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Inhalational therapy, today, happens to be the mainstay of treatment in obstructive airway diseases (OADs), such as asthma, chronic obstructive pulmonary disease (COPD), and is also in the present, used in a variety of other pulmonary and even non-pulmonary disorders. Hand-held inhalation devices may often be difficult to use, particularly for children, elderly, debilitated or distressed patients. Nebulization therapy emerges as a good option in these cases besides being useful in the home care, emergency room and critical care settings. With so many advancements taking place in nebulizer technology; availability of a plethora of drug formulations for its use, and the widening scope of this therapy; medical practitioners, respiratory therapists, and other health care personnel face the challenge of choosing appropriate inhalation devices and drug formulations, besides their rational application and use in different clinical situations. Adequate maintenance of nebulizer equipment including their disinfection and storage are the other relevant issues requiring guidance. Injudicious and improper use of nebulizers and their poor maintenance can sometimes lead to serious health hazards, nosocomial infections, transmission of infection, and other adverse outcomes. Thus, it is imperative to have a proper national guideline on nebulization practices to bridge the knowledge gaps amongst various health care personnel involved in this practice. It will also serve as an educational and scientific resource for healthcare professionals, as well as promote future research by identifying neglected and ignored areas in this field. Such comprehensive guidelines on this subject have not been available in the country and the only available proper international guidelines were released in 1997 which have not been updated for a noticeably long period of over two decades, though many changes and advancements have taken place in this technology in the recent past. Much of nebulization practices in the present may not be evidence-based and even some of these, the way they are currently used, may be ineffective or even harmful. Recognizing the knowledge deficit and paucity of guidelines on the usage of nebulizers in various settings such as inpatient, out-patient, emergency room, critical care, and domiciliary use in India in a wide variety of indications to standardize nebulization practices and to address many other related issues; National College of Chest Physicians (India), commissioned a National task force consisting of eminent experts in the field of Pulmonary Medicine from different backgrounds and different parts of the country to review the available evidence from the medical literature on the scientific principles and clinical practices of nebulization therapy and to formulate evidence-based guidelines on it. The guideline is based on all possible literature that could be explored with the best available evidence and incorporating expert opinions. To support the guideline with high-quality evidence, a systematic search of the electronic databases was performed to identify the relevant studies, position papers, consensus reports, and recommendations published. Rating of the level of the quality of evidence and the strength of recommendation was done using the GRADE system. Six topics were identified, each given to one group of experts comprising of advisors, chairpersons, convenor and members, and such six groups (A-F) were formed and the consensus recommendations of each group was included as a section in the guidelines (Sections I to VI). The topics included were: A. Introduction, basic principles and technical aspects of nebulization, types of equipment, their choice, use, and maintenance B. Nebulization therapy in obstructive airway diseases C. Nebulization therapy in the intensive care unit D. Use of various drugs (other than bronchodilators and inhaled corticosteroids) by nebulized route and miscellaneous uses of nebulization therapy E. Domiciliary/Home/Maintenance nebulization therapy; publichealth care workers education, and F. Nebulization therapy in COVID-19 pandemic and in patients of other contagious viral respiratory infections (included later considering the crisis created due to COVID-19 pandemic). Various issues in different sections have been discussed in the form of questions, followed by point-wise evidence statements based on the existing knowledge, and recommendations have been formulated.

During the times of the ongoing COVID pandemic, aerosol-generating procedures such as bronchoscopy have the potential of transmission of severe acute respiratory syndrome coronavirus 2 to the healthcare workers. The decision to perform bronchoscopy during the COVID pandemic should be taken judiciously. Over the years, the indications for bronchoscopy in the clinical practice have expanded. Experts at the Indian Association for Bronchology perceived the need to develop a concise statement that would assist a bronchoscopist in performing bronchoscopy during the COVID pandemic safely. The current Indian Association for Bronchology Consensus Statement provides specific guidelines including triaging, indications, bronchoscopy area, use of personal protective equipment, patient preparation, sedation and anesthesia, patient monitoring, bronchoscopy technique, sample collection and handling, bronchoscope disinfection, and environmental disinfection concerning the coronavirus disease-2019 situation. The suggestions provided herewith should be adopted in addition to the national bronchoscopy guidelines that were published recently. This statement summarizes the essential aspects to be considered for the performance of bronchoscopy in COVID pandemic, to ensure safety for both for patients and healthcare personnel.

Background: India has high prevalence of chronic respiratory diseases, especially bronchial asthma and chronic obstructive pulmonary disease (COPD). Both of these are also recognized as risk factors for severity and poor outcome of COVID-19 disease. It is assumed that COVID-19 outbreak as well as an effective lockdown might have affected the incidence and outcome of some of these. Methods: To substantiate above hypothesis, an online survey was mailed to 547 pulmonologists across the country; 314 (54.7%) responses were recorded in the given period. The survey included observations on incidence and outcome of common chronic respiratory diseases such as asthma, COPD and interstitial lung diseases (ILD) in predefined pre-COVID and during COVID period in a total of 24 questions. Results: It was observed that the reduction in incidence of outpatient department cases, acute exacerbations and hospitalization for asthma, COPD and ILD was statistically significant. The outcome of acute exacerbations of these chronic respiratory diseases was analyzed in terms of discharges from hospitals after satisfactory recovery and/ or mortalities. For COPD and ILD, it was not significantly affected during the COVID period. At the same time, a relatively higher proportion of acute asthma mortality was reported. A wide variation in nebulization practices was reported during this period. The doses of inhaled corticosteroids in asthma management were not affected. A wide variety of factors including effective lockdown, better quality of air and regular use of masks were probably responsible for reduced incidence of exacerbations of these chronic respiratory diseases. Conclusion: We conclude that while outpatient department visits, acute exacerbations and hospitalizations were significantly reduced during COVID-19 period, outcome of asthmatics was relatively unfavorable as compared to that of COPD and ILDs.

Nebulized medications are frequently administered in both acute and chronic conditions-viz., management of acute asthma and COPD exacerbations, home maintenance treatment for chronic respiratory diseases like COPD, bronchiectasis, and cystic fibrosis, etc. However, the viral pandemic of COVID-19 has raised concerns on the use of nebulization. This document is intended to provide guidance to physicians on the role and use of nebulization in the current pandemic, based on current evidence and understanding.

Influenza, a common cause of acute respiratory infections, is an important health problem worldwide, including in India. Influenza is associated with several complications; people with comorbidities and the elderly are at a higher risk for such complications. Moreover, the influenza virus constantly changes genetically, thereby worsening therapeutic outcomes. Vaccination is an effective measure for the prevention of influenza. Despite the availability of global guidelines on influenza vaccination in adults, country-specific guidelines based on regional variation in disease burden are required for better disease management in India. With this aim, the Indian Chest Society and National College of Chest Physicians of India jointly conducted an expert meeting in January 2019. The discussion was aimed at delineating evidence-based recommendations on adult influenza vaccination in India. The present article discusses expert recommendations on clinical practice guidelines to be followed in India for adult influenza vaccination, for better management of the disease burden.

Similar to the global scenario, pneumococcal diseases are a significant health concern in India. Pneumococcal diseases occur frequently among adults and are largely preventable through vaccines. Globally, several guidelines and recommendations are available for pneumococcal vaccination in adults. However, owing to wide variations in the disease burden, regulatory landscape, and health-care system in India, such global guidelines cannot be unconditionally implemented throughout the country. To address these gaps, the Indian Chest Society and National College of Chest Physicians of India jointly conducted an expert meeting in January 2019. The aim of the discussion was to lay down specific evidence-based recommendations on adult pneumococcal vaccination for the country, with a view to further ameliorate the disease burden in the country. This article presents an overview of the closed-door discussion by the expert members on clinical practice guidelines to be followed for adult pneumococcal vaccination in India.

Bronchial thermoplasty (BT) is an interventional bronchoscopic treatment for severe asthma. There is a need to define patient selection criteria to guide clinicians in offering the appropriate treatment options to patients with severe asthma. Methodology: An expert group formed this statement under the aegis of the Indian Chest Society. We performed a systematic search of the MEDLINE and EMBASE databases to extract evidence on patient selection and the technical performance of BT. Results: The experts agreed that the appropriate selection of patients is crucial and proposed identification of the asthma phenotype, a screening algorithm, and inclusion/exclusion criteria for BT. In the presence of atypical clinical or chest radiograph features, there should be a low threshold for obtaining a thoracic computed tomography scan before BT. The patient should not have had an asthma exacerbation in the preceding two weeks from the day of the procedure. A 5-day course of glucocorticoid should be administered, beginning three days before the procedure day, and continued until the day following the procedure. General Anesthesia (total intravenous anesthesia with a neuromuscular blocker) provides ideal conditions for performing BT. A thin bronchoscope with a 2.0 mm working channel is preferable. An attempt should be made to deliver the maximum radiofrequency activations. Middle lobe treatment is not recommended. Following the procedure, overnight observation in the hospital, and a follow-up visit, a week following each treatment session, is desirable. Conclusion: This position statement provides practical guidance regarding patient selection and the technical performance of BT for severe asthma.

BackgroundIdentifying risk factors for poor outcomes can help with risk stratification and targeting of treatment. Risk factors for mortality and exacerbations have been identified in bronchiectasis but have been almost exclusively studied in European and North American populations. This study investigated the risk factors for poor outcome in a large population of bronchiectasis patients enrolled in India.MethodsThe European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) and Respiratory Research Network of India (EMBARC-India) registry is a prospective observational study of adults with computed tomography-confirmed bronchiectasis enrolled at 31 sites across India. Baseline characteristics of patients were used to investigate associations with key clinical outcomes: mortality, severe exacerbations requiring hospital admission, overall exacerbation frequency and decline in forced expiratory volume in 1 s.Results1018 patients with at least 12-month follow-up data were enrolled in the follow-up study. Frequent exacerbations (≥3 per year) at baseline were associated with an increased risk of mortality (hazard ratio (HR) 3.23, 95% CI 1.39–7.50), severe exacerbations (HR 2.71, 95% CI 1.92–3.83), future exacerbations (incidence rate ratio (IRR) 3.08, 95% CI 2.36–4.01) and lung function decline. Coexisting COPD, dyspnoea and current cigarette smoking were similarly associated with a worse outcome across all end-points studied. Additional predictors of mortality and severe exacerbations were increasing age and cardiovascular comorbidity. Infection with Gram-negative pathogens (predominantlyKlebsiella pneumoniae) was independently associated with increased mortality (HR 3.13, 95% CI 1.62–6.06), whilePseudomonas aeruginosainfection was associated with severe exacerbations (HR 1.41, 95% CI 1.01–1.97) and overall exacerbation rate (IRR 1.47, 95% CI 1.13–1.91).ConclusionsThis study identifies risk factors for morbidity and mortality among bronchiectasis patients in India. Identification of these risk factors may support treatment approaches optimised to an Asian setting.

Context: Multiple environmental factors are associated with development of hypersensitivity pneumonitis (HP), and diagnostic algorithms for the diagnosis of HP have been proposed in recent perspectives. Aims: We analyzed the data of patients with HP from interstitial lung disease (ILD)-India registry. The analysis was performed to (1) find the prevalence of HP, (2) reclassify HP as per a recently proposed classification criterion to assess the level of diagnostic certainty, and (3) identify the causative agents for HP. Setting and Designs: This was a prospective multicenter study of consecutive, consenting adult patients with new-onset ILD from 27 centers across India (March 2012–April 2015). Materials and Methods: The diagnoses were based on prespecified working clinical criteria and multidisciplinary discussions. To assess strength of diagnosis based on available clinical information, patients with HP were subclassified into definite HP, HP with high level of confidence, and HP with low level of confidence using a recent classification scheme. Results: Five hundred and thirteen of 1084 patients with new-onset ILD were clinically diagnosed with HP and subclassified as HP with high level of confidence (380, 74.1%), HP with low level of confidence (106, 20.7%), and definite HP (27, 5.3%). Exposures among patients with HP were birds (odds ratios [OR]: 3.52, P < 0.001), air-conditioners (OR: 2.23, P < 0.001), molds (OR: 1.79, P < 0.001), rural residence (OR: 1.64, P < 0.05), and air-coolers (OR: 1.45, P < 0.05). Conclusions: About 47.3% of patients with new-onset ILD in India were diagnosed with HP, the majority of whom were diagnosed as HP with a high level of confidence. The most common exposures were birds, cooling devices, and visible molds.

Although a simple and useful pulmonary function test, spirometry remains underutilized in India. The Indian Chest Society and National College of Chest Physicians (India) jointly supported an expert group to provide recommendations for spirometry in India. Based on a scientific grading of available published evidence, as well as other international recommendations, we propose a consensus statement for planning, performing and interpreting spirometry in a systematic manner across all levels of healthcare in India. We stress the use of standard equipment, and the need for quality control, to optimize testing. Important technical requirements for patient selection, and proper conduct of the vital capacity maneuver, are outlined. A brief algorithm to interpret and report spirometric data using minimal and most important variables is presented. The use of statistically valid lower limits of normality during interpretation is emphasized, and a listing of Indian reference equations is provided for this purpose. Other important issues such as peak expiratory flow, bronchodilator reversibility testing, and technician training are also discussed. We hope that this document will improve use of spirometry in a standardized fashion across diverse settings in India.

Background: Predictors of survival for interstitial lung disease (ILD) in the Indian population have not been studied. The primary objective of the study was to assess the Modified-Gender Age and Physiology (M-GAP) score to predict survival in patients with ILD seen in clinical practice. We also analyzed the role of demographic and radiological characteristics in predicting the survival of patients with ILD. Materials and Methods: In the ILD India registry, data were collected from 27 centers across 19 cities in India between March 2012 and June 2015. A single follow-up was conducted at 18 centers who agreed to participate in the follow-up in 2017. M-GAP score (range 0–5) was calculated with the following variables: age (≤60 years 0, 61–65 years 1, and >65 years 2), gender (female 0, male 1), and forced vital capacity% (>75% 0, 50%–75% 1, and >75% 2). A score of 0–3 and score of 4 and 5 were classified into Stage 1 and 2, respectively. Other predictors of survival, such as the history of tuberculosis, smoking, and the presence of honeycombing on computed tomography scan, were also evaluated. Results: Nine hundred and seven patients were contacted in 2017. Among them, 309 patients were lost to follow-up; 399 were alive and 199 had died. M-GAP was significantly associated with survival. Similarly, other predictors of survival were ability to perform spirometry (hazard ratio [HR]: 0.49, 95% confidence interval [CI]: 0.34–0.72), past history of tuberculosis (HR: 1.57, 95% CI: 1.07–2.29), current or past history of smoking (HR: 1.51, 95% CI: 1.06–2.16), honeycombing (HR: 1.81, 95% CI: 1.29–2.55), a diagnosis of connective tissue disease -ILD (HR: 0.41, 95% CI: 0.22–0.76), and sarcoidosis (HR: 0.24, 95% CI: 0.08–0.77). Conclusion: In a subgroup of patients with newly diagnosed ILD enrolled in ILD India registry and who were available for follow-up, M-GAP score predicted survival. Honeycombing at the time of diagnosis, along with accurate history of smoking, and previous history of tuberculosis were useful indices for predicting survival.

Background: Aspiration community-acquired pneumonia (ACAP) and community-acquired pneumonia (CAP) in patients with aspiration risk factors (AspRFs) are infections associated with anaerobes, but limited evidence suggests their pathogenic role. Research Question: What are the aspiration risk factors, microbiology patterns, and empiric anti-anaerobic use in patients hospitalized with CAP? Study Design and Methods: This is a secondary analysis of GLIMP, an international, multicenter, point-prevalence study of adults hospitalized with CAP. Patients were stratified into three groups: (1) ACAP, (2) CAP/AspRF+ (CAP with AspRF), and (3) CAP/AspRF- (CAP without AspRF). Data on demographics, comorbidities, microbiological results, and anti-anaerobic antibiotics were analyzed in all groups. Patients were further stratified in severe and nonsevere CAP groups. Results: We enrolled 2,606 patients with CAP, of which 193 (7.4%) had ACAP. Risk factors independently associated with ACAP were male, bedridden, underweight, a nursing home resident, and having a history of stroke, dementia, mental illness, and enteral tube feeding. Among non-ACAP patients, 1,709 (70.8%) had CAP/AspRF+ and 704 (29.2%) had CAP/AspRF-. Microbiology patterns including anaerobes were similar between CAP/AspRF-, CAP/AspRF+ and ACAP (0.0% vs 1.03% vs 1.64%). Patients with severe ACAP had higher rates of total gram-negative bacteria (64.3% vs 44.3% vs 33.3%, P =.021) and lower rates of total gram-positive bacteria (7.1% vs 38.1% vs 50.0%, P 50% in all groups) independent of AspRFs or ACAP received specific or broad-spectrum anti-anaerobic coverage antibiotics. Interpretation: Hospitalized patients with ACAP or CAP/AspRF+ had similar anaerobic flora compared with patients without aspiration risk factors. Gram-negative bacteria were more prevalent in patients with severe ACAP. Despite having similar microbiological flora between groups, a large proportion of CAP patients received anti-anaerobic antibiotic coverage.

Background and objective In the SENSCIS trial in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), nintedanib reduced the rate of decline in forced vital capacity (FVC) (mL/year) over 52 weeks by 44% in comparison with placebo, with manageable adverse events in most patients. We analyzed the efficacy and safety of nintedanib in patients of Asian race. Methods Patients with SSc-ILD were randomized to receive nintedanib or placebo. The outcomes over 52 weeks were analyzed in Asian versus non-Asian patients. Results Of the 288 patients in each treatment group, 62 (21.5%) in the nintedanib group and 81 (28.1%) in the placebo group were Asian; 90.2% of the Asian patients were enrolled in Asian countries. In the placebo group, the rate of FVC decline over 52 weeks was consistent between Asian and non-Asian patients (−99.9 and −90.6 mL/year, respectively). The effect of nintedanib on reducing the rate of FVC decline over 52 weeks was consistent between Asian (difference, 44.3 mL/year [95% CI: −32.8, 121.4]) and non-Asian patients (difference, 39.0 mL/year [95% CI: −5.1, 83.1]) (treatment-by-time-by-subgroup interaction, p = 0.91). Diarrhea was the most frequent adverse event and was reported in similar proportions of Asian and non-Asian patients in the nintedanib group (80.6% and 74.3%, respectively) and placebo group (28.4% and 32.9%, respectively). Conclusions In patients with SSc-ILD, nintedanib had a consistent benefit on slowing the progression of SSc-ILD in Asian and non-Asian patients, with a similar adverse event profile. Trial registration ClinicalTrials.gov NCT02597933 .

Bronchiectasis is reported to be more frequent in females. There have been no large studies examining the impact of biological sex on disease characteristics, aetiology, severity, quality of life or management. Patients were prospectively enrolled from 2015 -2018 in 3 linked bronchiectasis registries in Australia, Europe and India. Disease information was collected, including quality of life (QoL-B). Analyses were conducted cross-sectionally using baseline data. 13859 patients were included from 29 countries. 57.6% were female. The highest proportion of females were in Australia (70.9%) and lowest in India (43.1%). Male sex was associated with bronchiectasis caused by COPD and TB; while female sex was associated with post-infectious bronchiectasis, asthma, connective tissue disease and NTM. Men had more severe disease as measured by the bronchiectasis severity index, but females more likely to be infected with Pseudomonas aeruginosa. Women had greater disease impact across multiple QoL-B domains including social, emotional and treatment burden domains. Cough and sputum purulence were more severe in women while men most frequently complained of wheeze and breathlessness. Men were significantly less likely to receive evidence based interventions including testing for immunodeficiency and ABPA, long term antibiotic treatment for frequent exacerbators, pulmonary rehabilitation and airway clearance techniques. Conclusion: There are striking sex differences in aetiology, severity, quality of life and treatment in patients with bronchiectasis

Rationale: Interstitial lung disease (ILD) is a heterogeneous group of acute and chronic inflammatory and fibrotic lung diseases. Existing ILD registries have had variable findings. Little is known about the clinical profile of ILDs in India.Objectives: To characterize new-onset ILDs in India by creating a prospective ILD using multidisciplinary discussion (MDD) to validate diagnoses.Methods: Adult patients of Indian origin living in India with new-onset ILD (27 centers, 19 Indian cities, March 2012–June 2015) without malignancy or infection were included. All had connective tissue disease (CTD) serologies, spirometry, and high-resolution computed tomography chest. ILD pattern was defined by high-resolution computed tomography images. Three groups independently made diagnoses after review of clinical data including that from prompted case report forms: local site investigators, ILD experts at the National Data Coordinating Center (NDCC; Jaipur, India) with MDD, and experienced ILD experts at the Center fo...

Introduction: Allergic bronchopulmonary aspergillosis (ABPA) is a severe allergic inflammatory response to Aspergillus fumigatus and is often an unrecognized cause for bronchiectasis. Aims and Objectives: In this study, we aim to examine the burden of ABPA in the India bronchiectasis registry, the screening test perform and the phenotype characterization. Material and Methods: Among the 2197 cases of bronchiectasis enrolled in the Indian bronchiectasis registry, we examined the number of patients screened for ABPA and the proportion of screened positive ABPA cases. We also looked for phenotypic characterization using HRCT features, total IgE, eosinophil count, IgE and IgG specific to aspergillus and by skin prick test. Results: Out of 2197 cases of bronchiectasis cases register at 31 sites, only 387 (17%) cases were screened for ABPA 190 (49.1%) at 24 sites. Among these 190 cases, 99(52.1%) of all ABPA cases were male (mean age 44.85 ± 15.09 yr). 62.03% had the bronchiectasis for more than five years. Serum eosinophil count was raised in 115(60.5%) out of 146 (76.84%) cases. Raised IgG level was observed in 71 (74.74%) out of 95 (50%) cases. Specific IgE to aspergillus was raised in 117 (84.17%) out of 139 (73.16%) cases. Aspergillus skin prick test was tested positive in 83 (83%) out of 100 (52.63%) cases. Total IgE count was raised in 97 (54.19%) cases out of 179 (94.2%). Obstructive airways disease was found in 74 (52.5%) out of 141 (74.21%) cases tested by spirometry. The right middle lobe was involved in 66.32% cases in HRCT. Conclusion: Despite the universal screening for ABPA recommended by the ERS, only 17% were screened for ABPA of which 50% showed evidence of underline ABPA.

Cartridge based nucleic acid amplification test (CBNAAT) is used widely to detect Mycobacterium Tuberculosis infection. It detects DNA sequences specific for Mycobacterium Tuberculosis and rifampicin resistance by polymerase chain reaction. The reults are available in two hours, making it the fastest method to detect Tuberculosis and rifampicin resistance. It has shown to have high sensitivity and specificty in smear positive pulmonary tuberculosis (98%and98% respectively) and a resonable result in smear negative cases (67%and99% respectively). Tubercular pleural effusion is the second most common extrapulmonary tuberculosis. The diagnosis is mainly based on pleural fluid or pleural biopsy histopathology, pleural biopsy histopathology being considered as gold standard for diagnosis. At our center, utility of CBNAAT was evaluated on pleural biopsy samples obtained thoracoscopically. Thoracoscopy was performed in patient with indeterminate pleural effusions and multiloculated pleural effusions. The biposy sample obtained was subjected to Histopathology and CBNAAT. As our center is recognised by Government of India for programme “Universal Access for Tuberculosis Care”, the CBNAAT was done free of cost. Considering histopathology as gold standard, sensitivity and specificity of CBNAAT was calculated.

Introduction: We present preliminary data from the multicenter prospective Indian Sleep Registry which started patient enrollment in July 2018. Data collection embraces areas like epidemiology, etiology, co-morbidities and management strategies and will help identify Phenotypes of Sleep Apnea (SA) Aims and Objective: Formulation of a cross sectional database for defining SA in India Methods: Registry will collect online data from more than 20 sites (hospitals, research centers, institutes). Cases will be entered once at the baseline & after one year. Sites are being trained for data entry, to maintain the quality of data. Sites share the raw data on the online portal at the National Data Collection Centre (NDCC) to ensure uniformity in diagnosis. Data collection will be ongoing for 5years Results: So far, 6 sites have contributed 258 cases from all regions of the country (image1). 204 male (79%) & 54 (21%) females. Mean age 50.78 (+13.60), majority belonged to affluent section of the society. 55.81% patients were obese, 29.45% were overweight. A weak positive correlation was found between severity of OSA and STOP BANG questionnaire (r = 0.012), OSA and Epworth Sleepiness Scale(r = 0.013) at p Conclusion: This is the first effort to build a National Sleep Registry in South Asia. Males with higher BMI and higher socioeconomic status are at risk for SA in Indian population. Daytime sleepiness and screening tool seem to be weak predictors. The Indian sleep registry will play an important role in characterizing and phenotyping SA in this country.

Patient: Female, 36 Final Diagnosis: Mediastinal cystic hygroma Symptoms: Chest discomfort Medication: — Clinical Procedure: — Specialty: Pulmonology Objective: Unusual clinical course Background: Lymphangioma is an atypical non-malignant, lymphatic lesion that is congenital in origin. Lymphangioma is most frequently observed in the head and neck, but can occur at any location in the body. About 65% of lymphangiomas are apparent at birth, while 80–90% are diagnosed by two years of age. Occurrence in adults is rare, as evidenced by less than 100 cases of adult lymphangiomas reported in the literature. Case report: A 36-year-old Indian woman with a medical history of recurrent pleural effusions presented with chief complaints of dyspnea on exertion for one year and a low-grade fever for one month. A thorax CT revealed left-sided pleural effusion with thin internal septations. Thoracoscopy revealed a large cystic lesion arising from the mediastinum from the hilum surrounding the mediastinal great vessels. The diagnosis of lymphangioma was confirmed via histopathologic examination of the cyst. It was managed with partial cystectomy along with the use of a sclerosing agent (talc). Conclusions: The size and location of lymphangiomas can vary, with some patients presenting with serious problems like respiratory distress, while others may be asymptomatic. Complete cyst resection is the gold standard treatment for mediastinal cystic lymphangioma. Partial cyst resection along with the use of sclerosing agents can be an effective option when complete cystectomy is not possible. Although lymphangioma is a rare patient condition, it should be included in the differentials for patients presenting with pleural effusions. Also, a biopsy should be done at the earliest opportunity to differentiate lymphangioma from other mediastinal malignant tumors.

Introduction: Bronchiectasis is a heterogeneous disease. Previous phenotyping of this disease has been based solely on 5 European databases which do not really account for the remaining world population. Aims and Objectives: This study looked at the Indian EMBARC Registry to try and identify discrete patient groups with different clinical and biological characteristics. Methodology: Principal component and cluster analyses were performed using demographics, co-morbidities, clinical, radiological, physiological and microbiological variables collected during the stable disease state. Results: The data from 1368 prospectively enrolled outpatients from 33 centres in India from the EMBARC India Registry were included for the cluster analysis. Among these patients (mean age 54 years, 60% men) six clusters were identified. Our clusters were driven by patient demographics (age and sex), BMI (median BMI 21.48), aetiology of Bronchiectasis (Idiopathic 22.9%, post TB 34.3%, Post infectious 21.3%, ABPA 9.9%) Lung function (mean FEV1 53.36%, FVC 63.71%). Patients in the six clusters showed significant differences in terms of quality of life, exacerbations and hospitalisations. This is in complete contrast to the European cohort where four clusters were identified driven by the presence of chronic infection with Pseudomonas Aeruginosa or other pathogens and daily sputum production. Conclusion: Hence this is the first effort to define discrete phenotypes of Bronchiectasis patients in an Asian population. Identification of six clinical phenotypes in Bronchiectasis could favour focused treatments in future interventional studies designed to alter the natural history of the disease.

Introduction and Methods: The European Multicenter Bronchiectasis Audit and Research Collaboration (EMBARC) network established across 23 European countries was extended to India to explore unique features of Non-cystic fibrosis Bronchiectasis in this sub-continent. 34 investigator sites located across different parts of India contributed to 2062 cases of Non-CF Bronchiectasis, of which post-tuberculosis lung disease was identified as the biggest risk factor contributing to 34.7% (N=717) cases. The aim of this study was to describe the unique features of Post-TB Bronchiectasis observed on HRCT in this Indian cohort. Results: The mean (± SD) age of the 717 cases of Post-TB Bronchiectasis was 54.9 ± 14 yrs with a M:F ratio of 60:40. The location of Bronchiectasis was 71% (67.7,74.3) in the upper lobes, 66% (62.5,69.5) in the middle lobe/lingula and 60% (56.4,63.6) in the lower lobes. The cystic type was the most common 56% (53.1,60.4), followed by cylindrical 46% (42.3,49.6) and varicose 8.9% (6.8,10.9). More than 43.8% had bilateral disease. 13.6% (11.3,16.3) of the cases presented with hemoptysis that required hospitalization. Sputum microbiological samples collected in 39.8% of the cases isolated Pseudomonas in 17.1% during stable disease and 18.9% during exacerbations, while Klebsiella was isolated in 4.5% and 16.0% of cases respectively. Myco TB was isolated in the sputum in 5% of cases. 39.5% of the subjects had FEV1/FVC values Conclusion: Post TB lung disease is the commonest cause for Non CF Bronchiectasis in India and presents with unique features that are distinct from those reported in the western world.

Background: The Indian Bronchiectasis registry is an extension of EMBARC Registry started in 2015 by Respiratory Research Network of India to get better insights into its demographics, aetiology, co-morbidities and treatment practices in India. Methods: 18 investigator sites of the RRNI across India completed a web-based registry case report comprising of demographic details, risk factors, aetiology, HRCT, spirometry and treatment. Results: 964 patients of non-CF bronchiectasis were enrolled in this registry, of which 581(60.27%) were males. The mean age of participants was 52.59±15.32 years. The commonest pattern on HRCT was Cystic Bronchiectasis (66%) followed by cylindrical(41.2%) and varicose(12.7%) types. Cardiovascular disease was the commonest comorbidity(17%) followed by diabetes(15.8% ). The commonest aetiology for Bronchiectasis was Post TB(32.16%), followed by Idiopathic(23.5%) and post Infective(22.5%). ABPA related Bronchiectasis was seen in 11.4% cases. The quality of life (MRC Dyspnoea scale) was worst when the aetiology was TB(MRC≥3= 65%). Majority were never-smokers(70.6%) with only 5.7% being current smokers. The commonest inhaled prescriptions were ICS/LABA combination(71.75%) followed by Tiotropium(22.54%) and LABA(22.11%). Only 241 patients were prescribed antibiotics, of which 55.19% patients received low-dose macrolides(Azithromycin-31.1%), while cyclical antibiotic therapy was given to 29.4% patients. The commonest non-respiratory prescription was PPI(33%) followed by a statin(8.6%). 41.90% patients received pneumococcal vaccine. 44.09% received Influenza vaccine in last 1 year. Conclusions: EMBARC India Registry offers novel insights into non- CF Bronchiectasis in India.

Endobronchial leiomyomas belong to a rare group of endobronchial tumors accounting for less than 2% of endobronchial tumors. The majority of lung leiomyomas are parenchymal (51%), endobronchial (33%) and endotracheal (16%). Literature regarding leiomyoma is scarce with very few cases being reported. These tumors arise from smooth muscle. Two different rare clinical presentations of lung leiomyoma were observed at a same respiratory clinic and is being reported. Case 1:Non HIV female aged 42 years presented with features of acute interstitial pneumonia. When subjected for bronchoscopy it revealed multiple endobronchial swellings. Histopathological examination of these lesions showed features of leiomyoma & immunohistochemistry demostrating diffuse cytoplasmic reactivity for smooth muscle actin and desmin. Case 2:Non HIV young male aged 18 years presented with complete collapse of right lung. He was suspected to be a case of foriegn body in right main bronchus. Bronchoscopy was done. It showed presence of hard endobronchial growth at carina causing complete obstruction of right main bronchus. Biopsy of the mass showed presence of smooth muscle neoplasm confirmed by immunohistochemistry as leiomyoma. The mass appered to be originating from smooth muscle of right pulmonary artery, hitheto unreported in literature. These two cases of leiomyoma being reported from same centre had different rare presenting features of acute interstitial pneumonia and being originating from smooth muscle of right pulmonary artery respectively. The cases are also unique as they were not associated with HIV and were primarily in respiratory tract not associated multiple leiomyomatosis.

Background: Bronchoscopic lung cryobiopsy (BLC) is a novel technique for obtaining lung tissue for the diagnosis of diffuse parenchymal lung diseases. The procedure is performed using several different variations of technique, resulting in an inconsistent diagnostic yield and a variable risk of complications. There is an unmet need for standardization of the technical aspects of BLC. Methodology: This is a position statement framed by a group comprising experts from the fields of pulmonary medicine, thoracic surgery, pathology, and radiology under the aegis of the Indian Association for Bronchology. Sixteen questions on various technical aspects of BLC were framed. A literature search was conducted using PubMed and EMBASE databases. The expert group discussed the available evidence relevant to each question through e-mail and a face-to-face meeting, and arrived at a consensus. Results: The experts agreed that patients should be carefully selected for BLC after weighing the risks and benefits of the procedure. Where appropriate, consideration should be given to perform alternate procedures such as conventional transbronchial biopsy or subject the patient directly to a surgical lung biopsy. The procedure is best performed after placement of an artificial airway under sedation/general anesthesia. Fluoroscopic guidance and occlusion balloon should be utilized for positioning the cryoprobe to reduce the risk of pneumothorax and bleeding, respectively. At least four tissue specimens (with at least two of adequate size, i.e., ≥5 mm) should be obtained during the procedure from different lobes or different segments of a lobe. The histopathological findings of BLC should be interpreted by an experienced pulmonary pathologist. The final diagnosis should be made after a multidisciplinary discussion. Finally, there is a need for structured training for performing BLC. Conclusion: This position statement is an attempt to provide practical recommendations for the performance of BLC in DPLDs.