Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Its signs and symptoms are relatively non-specific, and patients often present with chronic cough, progressive dyspnea, resting or exertional hypoxemia, and inspiratory crackles on lung auscultation. Definitive diagnosis requires the exclusion of known causes of pulmonary fibrosis and identification of the usual interstitial pneumonia (UIP) pattern of disease either on high-resolution computed tomography (HRCT) scan of the chest or on surgical lung biopsy. Multidisciplinary discussion involving pulmonologists, radiologists, and pathologists with expertise in the diagnosis of IPF and other forms of interstitial lung disease is recommended and often required. Management focuses on anti-fibrotic therapy and early referral to lung transplant centers for those who are candidates. This review will discuss the current recommendations for the diagnosis, prognostication, and management of patients with IPF.

To assess characteristics and outcomes of patients hospitalized with interstitial lung diseases (ILD) and to analyze patient's comorbidities, procedures, and in-hospital outcomes.We identified patients hospitalized with idiopathic pulmonary fibrosis and others ILD such as hypersensitivity pneumonitis, cryptogenic organizing pneumonia, lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, and sarcoidosis in Spain during 2014 and 2015.We identified 14,565 discharges among patients admitted for ILD in Spain during the study period: idiopathic pulmonary fibrosis (IPF) in 42.32% (n = 6164), sarcoidosis in 37.65% (n = 5484), hypersensitivity pneumonitis in 10.55% (n = 1538), cryptogenic organizing pneumonia in 7.06% (n = 1028), pulmonary Langerhans cell histiocytosis in 1.48% (n = 215), and lymphangioleiomyomatosis in 0.94% (n = 136). The most common associated comorbidities according to those included in the Charlson Comorbidity Index (CCI) were COPD, diabetes, and congestive heart disease. The presence of pulmonary hypertension increased the probability of dying in patients with idiopathic pulmonary fibrosis (OR 1.36; 95%CI 1.06-1.73). Patients with cryptogenic organizing pneumonia had the longest length of hospital stay and the highest percentage of hospital readmissions (23.64%). The highest IHM corresponded to the idiopathic pulmonary fibrosis (14.94%). Computed tomography of the chest was the procedure more used during admissions for ILD.IPF was responsible for larger percentage of hospital admission among ILD in our study. In addition, the IHM were higher in IPF patients in comparison with those with other ILD. The most common associated comorbidity in ILD according to those included in the CCI was COPD. Computed tomography of the chest was the procedure more frequently used.

BACKGROUND The aim of this study was to assess the association between pulmonary vessel-related structures and cryptogenic fibrosing alveolitis (CFA) in a drug trial in a Chinese population using derived computed tomography (dCT) to evaluate functional reduction and survival. MATERIAL AND METHODS Discovery and validation cohorts were chosen separately by fulfilment of drug trial entry criteria, and we enrolled 269 and 292 consecutive patients, respectively. CFA patients who had undergone imaging based on volumetric non-contrast CT at our hospital were subjected to pulmonary vessel-related structure (PVS) measures and dCT to forecast mortality and reduction in reduced forced vital capacity of CFA. RESULTS The best forecaster of survival and reduction in terms of reduced forced vital capacity were found to be the dCT-generated outcomes in terms of PVS scores. Patients having less extensive disease highlighted the dCT outcomes through outperformance of CFA measures. When we used the cohort enhancement device, we found reduction in the requisite sample size of a CFA drug trial by 31% with the use of more than 5.0% dCT PVS score. CONCLUSIONS We found an association between CFA and PVS using dCT and it is far better than the results achieved so far by use of criterion standard measures. Additionally, reduction in the restrictive trial costs was also achieved by using cohort enhancement in a CFA drug trial setting, as PVS scores forced us to decrease the size of required CFA drug trial population by 30%. Interestingly, patients who had to take antifibrotic medication for longer periods had longer survival and less decreases forced vital capacity, as identified by PVS scores.

Objective Human intelectin-1 (hITLN-1) binds to galactofuranosyl residues, which are present in the microbial cell wall, but which are absent in mammalian tissues, and has been suggested to play an immunological role against microorganisms. However, the involvement of hITLN-1 in the pathogenesis of diffuse pulmonary diseases remains unknown. The aim of this study was to compare the hITLN-1 concentrations in the bronchoalveolar lavage (BAL) fluid of patients with diffuse pulmonary diseases. Methods The cell components and concentrations of hITLN-1 were analyzed in the BAL fluid of 8 patients with idiopathic chronic eosinophilic pneumonia (ICEP), 3 patients with drug-induced eosinophilic pneumonia, 4 patients with hypersensitivity pneumonitis (HP), 11 patients with sarcoidosis, 9 patients with cryptogenic organizing pneumonia, and 5 patients with idiopathic fibrosing interstitial pneumonia (fibrosing nonspecific interstitial pneumonia or usual interstitial pneumonia). Results The hITLN-1 concentrations in the BAL fluid of patients with ICEP and HP were higher than in those with other diseases. In the ICEP group, no significant difference was observed in the hITLN-1 concentrations of patients with or without a history of bronchial asthma. Conclusion The results of the present study suggest that hITLN-1 may be involved in the pathogenesis of ICEP and HP, and that an increase in the hITLN-1 concentration in the BAL fluid may represent a new biomarker for these diseases.

Objective: To assess the efficacy and safety of transbronchial cryobiopsy (TBCB) for the etiologic evaluation of diffuse lung disease (DLD). Methods: Between December 2015 to April 2017, a total of 38 patients with DLD met the inclusion criteria for TBCB in the First Affiliated Hospital of Chongqing Medical University, and 35 of them consented to undergo the procedure under rigid or flexible bronchoscopy. On the tissues obtained from the 35 patients, histopathologic and microbiological evaluations were performed, and together with clinical and radiological manifestations, diagnoses were made and the efficacy of TBCB in the diagnosis of DLD was confirmed, and then therapies were planned accordingly. Complications of the biopsy procedures were recorded. Results: Of the 35 patients who were enrolled, 24 underwent TBCB under rigid bronchoscopy and 11 under flexible bronchoscopy. Another 3 patients refused the procedure due to disinclination to invasive examinations. One single procedure of TBCB took (51.8±19.2) min on average, the median number of tissues obtained was 6 (5, 8), and the median area of tissues was 15 (9, 20) mm(2).Definite diagnoses were reached in 33 patients, including idiopathic nonspecific interstitial pneumonia (n=8), connective tissue disease-interstitial lung disease (n=8), occupational lung disease (n=4), idiopathic pulmonary fibrosis (n=3), interstitial pneumonia with autoimmune features (n=3), tuberculosis (n=2), cryptogenic organization pneumonia (n=1), acute interstitial pneumonia (n=1), pulmonary infection (n=1), hypersensitivity pneumonia (n=1) and sarcoidosis (n=1). Diagnostic yield was 94.3% (33 out of 35 cases diagnosed). Pneumothorax occurred in 3 patients (1 patients with mild pneumothorax , 1 moderate and 1 severe), and were resolved with thoracic puncture or pleural drainage. Bleeding occurred in all 24 patients who received TBCB under rigid bronchoscopy (11 patients with mild bleeding, 12 moderate and 1 severe) and was controlled after coagulation measures. After one month of treatment according to the diagnoses acquired with cryobiopsy, the condition was cured in 1 patient (3.0%), alleviated in 17 (51.5%), stable in 11 (33.3%), and deteriorated in 4 (12.1%). Conclusion: TBCB yields reliable diagnoses with a good safety profile.

CT scanning of the chest is one of the most important imaging modalities available to a pulmonologist. The advent of high-resolution CT scanning of the chest has led to its increasing use. Although chest radiographs are still useful as an initial test, their utility is limited in the diagnosis of lung diseases that depend on higher resolution images such as interstitial lung diseases and pulmonary vascular diseases. Several metaphoric chest CT scan signs have been described linking abnormal imaging patterns to lung diseases. Some of these are specific to a disease, whereas others help narrow the differential diagnosis. Recognizing these imaging patterns and CT scan signs are thus vitally important. In the present article, we describe a comprehensive list of the commonly encountered metaphoric chest CT scan signs and their clinical relevance.

Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is one of a spectrum of idiopathic interstitial pneumonia. IPF is an increasingly common condition which poses many diagnostic and therapeutic challenges leading to misdiagnosis and mismanagement. We presented a 55-year-old male textile trader who was initially managed as sputum-negative pulmonary tuberculosis before histology report. He presented to our clinic with Breathlessness and cough of 3 years and 2.5 years, respectively. He had commenced anti-tuberculosis two months before presentation without significant relief. General Physical examination and vital signs were essentially normal. SPO2 was 96% on room air. Chest Examination revealed end-inspiratory bi-basal velcro-like crackles. Other systemic examinations were normal. Radiological examination by way of chest X- ray and chest CT showed features suggestive of IPF. The patient also had open Lung biopsy for histology and spirometry which demonstrated restrictive ventilatory function pattern. A diagnosis of Interstitial lung disease probably Idiopathic Pulmonary Fibrosis was entertained. He was commenced on Tab prednisolone, Tab Rabeprazole, with minimal improvement. IPF have often been misdiagnosed and treated as pulmonary tuberculosis with unfavorable outcome.

Multidisciplinary concertation is mandatory in order to assess interstitial pneumonias. The study of the bronchoalveolar lavage helps evoking a diagnosis according to the lavage profile. In lymphocytic alveolitis, immunocytochemistry, or in flux cytometry are necessary in order to identify the different clusters of lymphocytes implicated. Our objective was to evaluate the profile of 31 lymphocytic alveolitis using 2 different techniques which are the immunocytochemistry and the in flow cytometry in order to evaluate the efficacy of each technique and to compare the different results to the final diagnoses. We describe a retrospective study about 31 patients admitted to our hospital in order to explore an interstitial pneumonia between January and July 2014. Bronchial endoscopy and bronchoalveolar lavage were performed in all cases. The sensitivity of the in flow cytometry was estimated to 53% and its specificity reached 33%. On the other hand, the immunocytochemistry presented a specificity of 42.8% and a sensitivity of 42.8%. The final diagnoses retained consisted in sarcoidosis in 12 cases, infectious pneumonia in 10 cases, hypersensitivity pneumonia in 3 cases, cryptogenic pneumonia in 3 cases, idiopathic fibrosis in 2 cases, and adenocarcinoma in 1 case. The relevance of both techniques depends on many factors. They necessitate an available material, well-trained technicians, and experimented pathologists.

Although bronchoalveolar lavage (BAL) is often used in the diagnosis of interstitial lung diseases (ILDs), its importance in investigating, in particular, idiopathic pulmonary fibrosis (IPF) is controversial. The cell distributions in the BAL are taken into account in the clinical routine, non-cellular characteristics of the BAL play no role.Using mathematical modeling of data, the present work investigated the extent to which BAL features enable drawing conclusions about the underlying ILK or help exclude IPF. Included in the calculation are cellular findings of the BAL, in addition the protein and albumin content of the BAL, the nicotine history (pack years), and spirometry (FEV1, IVC).Using linear discriminant analysis and creating classification trees, the relevance of the characteristics of 806 patients with ILK was examined (183 IPF, 191 cryptogenic organizing pneumonia, 147 lung involvement in autoimmune disease, 97 respiratory bronchiolitis interstitial lung disease, 118 extrinsic allergic alveolitis, 41 lymphocytic interstitial pneumonia (LIP), 23 non-specific interstitial pneumonia (NSIP), 88 controls).There was a close positive relationship between protein levels and lymphocytes in the group as a whole. No such correlations were seen in IPF and NSIP. Albumin was closely correlated with the protein content in all groups.The lymphocytes are best suited to distinguish between different ILDs. Yet, a reliable calculation of the ILD is not possible on the basis of the investigated factors, the classification error ranged from 23.5 % (IPF) to 100 % (LIP, NSIP).Constellations that likely (> 99 %) speak against an IPF are lymphocytosis > 34 % or protein content > 347 mg/l. The same applies to the constellation: lymphocytes > 25 % together with protein > 250 mg/l.In ILD, BAL findings can narrow the diagnosis, but they are seldom diagnostic. BAL can make an important contribution to excluding of IPF.

Interstitial lung diseases (lLD) are a large group of rare diseases, with difficult diagnosis and management. Very little is known about prevalence, diagnosis and management of ILDs in Romania. This study aims to gather information on how ILDs are diagnosed and managed in Romania, focusing on a tertiary hospital with expertise and equipment needed for accurate diagnosis. We analyzed retrospectively the files of patients admitted with ILD in 2011 in "Marius Nasta" Institute of Pulmonology Bucharest. There were 178 eligible patient files with ILDs and 186 sarcoidosis cases. The ILD diagnosis were: 41 cases idiopathic pulmonary fibrosis (IPF), collagen disease associated ILD (29 cases), hypersensitivity pneumonia (19 cases), alveolar proteinosis (9 cases), cryptogenic organizing pneumonia (9 cases), undefined ILD (46 cases), other (25 cases). The investigations used for the diagnosis were: chest X-ray (100%), spirometry (157pts, 88.21%), diffusion capacity (127 pts, 71.43%, broncho-alveolar lavage (92 pts, 51.69%), CT scan (141 pts, 79.22%), lung biopsy (26 pts, 14.6%), similar to other European centers, but fewer lung biopsies are performed. There is need for a prospective registration of ILD cases in a national registry, for creating local guidelines for diagnosis of ILDs, to improve the suspicion of ILD and referring of patients to specialized centers. Diagnosis can be improved by a multidisciplinary approach of each case, involving the clinician, the radiologist, the pathologist and the thoracic surgeon.

Bronchoalveolar lavage (BAL) is a useful technique for differential diagnosis of various interstitial lung diseases (ILDs) and is usually realized by analysis of the differential cell count. This study was conducted to estimate the value of bronchoalveolar lavage fluid (BALF) total cell count (TCC) in the diagnosis of ILD. We analyzed 237 BAL samples from patients with ILD: sarcoidosis (SA), idiopathic pulmonary fibrosis (IPF), cryptogenic organizing pneumonia (COP), hypersensitivity pneumonitis (HP), chronic eosinophilic pneumonia (CEP), and smoking-related ILD (sr-ILD). The control group consisted of 30 healthy volunteers. The statistical analysis revealed significant differences in the BALF TCC between healthy controls and patients with SA, IPF, HP, COP, sr-ILD, and eosinophilic disorders (mean values 6.9 vs. 14.5, 22.5, 22.8, 20.7, 64.5, and 27.3 × 10(6), respectively). Logistic regression revealed a significant relation between the TCC and ILD diagnosis. We conclude that the TCC, as well as the value of total number of inflammatory cells, should be reported in the description of BAL.

The interstitial lung diseases are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the pulmonary interstitium. In 2002, the American Thoracic Society and the European Respiratory Society revised the classification of interstitial lung diseases and introduced the term diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are a subtype of diffuse parenchymal lung disease. The idiopathic interstitial pneumonias are subdivided into usual interstitial pneumonia (with its clinical counterpart idiopathic interstitial pneumonia), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis interstitial lung disease, and lymphocytic pneumonia. Sarcoidosis and hypersensitivity pneumonitis are the 2 most common granulomatous diffuse parenchymal lung diseases. Rheumatoid arthritis, systemic sclerosis, and dermatomyositis/polymyositis (causing antisynthetase syndrome) are diffuse parenchymal lung diseases of known association because these conditions are associated with connective tissue disease. Hermansky-Pudlak syndrome is a rare genetic diffuse parenchymal lung disease characterized by the clinical triad of pulmonary disease, oculocutaneous albinism, and bleeding diathesis. This review provides an overview of the chronic fibrosing diffuse parenchymal lung diseases. Its primary objective is to illuminate the clinical challenges encountered by clinicians who manage the diffuse parenchymal lung diseases regularly and to offer potential solutions to those challenges. Treatment for the diffuse parenchymal lung diseases is limited, and for many patients with end-stage disease, lung transplantation remains the best option. Although much has been learned about the diffuse parenchymal lung diseases during the past decade, research in these diseases is urgently needed.