Clubbing in patients with fibrotic interstitial lung diseases.

Clubbing is associated with poor prognosis and is variably present in patients with idiopathic pulmonary fibrosis (IPF), but is also seen in other fibrotic interstitial lung diseases (ILDs). Little is known about the best methodology to assess clubbing in ILDs and, hence, the prevalence and clinical utility and clinical significance of clubbing. We therefore aimed to evaluate the agreement between different clubbing assessment methods in patients with fibrotic ILDs. Additionally, we assessed the prevalence of clubbing in different fibrotic ILDs and related clubbing to disease severity and quality of life. Consecutive outpatients with fibrotic ILDs of two tertiary referral centers were included. Clubbing was assessed with the phalangeal depth ratio, the digital index, the Schamroth sign test, and by the treating physicians and investigator. We included 153 patients (100 men), mean age 65 (range 33-88), mean FVC 79% (25-145%), mean TLCOc 50% (16-104%). Different methods for assessment of clubbing had poor correlation, and as a result, clubbing prevalence varied according to the method used, ranging from 7 to 42% in the total group of patients and 7-52% in IPF. The degree of clubbing did not correlate with FVC or TLCOc (p > 0.2) or with quality of life scores, but lower mean TLCOc scores were seen in patients with clubbing than in those without. Clubbing was present in 7-42% of our fibrotic ILD cohort and showed no correlation with disease severity. Although considered an important clinical feature, assessment methods for clubbing showed no to poor agreement. Further studies are therefore needed to gain more insight into measuring clubbing reliably and the possible prognostic value and evolution of clubbing.

van Manen MJG ，Vermeer LC ，Moor CC ，Vrijenhoeff R ，Grutters JC ，Veltkamp M ，Wijsenbeek MS ... -  《-》

Spectrum of interstitial lung disease from a tertiary care hospital in Karachi.

Sarwar Zubairi AB ，Hassan M ，Shahzad T ，Sarwar S ，Abbas A ，Ahmad H ，Irfan M ... -  《JOURNAL OF THE PAKISTAN MEDICAL ASSOCIATION》

Interstitial Lung Diseases in Developing Countries.

More than 100 different conditions are grouped under the term interstitial lung disease (ILD). A diagnosis of an ILD primarily relies on a combination of clinical, radiological, and pathological criteria, which should be evaluated by a multidisciplinary team of specialists. Multiple factors, such as environmental and occupational exposures, infections, drugs, radiation, and genetic predisposition have been implicated in the pathogenesis of these conditions. Asbestosis and other pneumoconiosis, hypersensitivity pneumonitis (HP), chronic beryllium disease, and smoking-related ILD are specifically linked to inhalational exposure of environmental agents. The recent Global Burden of Disease Study reported that ILD rank 40th in relation to global years of life lost in 2013, which represents an increase of 86% compared to 1990. Idiopathic pulmonary fibrosis (IPF) is the prototype of fibrotic ILD. A recent study from the United States reported that the incidence and prevalence of IPF are 14.6 per 100,000 person-years and 58.7 per 100,000 persons, respectively. These data suggests that, in large populated areas such as Brazil, Russia, India, and China (the BRIC region), there may be approximately 2 million people living with IPF. However, studies from South America found much lower rates (0.4-1.2 cases per 100,000 per year). Limited access to high-resolution computed tomography and spirometry or to multidisciplinary teams for accurate diagnosis and optimal treatment are common challenges to the management of ILD in developing countries.

Rivera-Ortega P ，Molina-Molina M 《Annals of Global Health》

Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis.

Fisher JH ，Kolb M ，Algamdi M ，Morisset J ，Johannson KA ，Shapera S ，Wilcox P ，To T ，Sadatsafavi M ，Manganas H ，Khalil N ，Hambly N ，Halayko AJ ，Gershon AS ，Fell CD ，Cox G ，Ryerson CJ ... -  《BMC Pulmonary Medicine》

Evaluation of progressive pulmonary fibrosis in non-idiopathic pulmonary fibrosis-interstitial lung diseases: a cross-sectional study.

Progressive pulmonary fibrosis is the symptomatic, physiological, and radiological progression of interstitial lung diseases. The aim of this study was to examine the relationship between progressive pulmonary fibrosis and demographic characteristics and to evaluate the effect on clinical outcomes and mortality. This cross-sectional study included 221 patients diagnosed with non-idiopathic pulmonary fibrosis interstitial lung diseases who were followed in the last 5 years. Patient symptoms, clinical, radiological, and demographic data were examined. Risk factors for the development of progressive pulmonary fibrosis and the relationship with clinical outcomes and mortality were examined. Of the patients, 33.0% (n = 73) had fibrotic idiopathic nonspecific interstitial pneumonia (iNSIP), 35.7% (n = 79) had fibrotic hypersensitivity pneumonia (HP), 18.1% (n = 40) had fibrotic connective tissue disease (CTD) interstitial lung diseases (ILD), and 13.1% (n = 29) had postinfectious fibrotic ILD. The progressive pulmonary fibrosis development rates of the subtypes were 46.5% iNSIP (n = 34), 86.0% fibrotic HP (n = 68), 42.5% fibrotic CTD-ILD (n = 17), and 20.7% postinfectious ILD (n = 6). The presence of progressive pulmonary fibrosis was associated with the development of respiratory failure and mortality (odds ratio [OR]: 2.70, 95% CI: 1.04-7.05 and OR: 2.13, 95% CI: 1.23-3.69). Progressive pulmonary fibrosis development was higher in hypersensitivity pneumonia patients with farmer's lung (OR: 5.06, 95% CI: 1.02-25.18). Progressive pulmonary fibrosis was more prevalent in older patients. Farming was an important risk factor in the development of hypersensitivity pneumonia-progressive pulmonary fibrosis. Respiratory failure and mortality were higher in those who developed progressive pulmonary fibrosis.

Erçen Diken Ö ，Güngör Ö ，Akkaya H 《BMC Pulmonary Medicine》