have been used for the treatment of idiopathic interstitial pneumonia (IIP), efficacy of these toxic drugs are unclear because previous reports included the patients who did not undergo surgical lung biopsy and none evaluated the response according to histopathologic entities of IIP. To answer this, we retrospectively analyzed the treatment response and side effects of corticosteroids and cyclophosphamide therapy in patients with idiopathic UIP and NSIP. METHODS: Among 61 patients with UIP and 26 patients with NSIP diagnosed by surgical lung biopsy at Samsung Medical Center from July 1996 to June 2002, those who received corticosteroid or cyclophosphamide therapy for at least 6 months and were followed for at least one year after the initiation of treatment were enrolled (32 UIP, 23 NSIP). Treatment response of 55 patients was assessed by ATS response criteria (clinical symptoms, pulmonary function test and radiological findings).Adverse reactions to either agent (42 cases of cyclophosphamide+/-low-dose prednisolone, 49 cases of prednisolone alone) were also analyzed. RESULTS: Irrespective of treatment regimen, NSIP showed more favorable response than UIP (6 months: 78.3% vs. 9.4%, 12 months: 69.6% vs. 9.4%, p<0.001). Cyclophosphamide showed comparable response to corticosteroid in NSIP while its efficacy was as poor as those of corticosteroid therapy in UIP. Significant adverse reaction to drug more frequently occurred in corticosteroid group (35.7%) than cyclophosphamide group (14.3%) (p=0.017). CONCLUSION: Cyclophosphamide is effective and more tolerable than corticosteroids in the treatment of idiopathic nonspecific interstitial pneumonia.
Subject(s)
Humans , Adrenal Cortex Hormones , Biopsy , Cyclophosphamide , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Lung , Lung Diseases, Interstitial , Prednisolone , Respiratory Function Tests , Retrospective StudiesABSTRACT
BACKGROUND: There have been several studies showing that angiotensin II and the angiotensin convertingenzyme (ACE) contribute to the activation of fibroblast including the pulmonary fibrosis, and apoptosis of the al veolar epithelium in idiopathic intersititial pneumonia. This study was performed to identify the relationship between the serum angiotensin II. ACE and the pulmonary function test (PFT), the dyspnea score, and the cell fraction of the bronchoalveolar lavage fluid (BALF). METHODS: Twenty three patients with idiopathic interstitial pneumonia from March, 1999 to October, 2001 at Gachon medical school were enrolled in this study. They were divided into IPF(UIP) (16) and NSIP (7) group. Twelve of the idiopathic interstitial pneumonia patients (UIP : 5, NSIP: 7) were diagnosed by an open lung biopsy, 11 of IPF patients were diagnosed by the American Thoracic Society (ATS) diagnostic criteria. The PFT values, dyspnea score, serum ACE and angiotensin II were measured, and a bronchoscopy was performed to obtain the BALF. RESULTS: Of all the patients, 7 were in the normal range and 14 showed an increase in the serum level of angiotensin II. In terms of the serum ACE level, 14 patients had an increased level. The DLCO% of the angiotensin II. increased group was significantly lower than the not-increased group (p=0.021). Other factors did not correlate with the serum ACE or the angiotensin II increased group and not-increased group. CONCLUSION: These results suggest that an increased angiotensinII serum level may be associated with in crease in the of alveolar capillary block in the progression of pulmonary fibrosis in idiopathic interstitial pneumonia.
Subject(s)
Humans , Angiotensin II , Angiotensins , Apoptosis , Biomarkers , Biopsy , Bronchoalveolar Lavage Fluid , Bronchoscopy , Capillaries , Dyspnea , Epithelium , Fibroblasts , Fibrosis , Idiopathic Interstitial Pneumonias , Lung , Peptidyl-Dipeptidase A , Pneumonia , Pulmonary Fibrosis , Reference Values , Respiratory Function Tests , Schools, MedicalABSTRACT
BACKGROUND: Idiopathic interstitial pneumonia is characterized by chronic inflammation and pulmonary fibrosis. The clara cell 10 kD protein (CC10, also designated CC16) is synthesized by the bronchial epithelium and has been suggested to have a potent anti-inflammatory effect. Therefore, CC-10 might be a candidate for controlling the inflammatory events in patients with idiopathic interstitial pneumonia. The aim of this study was to determine if the degrees of pulmonary fibrosis in idiopathic interstitial pneumonia is associated with CC-10 in the BAL fluid. METHODS: The BAL fluid was collected from 29 patients and 10 controls. Densitometric analysis of the western blot assay for the CC-10 was subsequently performed. The RI (relative intensity) of each band was compared according to the diagnosis, the radiological degrees of pulmonary fibrosis and the relative proportion of inflammatory cells in the BAL fluid. RESULTS: There were no differences in the CC-10 expression levels in the BAL fluid between the patients (RI 77.5+/-75.8%) and the controls (70.7+/-39.8%) (p>0.05). In addition, the degrees of pulmonary fibrosis and airway inflammation in patients with usual interstitial pneumonia were not associated with CC-10 expression in the BAL fluid (p>0.05). CONCLUSION: This study suggests that CC-10 expression is not associated with the degrees of pulmonary fibrosis in patients with usual interstitial pneumonia.
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Interstitial lung disease is a generic term for a heterogeneous group of lung disease that primarily affect the interstitium although the disease is not clearly restricted to the interstitium. The majority of interstitial lung diseases represent inflammatory insults to the microscopic anatomic space bounded by the basement membrane of epithelial and endothelial cells, which may occur as slowly developing process and ultimately end up as end-stage honeycomb fibrosis. The currently prevalent classification of interstitial pneumonia with practical utility and easy reproducibility pertaining only to idopathic interstitial pneumonia encompasses several different entities some of which may represent different aspects of the same condition. Honeycomb fibrosis is usually caused by a variety of pulmonary disease including chronic interstitial lung disease. It is important to recognize that usual inter-stitial pneumonia and honeycomb fibrosis are not synonymous. In the era of chemotherapy for malignant tumor, aggressive immunosuppression for autoimmune diseases and transplant recipients and acquired immunodeficiency syndrome, lung disease in the immunocompromised host has been common. Diagnostic lung biopsy becomes increasingly needed because proper treatment of interstitial lung disease relies on correct morphologic diagnosis. This review summarizes the pathologic spectrum of idiopathic interstitial pneumonias together with other inflammatory process with known or suggestive etiologies simulating interstitial pneumonias.