Background: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon, potentially fatal, hyperinflammatory syndrome that may rarely complicate the clinical course of disseminated Mycobacterium tuberculosis (MTB). The clinical course of tuberculosis-associated HLH (TB-HLH) has been reported to be unpredictable. Materials and Methods: Here we describe the clinicopathological features, laboratory parameters, management, and outcome data of a patient who satisfied the 2004 diagnostic criteria for HLH secondary to disseminated MTB; we also do a systematic review of the international literature on TB-HLH. The literature review (January 1975–March 2014) found that HLH complicated the clinical course of 63 tuberculosis patients (41 males, 22 females, mean age = 45 ± 23.5 years) with a high mortality rate of 49% (31/63 died). The mean serum ferritin level (n = 44/63) was 5963 ng/mL (range 500–38,539 ng/mL); and a higher proportion (54.2%) of patients had pancytopenia at presentation. On univariate analysis (n = 53/63), age >30 years [hazard ratio (HR): 2.79, 95% confidence interval (CI):1.03–7.56, P = 0.03], presence of comorbidities (HR 4.59, CI: 1.08–19.52, P = 0.04), marked hemophagocytosis in bone marrow (HR: 2.65, CI: 1.16–6.05, P = 0.02), and nonusage/delayed usage of antitubercular therapy (ATT) (HR: 3.44, CI: 1.51–7.87, P = 0.003) were associated with decreased survival, though none of these parameters attained statistical significance (P > 0.05) in multivariate analysis. Usage of corticosteroids and/or immunomodulator drugs (HR 1.00, CI: 0.66–3.22, P = 0.35) did not alter the outcome in these patients. Conclusion: HLH should be considered as a differential diagnosis in patients with tuberculosis who present with cytopenias, organomegaly, and coagulopathy. Strong clinical suspicion and early usage of ATT might be useful in reducing the morbidity and mortality. The utility of immunosuppressive/immunomodulator therapy lacks general concensus among treating physicians, and warrants further studies.

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Introduction: The majority of Indian hospitals do not provide intensive care unit (ICU) care or ward-based noninvasive positive pressure ventilation (NIV). Because no mechanical ventilation or NIV is available in these hospitals, the majority of patients suffering from respiratory failure die. Objective: To perform a cost-effective analysis of two strategies (ward-based NIV with concurrent standard treatment vs standard treatment alone) in chronic obstructive pulmonary disease (COPD) respiratory failure patients treated in Indian hospitals without ICU care. Materials and Methods: A decision-analytical model was created to compare the cost-effectiveness for the two strategies. Estimates from the literature were used for parameters in the model. Future costs were discounted at 3%. All costs were reported in USD (2012). One-way, two-way, and probabilistic sensitivity analysis were performed. The time horizon was lifetime and perspective was societal. Results: The NIV strategy resulted in 17.7% more survival and was slightly more costly (increased cost of $101 (USD 2012) but resulted in increased quality-adjusted life-years (QALYs) (1.67 QALY). The cost-effectiveness (2012 USD)/QALY in the standard and NIV groups was $78/QALY ($535.02/6.82) and $75/QALY ($636.33/8.49), respectively. Incremental cost-effectiveness ratio (ICER) was only $61 USD/QALY. This was substantially lower than the gross domestic product (GDP) per capita for India (1489 USD), suggesting the NIV strategy was very cost effective. Using a 5% discount rate resulted in only minimally different results. Probabilistic analysis suggests that NIV strategy was preferred 100% of the time when willingness to pay was >$250 2012 USD. Conclusion: Ward-based NIV treatment is cost-effective in India, and may increase survival of patients with COPD respiratory failure when ICU is not available.

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Background: Epidermal growth factor receptor (EGFR) mutation analysis has become an important part of the initial workup of non-squamous non-small cell lung cancer (NS-NSCLC) patients as it is now recognized both as a prognostic and predictive marker to therapy with EGFR tyrosine kinase inhibitors (TKI). Aim: In this retrospective study conducted at a University hospital, we evaluated the prevalence of EGFR mutations in patients with NS-NSCLC, clinico-pathological correlation and outcome to treatment with EGFR TKIs. Materials and Methods: Case records of 147 patients of NS-NSCLC in whom EGFR mutation status was tested were screened. EGFR mutation analysis was done using DNA sequencing by real time polymerase chain reaction method from tissue and cell blocks prepared from core biopsy, fine needle aspiration cytology and pleural fluid specimens. Results: EGFR mutations were seen in 30.6% of the 111 evaluable specimens, with a significantly higher rate in females (44% vs 19.6% P = 0.0072) as compared to men and non-smokers (41% vs 12% P = 0.0013) as against smokers. Most common mutations were observed in exons 19 (71%) and 21 (25%). The estimated median progression free survival for patients with and without mutations when treated with upfront TKIs was 12 months and 3 months respectively and the estimated median overall survival for patients with and without mutations was 20 and 9 months respectively. Conclusion: This study from India, further establishes the importance of upfront EGFR mutation testing in all NS-NSCLC patients, not only to prognosticate, but also to identify that subset of patients who could benefit from EGFR TKI therapy, early in the course of their disease.

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Background: The severity of exacerbation in chronic obstructive pulmonary disease (COPD) due to the overlap of obstructive sleep apnea syndrome (OSAS) is not known. Aims: To find out the 1) severity of acute exacerbation of COPD (AECOPD) in patients with overlap syndrome compared to only COPD, 2) prevalence of overlap syndrome in AECOPD, and 3) clinical characteristics of COPD compared to overlap syndrome. Materials and Methods: Fifty-one patients admitted with AECOPD were classified into; Mild exacerbation: Normal arterial blood gases (ABG) treated with antibiotics, Moderate: Normal ABG treated with parenteral corticosteroids, Severe: Type 1 respiratory failure, Very severe: Type 2 respiratory failure with normal pH and Life-threatening: Type 2 respiratory failure with pH <7.35. They were evaluated for OSAS with full polysomnography after the exacerbation subsided and analysed depending on presence or absence of overlap syndrome. Results: The majority of only COPD cases (26/38) had mild and moderate exacerbations whereas majority of overlap patients (9/13) had severe, very severe and life-threatening exacerbations (statistically significant, P = 0.021). Of 51 patients, 13 had OSAS i.e. the prevalence of overlap in AECOPD was 25.5%. The mean BMI in only COPD and overlap syndrome was 20.70 ± 8.03 kg/m² and 31.82 ± 5.80 kg/m² (P < 0.001), respectively. Metabolic syndrome was recorded in 2/36 (5.3%) patients in only COPD and 6/13 (46.2%) patients in overlap (P < 0.001). Conclusion: Overlap syndromes are more likely have respiratory failure compared to only COPD during AECOPD. AECOPD have a high prevalence of OSAS. Overlap syndrome have significantly higher likelihood of obesity and metabolic syndrome compared to only COPD.

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Pulmonary Cement Embolization (PCE) is a rare complication of vertebroplasty surgery. There is no clear guideline for management of this entity. There is no definite protocol for anticoagulation in PCE. This is a case report of our patient who was diagnosed to have Pulmonary Cement Embolization, which was quite significant involving both lungs. She was successfully managed without long term anticoagulation.

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Rituximab is a chimeric monoclonal antibody that targets CD-20 antigen expressed in more than 90% of all B cell non-Hodgkin's lymphoma (NHL). We report a case of 33-year-old female without any comorbidities, newly diagnosed with stage IIIB follicular lymphoma treated with rituximab-based chemotherapy. Patient developed exertional dyspnea and dry cough after the fourth cycle of rituximab-based chemotherapy. Diagnostic high-resolution computed tomography (HRCT) of the lungs revealed bilateral patchy ground glass opacities suggestive of interstitial lung disease (ILD). It was managed successfully with supplemental oxygen and corticosteroids with discontinuation of the Rituximab. Extensive review of the literature did not reveal ample of material on rituximab-induced ILD (RTX-ILD).

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A 35-year-old lady was seen in the outpatient clinic owing to fever, cough with mucopurulent expectoration, and breathlessness for the duration of 1 month. She had history of similar episodes treated with antibiotics four times during last 2 years. There was no history of recurrent sinusitis, diarrhea, and skin or soft tissue infection. She had no history of diabetes mellitus or steroid intake. She denied any history of facial trauma or dental infection in the past. There was no history of tuberculosis in her or in the family. Radiograph and CT scan of the chest revealed right upper lobe consolidation. Flexible fibreoptic bronchoscopy revealed multiple nodules at opening of right upper lobe bronchus. This clinicopathological conference describes the details of differential diagnoses, difficulties in achieving the final diagnosis and management of such patient.

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Background and Aim: Studying the recurrence of smear-positive pulmonary tuberculosis (TB) is a convenient way to evaluate the effectiveness of TB control programs and identify vulnerable patients. In the present study, the rate of recurrence of TB and its predictors were determined in Iran. Materials and Methods: This study was a retrospective cohort. Eligible people were patients with smear-positive TB who were diagnosed from 2002 to 2011. The end of the follow-up time was December 2013. The number of people who entered the cohort was 1,271 subjects. In order to determine the predictors of recurrence, multivariate logistic regression was used. Analysis was done using SPSS 20. Results: The recurrence incidence was 8.3% and in 85.9% of these patients, it occurred in the time interval of 1-5 years after successful treatment. The recurrence rate was not significantly related to gender, age group, and diabetes. But it was significantly higher in patients whose sputum smear grading before treatment was 2 + or more, patients with positive sputum smear at the end of the second month of the treatment, patients who had completed treatment, and patients who were smokers (P < 0.05). Conclusions: Our study showed that a considerable percentage of smear-positive pulmonary TB patients experience recurrence and that some patients are at a higher risk of recurrence.

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Endomterisois is usually found in women of child-bearing age. A case is presented of massive right-sided pleural effusion caused by endometriosis. The final diagnosis was made by thoracoscopic pleural biopsy. Physicians should be aware of this potentially treatable cause of pleural effusion having excluded other possibilities such as malignancy and tuberculosis.

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Lungs are target organs for toxic effects of various drugs due to many reasons. Diphenylhydantoin (DPH) is reported to have many extrapulmonary side effects. We are presenting a case of acute hypersensitivity pneumonitis (HP) secondary to DPH, presenting with respiratory failure. Acute HP with respiratory failure is an uncommon drug side effect of the DPH therapy and is a diagnosis of exclusion. It requires detailed workup and exclusion of other causes along with evidence of improvement in the patient's condition after withholding DPH.

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Background: Chronic obstructive pulmonary disease (COPD) was ranked the sixth-most common cause of death worldwide in 1990, but now it is the third-most common cause. The goal of the present study was to assess the prevalence and determine the causes and risk factors of COPD in Tehran. Materials and Methods: This study followed a stratified cluster sampling strategy with proportional allocation within strata. The target population was all non-institutionalized inhabitants, aged 18 to 40 in one group and over 40 in another who resided in Tehran in 2013. The core questionnaire was developed from pre-existing validated questionnaires that had already been used in multi-national studies. The single most important outcome measure obtained as part of this protocol was spirometry before and after the administration of 200 mg (two puffs) of salbutamol. Results: The most commonly reported respiratory symptoms were: sputum production in 291 patients (16.2%) [95% confidence interval (CI): 14.5-17.9%], chronic cough in 171 (9.5%) (95% CI: 8.2-10.9%), wheezing in 377 (21.0%) (95%CI: 19.1-22.9%) and dyspnea in 388 patients (21.6%) (95% CI: 19.7-23.5%). The overall COPD prevalence defined by the post-bronchodilator spirometric functional criteria was 9.2%. This value in men (10.1%) was higher than in women (8.5%); the prevalence was significantly higher in subjects aged over 55 years (P ≤ 0.002). The prevalence of COPD was strongly dependent on smoking status, especially in ex-smokers, and increased considerably with age. 69% of patients with COPD were non-smoker. Conclusion: The high prevalence of verified COPD, a great deal of which was undiagnosed before by a physician, highlights the necessity of raising awareness of this disease among health professionals, and use of spirometry in the primary care setting. A future cross-sectional and prospective cohort study should be performed to explore all risk factors and their impact on decline in lung function and worsening of respiratory symptoms especially in non-smokers.

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Pseudochylothorax, also known as chyliform effusion rich in cholesterol crystals, is a rare entity that sometimes occurs in long-standing rheumatoid arthritis (RA) and is usually associated with thickened pleura. There have only been a few case reports in the literature on pseudochylothorax unassociated with pleural thickening and with a short duration of articular symptoms in patients with RA. We report the case of a 70-year-old male patient with a history of RA and heart failure due to severe aortic stenosis, who presented with signs and symptoms of decompensated heart failure due to a moderate right-sided pleural effusion that was consequently proved to be pseudochylothorax unassociated with pleural thickening on chest computed tomography (CT) scan. The patient's outcome was favorable after thoracocentesis was carried out and leflunomide was added to the standard heart failure treatment.

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Trans-mediastinal herniation of lung is seen occasionally but herniation of bulla across the mediastinum is rare. We report two cases with trans-mediastinal herniation of bullae leading to an unusual line appearing near the mediastinum. We propose the name 'Semilunar sign'for this radiological sign because of its resemblance to the half-moon shape.

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Objectives: To study unusual lesions in the mediastinum, which do not originate from the thymus, lymph nodes, neural tissues or germ cells, and tissues that normally engender pathologic lesions in the mediastinum. Materials and Methods: Of the 65 cases seen, 12 unusual lesion were encountered in a 5^½ year period from 2006 to 2011. Results: Two cases of nodular colloid goiter and one each of the mediastinal cyst, undifferentiated carcinoma, and Langerhans cell histiocytosis (LCH) affected the anterosuperior mediastinum. In the middle mediastinum, one case each of the mesothelioma, malignant gastrointestinal stromal tumor (GIST), squamous cell carcinoma (SCC), solitary fibrous tumor (SFT), and pleomorphic sarcoma (PS) was seen. One case of meningeal melanocytoma (Mme) and primary pleural liposarcoma (PL) involved the posterior mediastinum. Persistent disease was seen in LCH after 2 years. Of all the cases with malignant lesions, only the patient with SCC was alive after 1 year. Conclusion: The cases of primary and SCC, LCH, melanocytoma, liposarcoma and PS, and GIST are unexpected and very rarely have paradigms in the mediastinum. Radiologic impression and knowledge of the compartment where these lesions arose from hardly assisted in arriving at a definitive opinion as the lesions were not typical of this location. A high index of suspicion and the immunohistochemical profile facilitated the final diagnosis.

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Context: For the follow-up of patients with idiopathic interstitial pneumonias (IIP), it is unclear which parameters of pulmonary function tests (PFT) and exercise testing would correlate best with high-resolution computed tomography (HRCT).Aim: To find out the correlation of symptom scores, PFTs and exercise testing with HRCT scoring in patients diagnosed as idiopathic interstitial pneumonia. Settings and Design: Cross-sectional study done in pulmonary medicine outpatients department of a tertiary care hospital in South India. Materials and Methods: Consecutive patients who were diagnosed as IIP by a standard algorithm were included into the study. Cough and dyspnea were graded for severity and duration. Pulmonary function tests and exercise testing parameters were noted. HRCT was scored based on an alveolar score, an interstitial score and a total score. The HRCT was correlated with each of the clinical and physiologic parameters. Pearson's/Spearman's correlation coefficient was used for the correlation of symptoms and parameters of ABG, PFT and 6MWT with the HRCT scores. Results: A total of 94 patients were included in the study. Cough and dyspnea severity (r = 0.336 and 0.299), FVC (r = −0.48), TLC (r = −0.439) and DLCO and distance saturation product (DSP) (r = −0.368) and lowest saturation (r = −0.324) had significant correlation with total HRCT score. Among these, DLCO, particularly DLCO corrected % of predicted, correlated best with HRCT score (r = −0.721).Conclusion: Symptoms, PFT and exercise testing had good correlation with HRCT. DLCO corrected % of predicted correlated best with HRCT.

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Bronchial artery embolization is the treatment of choice for the management of life-threatening massive hemoptysis. Chronic pulmonary thromboembolism (PTE) is one of the rare causes of hemoptysis. Management of hemoptysis in chronic PTE is a point of debate. In this article, we have reported one case of hemoptysis in chronic PTE managed successfully with bronchial artery embolization.

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Cystic lesions of the mediastinum may be congenital or acquired. The differential diagnosis depends on their location in the mediastinum. Cysts in the posterior mediastinum are generally developmental cysts and are neurogenic or of foregut origin. We report the case of a 14-year-old boy, who presented with dry cough and progressively increasing breathlessness, and was found to have a cystic lesion in the posterior mediastinum. Fine needle aspiration from the cyst helped make a diagnosis of tuberculosis.

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Closed pleural biopsy used to be a popular method of evaluation of pleural effusion. With the advent of thoracoscopy, this valuable method is being neglected. Studies have shown that closed pleural biopsy especially done with image guidance has high yield and low complication rate as compared to thoracoscopy. Given the ease of the procedure and the less cost involved, imaged guided closed pleural biopsy should be considered as the initial diagnostic step in undiagnosed pleural biopsy especially in developing countries with high prevalence of tuberculosis.

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This is a case report of a young 20-year-old male who was referred for an endobronchial ultrasound (EBUS) procedure to investigate a fever and a left hilar lymph node and patchy pleural-based opacities seen on CT of the chest. During the EBUS procedure besides the left hilar node, a large thrombus was noticed in the main pulmonary artery and another one in the right pulmonary artery. EBUS proved invaluable in making a diagnosis in an unsuspected case of pulmonary embolism.

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In the modern management of pleural diseases, thoracoscopy has a clear advantage over closed pleural biopsy. By way of its high yield, both in malignant pleural disease and pleural Tuberculosis – the two commonest cause of undiagnosed pleural effusion, thoracoscopy has the added advantage of faster symptom relief and offering effective pleurodesis. This makes it an attractive diagnostic and therapeutic procedure of choice and features high in the algorithms of many international guidelines on the approach to pleural diseases.

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Aims: To compare the metabolic activity of peripheral neutrophils in patients diagnosed with chronic obstructive pulmonary disease (COPD) with that of healthy, nonsmoking volunteers. Materials and Methods: Venous blood samples were taken from patients diagnosed with COPD as well as from healthy nonsmokers. Each sample was subjected to the nitro blue tetrazolium (NBT) test in which neutrophils exhibiting elevated metabolic activity were detected by light microscopy. The test was repeated after stimulation with Escherichia coli (E. coli) endotoxin with fresh samples. Neutrophils showing dye uptake were then counted in each case. Results: We found that the mean numbers of activated neutrophils without and with the addition of endotoxin were 19% and 23%, respectively, in the control group and 56% and 62%, respectively, in the test group. Two-sample t-test statistic revealed that there was a significant (P < 0.01) increase in neutrophilic metabolic activity in patients with COPD as compared to that in healthy volunteers. This significance remained even after stimulation using E. coli endotoxin. Conclusion: The results hint at a potentially relevant pathogenic mechanism in COPD related to the metabolic activity of neutrophils. By exhibiting enhanced metabolic activity, neutrophils in the COPD patients are more likely to be involved in damaging lung tissues.

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Endobronchial tumors infiltrating the carina is a formidable challenge to surgeons in view of difficult surgical access to the carina, especially on the left side, problems of securing the airway intra-operatively, technically challenging anastomosis due to anatomical location, and high post-operative morbidity and mortality. We present our surgical experience of two cases of left carinal pneumonectomy which was undertaken for resectable primary salivary gland type tumors of lung.

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A drop in the air-fluid level in the postpneumonectomy space on a chest radiogram is an early sign of bronchopleural fistula (BPF). Any suspicion of BPF points to the need for urgent evaluation and appropriate management. Very rarely may this drop occur without the existence of a fistula, but such a condition is defined as benign emptying of the postpneumonectomy space. We share our successful conservative management in a case of postpneumonectomy space emptying with a suspicion of BPF.

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