Clinical outcomes of bronchiectasis in India: data from the EMBARC/Respiratory Research Network of India registry.

BACKGROUND: Identifying 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. METHODS: The 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. RESULTS: 1018 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 (predominantly Klebsiella pneumoniae) was independently associated with increased mortality (HR 3.13, 95% CI 1.62-6.06), while Pseudomonas aeruginosa infection 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). CONCLUSIONS: This 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.

COPD in Biomass exposed nonsmokers: a different phenotype.

INTRODUCTION: Chronic obstructive pulmonary disease, most commonly caused by tobacco smoking, is increasingly reported in nonsmoking patients exposed to domestic combustion of biomass fuels. This is particularly common among rural women in the low- and middle-income countries. It is highly likely that the disease in these patients is a different clinical phenotype. AREAS COVERED: This manuscript covers epidemiological and clinical aspects of biomass-exposed COPD and also dwells upon its different phenotypic characteristics. EXPERT OPINION: Chronic obstructive pulmonary disease in biomass-exposed, nonsmoker patients which predominantly presents with features of chronic bronchitis is more likely a distinct phenotype with greater likelihood of small airway involvement.

Bronchiectasis in India: results from the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) and Respiratory Research Network of India Registry.

BACKGROUND: Bronchiectasis is a common but neglected chronic lung disease. Most epidemiological data are limited to cohorts from Europe and the USA, with few data from low-income and middle-income countries. We therefore aimed to describe the characteristics, severity of disease, microbiology, and treatment of patients with bronchiectasis in India. METHODS: The Indian bronchiectasis registry is a multicentre, prospective, observational cohort study. Adult patients (≥18 years) with CT-confirmed bronchiectasis were enrolled from 31 centres across India. Patients with bronchiectasis due to cystic fibrosis or traction bronchiectasis associated with another respiratory disorder were excluded. Data were collected at baseline (recruitment) with follow-up visits taking place once per year. Comprehensive clinical data were collected through the European Multicentre Bronchiectasis Audit and Research Collaboration registry platform. Underlying aetiology of bronchiectasis, as well as treatment and risk factors for bronchiectasis were analysed in the Indian bronchiectasis registry. Comparisons of demographics were made with published European and US registries, and quality of care was benchmarked against the 2017 European Respiratory Society guidelines. FINDINGS: From June 1, 2015, to Sept 1, 2017, 2195 patients were enrolled. Marked differences were observed between India, Europe, and the USA. Patients in India were younger (median age 56 years [IQR 41-66] vs the European and US registries; p<0·0001]) and more likely to be men (1249 [56·9%] of 2195). Previous tuberculosis (780 [35·5%] of 2195) was the most frequent underlying cause of bronchiectasis and Pseudomonas aeruginosa was the most common organism in sputum culture (301 [13·7%]) in India. Risk factors for exacerbations included being of the male sex (adjusted incidence rate ratio 1·17, 95% CI 1·03-1·32; p=0·015), P aeruginosa infection (1·29, 1·10-1·50; p=0·001), a history of pulmonary tuberculosis (1·20, 1·07-1·34; p=0·002), modified Medical Research Council Dyspnoea score (1·32, 1·25-1·39; p<0·0001), daily sputum production (1·16, 1·03-1·30; p=0·013), and radiological severity of disease (1·03, 1·01-1·04; p<0·0001). Low adherence to guideline-recommended care was observed; only 388 patients were tested for allergic bronchopulmonary aspergillosis and 82 patients had been tested for immunoglobulins. INTERPRETATION: Patients with bronchiectasis in India have more severe disease and have distinct characteristics from those reported in other countries. This study provides a benchmark to improve quality of care for patients with bronchiectasis in India. FUNDING: EU/European Federation of Pharmaceutical Industries and Associations Innovative Medicines Initiative inhaled Antibiotics in Bronchiectasis and Cystic Fibrosis Consortium, European Respiratory Society, and the British Lung Foundation.

Bronchoscopic lung cryobiopsy: An Indian association for bronchology position statement.

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.

Upper Respiratory Tract Tuberculosis.

Upper respiratory tract involvement in cases of tuberculosis (TB) of the head and neck continues to be described in the most recent reports from several different regions, including some from developed countries. Laryngeal TB is the most common of all forms of upper respiratory tract TB (URT-TB). Pulmonary lesions in URT-TB are present in about 20% of adults and about 50 to 60% of children. Systemic manifestations are uncommon. URT-TB is especially seen in patients with a variety of risk factors, such as the presence of human immunodeficiency virus (HIV) infection, diabetes, smoking, alcoholism, drug abuse, malignancies, and use of immunosuppressive drugs. Nodules or ulcerative lesions are seen on morphological examination. Endoscopic examination is required for mucosal lesions. Diagnosis of TB is suspected on an epidemiological basis in high-prevalence countries or from the failure of a patient to respond to routine treatment. Smear and/or histopathological examinations help in establishing the final etiological diagnosis. Treatment includes standard anti-TB chemotherapy for at least 6 months with four primary drugs during the initial intensive phase of 2 months and two or three primary drugs during the remaining maintenance phase of 4 months. Treatment is modified on the basis of culture and sensitivity reports in cases of suspected drug resistance. Surgical intervention may be required for some patients with abscess formation and progressive disease unresponsive to medical therapy. Airway obstruction, although rare, even in fulminant cases may require tracheostomy for relief.

Inflammatory myofibroblastic tumor of the trachea in the pediatric age group: case report and systematic review of the literature.

Inflammatory myofibroblastic tumors are uncommon tumors, which present as solitary masses in the pulmonary parenchyma. Tracheal involvement by these tumors is extremely rare and can be misdiagnosed as asthma. The closest histologic differential diagnoses are IgG4-related sclerosing pseudotumors, which are differentiated by IgG4 positivity. Fifty percent of inflammatory myofibroblastic tumors are positive for anaplastic lymphoma kinase gene rearrangements. The treatment modality of choice is surgical resection with therapeutic bronchoscopy reserved for patients presenting with acute airway obstruction or in those unwilling for surgery. New and upcoming treatments include anaplastic lymphoma kinase inhibitors like crizotinib.

Erlotinib usage after prior treatment with gefitinib in advanced non-small cell lung cancer: A clinical perspective and review of published literature.

Erlotinib and gefitinib are among the most widely researched, used and available molecularly targeted therapies for treatment of advanced non-small cell lung cancer (NSCLC). They are both tyrosine kinase inhibitors (TKIs) of the epidermal growth factor receptor (EGFR). In the past decade, there have been reports on clinical benefit from use of erlotinib after gefitinib failure in NSCLC patients. A review of published literature on this focussed topic is provided herein. Pooled analysis of published literature shows that majority of patients were female (60.6%), non-smokers (64.5%), had adenocarcinoma histology (88.3%) and were of East Asian ethnicity (92.3%). Presence of sensitizing EGFR mutation was detected in 48.4% of subjects. Disease control rates with prior gefitinib therapy and with subsequent erlotinib treatment were 79.4% and 45.4% respectively. Based upon our review, the most important predictive factor for clinical benefit from erlotinib identified was previous response to gefitinib. The exact explanations for the potential benefit from erlotinib use in this patient population is still not known and further studies are required to determine the role of molecular mechanisms especially those related to resistance to initial EGFR TKI therapy.

Novel treatment of a persistent bronchopleural fistula using a customized spigot.

An abnormal communication between the bronchus and the pleural cavity is known as a bronchopleural fistula (BPF). The incidence of postoperative BPF ranges from 1.5% to 28%, and is responsible for considerable morbidity and mortality. Various modalities have been used for treatment of BPF including surgery and endobronchial occlusion devices. The scope of therapy is limited in developing countries because of cost constraints and lack of availability of different occlusion devices. A novel method of BPF closure using a customized spigot and cyanoacrylate glue is described.

Incidental pathologically proven pulmonary hamartoma in a patient with carcinoma tongue.

Pulmonary hamartomas are usually clinically silent and found incidentally on chest radiographs. They can lead to diagnostic confusion especially in patients who have been previously treated for primary cancers at other sites. This can lead to consideration of metastatic malignancy as the primary diagnostic possibility. In this case, evaluation of a solitary pulmonary nodule (SPN) in a patient with carcinoma of tongue led to the diagnosis of pulmonary chondroid hamartoma. This highlights the fact that a pulmonary nodule in a patient with progressive cancer at another site does not always indicate pulmonary metastasis.