Asymmetrical Interstitial Lung Disease Suggested to Be Due to Hypoplasia of the Unilateral Pulmonary Artery: A Case Report with a 20-year Follow-up

We herein report a case of asymmetrical interstitial lung disease (ILD) that remained almost completely asymmetrical over time on chest computed tomography (CT). An open lung biopsy from the right lung showed severe pleural adhesion, obstruction of the pulmonary artery, and dilated systemic arteries in addition to the usual interstitial pneumonia pattern. Three-dimensional CT angiography showed partial defects of pulmonary arteries on the affected side. After excluding other known causes of ILD and gastroesophageal reflux, we suspected that decreased pulmonary artery perfusion in the present case may have been responsible for the observed asymmetrical unilateral fibrosis.     

Lower occurrence of idiopathic pulmonary fibrosis in Maori and Pacific Islanders

OBJECTIVE: Idiopathic pulmonary fibrosis (IPF), a chronic fibro-proliferative interstitial pneumonia, has not been reported to occur more frequently in any particular race. We have observed that our patients with IPF comprise a proportionately lower number of Maori and Pacific Islanders and set out to evaluate this further. METHODS: Retrospective analysis of an IPF database from a single tertiary respiratory institution was undertaken. Demographic and survival data were collected. Ethnicity was compared with 2001 New Zealand census data from the same catchment area. RESULTS: Eighty-seven cases of IPF were identified. Overall median survival was 46 months. Ethnicity data were available for 84 of the 87 cases. 76/84 (90%) were European, 6/84 (7%) were Asian or Indian, 2/84 (2%) were Maori, and 0/84 (0%) were Pacific Islanders. For Maori and Pacific Islanders, this represented a significant trend in difference when compared with ethnicity data from the hospital catchment population (P < 0.001). CONCLUSIONS: Our department is the tertiary referral centre for pulmonary disease in the upper North Island of New Zealand, and therefore referral centre bias is likely to be low. The preliminary observation that the occurrence of IPF is lower in those of Maori or Polynesian ethnicity warrants further study. This may, in part, help in our understanding of the pathogenic mechanisms in IPF.     

2018年特发性肺纤维化临床诊断指南解读

特发性肺纤维化(IPF) 是一种原因不明的间质性肺病(ILD)。2018 年国际间质病专家组对以影像学和组织病理学为基础的2011 年IPF 诊断标准进行了更新。文章就2018 年IPF 诊断标准新指南进行解读。     

Diagnosing fibrotic lung disease: When is high‐resolution computed tomography sufficient to make a diagnosis of idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis (IPF), a progressive and fatal diffuse parenchymal lung disease, is defined pathologically by the pattern of usual interstitial pneumonia (UIP). Unfortunately, a surgical lung biopsy cannot be performed in all patients due to comorbidities that may significantly increase the morbidity and mortality of the procedure. High-resolution computed tomography (HRCT) has been put forth as a surrogate to recognize pathological UIP. The quality of the HRCT impacts the ability to make a diagnosis of UIP and varies based on the centre performing the study and patient factors. The evaluation of the HRCT includes assessing the distribution and predominance of key radiographical findings, such as honeycomb, septal thickening, traction bronchiectasis and ground glass attenuation lesions. The combination of the pattern and distribution is what leads to a diagnosis and associated confidence level. HRCT features of definite UIP (subpleural, basal predominant honeycomb with septal thickening, traction bronchiectasis and ground glass attenuation lesions) have a high specificity for the UIP pathological pattern. In such cases, surgical lung biopsy can be avoided. There are caveats to using the HRCT to diagnose IPF in isolation as a variety of chronic pulmonary interstitial diseases may progress to a UIP pattern. Referral centres with experience in diffuse parenchymal lung disease that have multidisciplinary teams encompassing clinicians, radiologists and pathologists have the highest level of agreement in diagnosing IPF.     

特发性肺纤维化的综合诊断进展

特发性肺纤维化是最常见的一种特发性间质性肺炎,组织病理表现为普通型间质性肺炎,临床表现为进行性呼吸困难伴有刺激性干咳,确诊依赖于外科肺活检。但肺活检风险大、费用高,不易被患者接受。近年来研究发现综合HRCT、血清标志物、以及肺功能、支气管肺泡灌洗液等检查,可以对IPF作出早期准确的诊断。本文将从上述几个方面作一介绍。     

Unclassifiable interstitial lung disease: A review

Accurate classification of interstitial lung disease (ILD) requires a multidisciplinary approach that incorporates input from an experienced respirologist, chest radiologist and lung pathologist. Despite a thorough multidisciplinary evaluation, up to 15% of ILD patients have unclassifiable ILD and cannot be given a specific diagnosis. The objectives of this review are to discuss the definition and features of unclassifiable ILD, identify the barriers to ILD classification and outline an approach to management of unclassifiable ILD. Several recent studies have described the characteristics of these patients; however, there are inconsistencies in the definition and terminology of unclassifiable ILD due to limited research in this population. Additional studies are required to determine the appropriate evaluation and management of patients with unclassifiable ILD.     

Changes in the current classification of IIP: A critical review

Idiopathic interstitial pneumonias (IIP) are a heterogeneous group characterized by unknown aetiology. Establishment of a correct diagnosis of a distinct IIP requires a multidisciplinary approach integrating clinical presentation, physiological data, radiological appearance and histological findings. The 2013 update of the American Thoracic Society/European Respiratory Society classification summarises progress in the field of IIP and outlines potential areas for future research. The main entities defined by the 2002 statement on IIP are preserved, but major IIP are now distinguished from rare IIP and the new category of unclassifiable IIP is introduced. In addition, the existence of idiopathic non‐specific interstitial pneumonia as a separate chronic fibrosing IIP and idiopathic pleuroparenchymal fibroelastosis as a specific rare entity are acknowledged. Moreover, the major IIP are categorized according to the features chronic fibrosing, smoking‐related and acute/subacute clinical course. Furthermore, a clinical classification of IIP according to disease behaviour with suggestions for treatment goals and monitoring strategies is provided. The goal of this review is to discuss the areas of uncertainty in the updated multidisciplinary classification of IIP and point out potential consequences for clinical management.     

Do all patients with idiopathic pulmonary fibrosis warrant a trial of therapeutic intervention? A pro‐con perspective

Idiopathic pulmonary fibrosis (IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. However, there were two landmark trials published earlier this year, which have changed the management of this condition. Pirfenidone (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib (Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis‐1 and ‐2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population.     

Significance of lung shrinkage on CXR as a prognostic factor in patients with idiopathic pulmonary fibrosis

OBJECTIVE: The aim of this study was to determine whether the presence of lung shrinkage on CXR can predict diminished survival in patients with idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP). METHODOLOGY: In a hospital-based cohort study 68 subjects diagnosed with IPF/UIP by surgical lung biopsy or at autopsy were observed for a mean of 7.6 years. The radiographic scores from Cherniack's method, pulmonary function tests, arterial blood gas, and haematological data were obtained at initial presentation. Longitudinal radiographic changes over a mean interval of 2.7 years were measured. Survival analysis was performed using Kaplan-Meier and Cox's proportional hazards regression analysis. RESULTS: At some point during the observation period 36 (53%) of 68 patients did not exhibit lung shrinkage and 32 (47%) of 68 patients showed lung shrinkage. Patients with lung shrinkage were more likely to have a diminished survival than those with lung preservation; median survival was 4.4 vs 7.8 years, respectively. Lung shrinkage during the observation period (hazard ratio, 3.89; 95% CI = 1.68-9.01; P= 0.001) was associated with lower rates of survival. CONCLUSION: In patients with IPF/UIP, lung shrinkage on CXR during the observation period was a poor prognostic factor.     

弥漫性肺疾病的疾病谱分析

目的通过对我科收治的471例弥漫性肺疾病的分析,总结其疾病谱的特点,以提高对弥漫性肺疾病的认识。方法统计分析2003年1月1日~2007年12月31日我科收治的471例弥漫性肺疾病患者,根据各种疾病的诊断标准进行归类。结果在471例弥漫性肺疾病患者中,特发性间质性肺炎184例,继发于免疫结缔组织疾病的间质性肺炎109例,特殊类型及其它病种94例,恶性肿瘤44例,感染相关40例。结论广义的间质性肺炎占弥漫性肺疾病的绝大多数,诊断中首先根据临床表现和辅助检查排除免疫结缔组织病,依据病理检查诊断各种特发性间质性肺炎,但应特别注意,约近20%的患者其病因仍是最为常见的肿瘤和感染,仅仅因其表现不典型而被延误诊治。     

Treatment of idiopathic pulmonary fibrosis: Is there anything new?

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic fibrosing interstitial pneumonia of unknown aetiology and is associated with the histological picture of usual interstitial pneumonia. Treatment in most cases is unsatisfactory and the prognosis remains poor. There is insufficient evidence to suggest that any treatment, apart from lung transplantation, improves survival or halts disease progression for IPF patients. Data on treatment response are limited by the paucity of clinical trails, the lack of homogenous clinical features, the small number of patients, and the absence of histological and radiological documentation in many cases. Anti-inflammatory medications such as corticosteroids, azathioprine and cyclophosphamide remain the commonly used medications. More recently, it has been proposed that IPF is a primary fibrotic disease rather than an inflammatory condition. Antifibrotic agents such as colchicine, pirfenidone and interferon-gamma (IFN-gamma) have been tried. However, a recent placebo-controlled trial has failed to demonstrate a significant effect of IFN-gamma on disease progression, lung function or quality of life in IPF patients, though a clinically significant survival benefit of the drug could not be ruled out.