NCCP-ICS joint consensus-based clinical practice guidelines on medical thoracoscopy.

ABSTRACT: Medical Thoracoscopy (MT) is commonly performed by respiratory physicians for diagnostic as well as therapeutic purposes. The aim of the study was to provide evidence-based information regarding all aspects of MT, both as a diagnostic tool and therapeutic aid for pulmonologists across India. The consensus-based guidelines were formulated based on a multistep process using a set of 31 questions. A systematic search of published randomized controlled clinical trials, open labelled studies, case reports and guidelines from electronic databases, like PubMed, EmBase and Cochrane, was performed. The modified grade system was used (1, 2, 3 or usual practice point) to classify the quality of available evidence. Then, a multitude of factors were taken into account, such as volume of evidence, applicability and practicality for implementation to the target population and then strength of recommendation was finalized. MT helps to improve diagnosis and patient management, with reduced risk of post procedure complications. Trainees should perform at least 20 medical thoracoscopy procedures. The diagnostic yield of both rigid and semirigid techniques is comparable. Sterile-graded talc is the ideal agent for chemical pleurodesis. The consensus statement will help pulmonologists to adopt best evidence-based practices during MT for diagnostic and therapeutic purposes.

Silicosis-Allergic Bronchopulmonary Aspergillosis Overlap-A Rare Duet!

Silicosis is a progressive pneumoconiosis caused by inhalation of crystalline silica dust commonly seen in workers of construction sites, flour mills, and mining. Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to Aspergillus fumigatus antigens commonly encountered in patients with asthma and cystic fibrosis. We report a case of 60-year-old flour mill worker presented with clinico-radiological features of silicosis; further evaluation was found to have an overlap of ABPA in view of severe atopic symptoms. We describe a rare duet of silicosis with ABPA overlap.

Prevalence of pre-extensively drug-resistant tuberculosis (Pre XDR-TB) and extensively drug-resistant tuberculosis (XDR-TB) among extra pulmonary (EP) multidrug resistant tuberculosis (MDR-TB) at a tertiary care center in Mumbai in pre Bedaquiline (BDQ) era.

Background: Drug-resistant tuberculosis (DR-TB) is the most exigent and calamitous challenge encountered in treatment of TB. Extra pulmonary (EP) DR-TB poses a complex diagnostic and therapeutic challenge owing to myriad of presentations and paucibacillary nature. Data available on this subset is limited. We studied the prevalence of EPDR-TB cases among the total DR-TB cases visiting our Programmatic management of Drug-Resistant TB (PMDT) site. We also studied the demographic and microbiological profile of these cases and analyzed the prevalence of pre-extensively drug-resistant TB (pre XDR-TB) and extensively drug-resistant TB (XDR-TB) among patients of EPDR-TB in pre Bdq era. Results: Of the 1086 DR-TB patients, 64 (5.89%) were cases of EPDR-TB. Seven out of 64 (10.93%) were primary EPDR-TB. The site wise distribution of cases was 34 (53.125%) lymph node DR-TB, 18 (28.125%) pleural DR-TB, 9 (14.0625%) spinal DR-TB/paraspinal abscess/psoas abscess, 1 case (1.5625%) each of abdominal DR-TB, sternal and gluteal abscess. On the basis of the second-line drug susceptibility testing (DST), patients were grouped into: (1) multidrug-resistant TB (MDR-TB), (2) MDR-TB with fluoroquinolone (FQ) resistance {pre XDR XDR-TB (FQ)}, (3) MDR-TB with second-line injectable (SLI) resistance {pre XDR XDR-TB (SLI)}, (4) XDR-TB. Of the 64 patients, 43 (67.185%) had MDR-TB, 19 (29.687%) had preXDR-TB (FQ), none had preXDR-TB (SLI) and 2 (3.125%) had XDR-TB. Gastro esophageal reflux disease (GERD) was the most common comorbidity seen in 26 (40.6%) patients, followed by anemia in 5 (7.8%), psychiatry problems 5 (7.8%), hypertension in 3 (4.6%), renal disorders in 2 (3.1%) while thyroid disorder, HIV and thalassemia in 1 each (1.5%). Conclusion: EPDR-TB forms a small but significant proportion of total DR-TB. Lymph node DR-TB is its most common subclass. Our study emphasises the momentousness and essentiality of baseline DST to FQ and SLI in patients of DR-TB. This enables an appropriate modification of therapy at baseline itself to better the treatment outcomes. We observed a strikingly high proportion of preXDR-TB (FQ) in our study group.

Genetic insight into Birt-Hogg-Dubé syndrome in Indian patients reveals novel mutations at FLCN.

BACKGROUND: Birt-Hogg-Dubé syndrome (BHDS) is a rare monogenic condition mostly associated with germline mutations at FLCN. It is characterized by either one or more manifestations of primary spontaneous pneumothorax (PSP), skin fibrofolliculomas and renal carcinoma (chromophobe). Here, we comprehensively studied the mutational background of 31 clinically diagnosed BHDS patients and their 74 asymptomatic related members from 15 Indian families. RESULTS: Targeted amplicon next-generation sequencing (NGS) and Sanger sequencing of FLCN in patients and asymptomatic members revealed a total of 76 variants. Among these variants, six different types of pathogenic FLCN mutations were detected in 26 patients and some asymptomatic family members. Two of the variants were novel mutations: an 11-nucleotide deletion (c.1150_1160delGTCCAGTCAGC) and a splice acceptor mutation (c.1301-1G > A). Two variants were Clinvar reported pathogenic mutations: a stop-gain (c.634C > T) and a 4-nucleotide duplication (c.1329_1332dupAGCC). Two known variants were: hotspot deletion (c.1285delC) and a splice donor mutation (c.1300 + 1G > A). FLCN mutations could not be detected in patients and asymptomatic members from 5 families. All these mutations greatly affected the protein stability and FLCN-FNIP2 interaction as observed by molecular docking method. Family-based association study inferred pathogenic FLCN mutations are significantly associated with BHDS. CONCLUSION: Six pathogenic FLCN mutations were detected in patients from 10 families out of 15 families in the cohort. Therefore, genetic screening is necessary to validate the clinical diagnosis. The pathogenic mutations at FLCN affects the protein-protein interaction, which plays key roles in various metabolic pathways. Since, pathogenic mutations could not be detected in exonic regions of FLCN in 5 families, whole genome sequencing is necessary to detect all mutations at FLCN and/or any undescribed gene/s that may also be implicated in BHDS.

Xeroderma Pigmentosum-Associated Childhood Interstitial Lung Disease.

Chromosomal breakage syndromes are a group of genetic disorders that are ascribable to the autosomal recessive mode of inheritance. Xeroderma pigmentosum is one of the chromosomal breakage syndromes which is due to the involvement of deformity in the deoxyribonucleic acid's nucleotide excision repair. Xeroderma pigmentosum is a genetic disorder, which is autosomal recessive, heterogeneous, and more common in cases of consanguinity, caused by mutations in at least 10 genes and 9 complementation groups. The disorder is very rare. Patients experience photophobia and extreme photosensitivity and have pigmentary changes in ultraviolet light-exposed areas of the body with freckling, premalignant, and malignant skin lesions arising in keratinocytes soon after the fleeting exposure to sunlight. Patients are also oversensitive to environmental mutagens such as cigarette smoke and possibly to the widely used agricultural insecticide, diazinon. Progressive neurological abnormalities along with some rare complications are also noticed among these patients. Symptoms and thoracic high-resolution computed tomography are considered for diagnosis. Only corticosteroids can be given to limit the progression of the disease. Xeroderma pigmentosum-related interstitial lung disease is one of the rarest forms and we thereby report an interesting case.

COVID Pneumonia in the Elderly - Not a Basket Case Always.

COVID 19 is a respiratory disease, caused by a novel coronavirus, which was christened as SARSCoV2. It has spread rapidly all over the globe, leading to a pandemic with high mortality, due to the lack of pre-existing immunity. The elderly population (old-old ; > 75 years) has been increasingly affected with more severe respiratory symptoms, as compared to the young old (60-75 years) and middle aged patients, probably due to the increasing presence of comorbidities. A greater proportion of the elderly have lymphopenia, raised inflammatory markers and increased D-dimer levels. We report two such cases with severe Adult Respiratory Distress Syndrome (ARDS), who improved with early diagnosis and management. Hence, prompt diagnosis and early intervention can avert a bad outcome.

Medical thoracoscopy without pleural fluid: How I do it.

Thoracoscopy is a commonly used minimally invasive procedure in the field of interventional pulmonology. While medical thoracoscopy is the widely preferred modality, modifications to the technique and expansion in the scope of its utility have always challenged the conventional approach. We describe a modified technique of medical thoracoscopy in absence of pleural effusion also known as dry thoracoscopy under sedation and local anaesthesia.

Clots in COVID: A Case Series.

As the novel coronavirus-2019 (COVID-19) pandemic spreads across the world, early recognition of the spectrum of symptoms and patterns of clinical presentation is crucial for optimal management. Emerging evidence shows that COVID-19 leads to a prothrombotic state and its association with pulmonary thromboembolism is well established. However, clinicians and the vascular community, in particular, should watch out for the nonpulmonary targets of this lethal virus as the failure to do so could give rise to disastrous consequences. The significance of raised D-dimer levels, whether it translates into thrombotic events for all patients and the need for universal oral anticoagulation postdischarge are issues that need urgent answers. We report a case series of five patients with thrombosis involving nonpulmonary sites. How to cite this article: Kaneria MV, Nadaf S, Desai U. Clots in COVID: A Case Series. Indian J Crit Care Med 2021;25(7):817-821.

Hydatid disease with Aspergilloma: A unique case report.

The coexisting presence of hydatid disease with aspergillus colonization is a rare finding. The 20-year-old presented with symptoms of hemoptysis with past history of tuberculosis. On further evaluation, the patient was diagnosed as a case of aspergilloma and managed conservatively. After one year of presenting with similar complaints, the patient was turned out to be hydatid disease with aspergillus colonization on the basis of clinic-radiological and bronchoscopic evaluation. Till now only a few case reports have been reported. We report a unique case report of a similar presentation.

Sarcoidosis at the Pulmonary Medicine Department of a tertiary care hospital in Mumbai. Our experience and The New Modified Criteria Clinical Radiological Physiological (TNMC CRP) score for sarcoidosis: A novel proposition to assess the functional status.

Sarcoidosis is a multisystemic granulomatous disease most commonly involving the pulmonary system and having a myriad of manifestations. However literature is scanty pertaining to the profile and scoring system in sarcoidosis. This study was undertaken to understand the profile of sarcoidosis and an endeavor to assess the functional status with a simplified scoring system. This was an observational study undertaken in the department of Pulmonary Medicine at a tertiary care. The profile of these patients was studied in terms of clinical features, radiological findings, the New Modified Criteria Clinical Radiological Physiological (TNMC CRP) score, six-minute walk distance (6MWD), spirometry, arterial blood gas parameters, serum angiotensin converting enzyme (ACE) levels and tissue biopsy histopathology. The 68 patients included 41 women and 27 men with a mean age of 42.7 years. They comprised of 18 (27%), 39 (57%), 4 (6%), 7 (10%) cases of stage 1, 2, 3, 4 sarcoidosis respectively. Most common presenting symptom and sign was progressive dyspnea 49 (72%), and peripheral lymphadenopathy 15 (22%). Serum ACE was elevated in 57 (83%). The average 6MWD was 360 meters. Most common high resolution computed tomography (HRCT) finding was mediastinal lymphadenopathy and peri-bronchovascular nodules. Spirometry was restrictive abnormality in 48 (96%) patients. Evidence of pulmonary hypertension (PH) was present in 32 (47%) patients. Tissue diagnosis revealed granulomatous inflammation in 51 biopsies with a transbronchial lung biopsy (TBLB) yield of 62%. The average TNMC CRP score was 5. There was a positive correlation between this score and 6MWD which was statistically significant. The score correlated with the functional status. Diagnosis of sarcoidosis warrants a comprehensive and multimodality approach. HRCT and tissue biopsy are the most important diagnostic armamentariums. Modified simplified scores help assess the functional status of the disease.

Birt-Hogg-Dubé syndrome - an unique case series.

Birt-Hogg-Dubé syndrome (BHDS) is an uncommon autosomal dominant syndrome. It is also known as Hornstein-Knickenberg syndrome. It is an inherited disorder culminating in mutations in folliculin coding gene (FLCN). The clinical exhibitions of the syn-drome are multi-systemic, comprising of a constellation of pulmonary, dermatologic and renal system manifestations. The most common presentations include fibrofolliculomas, renal cell carcinomas, lung cysts and spontaneous pneumothorax. The treatment is conservative with regular monitoring of the renal and lung parameters. Fibrofolliculomas may require surgical excision and recurrent events of pneumothorax may warrant pleurodesis. We reported a case series of 2 patients presenting with symptoms of progressive breathlessness along with dermatological manifestations and subsequently showing radiological manifestations of Birt-Hogg-Dubé syndrome in the form of lung cysts.

Prevalence and profile of sleep-disordered breathing and obstructive sleep apnea in patients with interstitial lung disease at the pulmonary medicine department of a tertiary care hospital in Mumbai.

BACKGROUND: Sleep-disordered breathing (SDB), predominantly obstructive sleep apnea (OSA), is a frequent phenomenon in interstitial lung disease (ILD) and may be associated with significant morbidity and mortality. METHODOLOGY: A prospective, observational, hospital-based study was conducted in a tertiary care hospital after ethics committee permission. The study group consisted of 100 consecutive ILD patients diagnosed by a multidisciplinary diagnosis. They were evaluated for the prevalence of SDB with a polysomnography after a comprehensive history, detailed clinical examination, calculation of various pretest probability scores, and relevant prerequisite workup. RESULTS: Out of the total 100 ILD patients, 44 were male (44%) and 56 were female (56%). SDB was present in 57 (57%) patients. Of these, 29 (29%) were found to have only nocturnal oxygen desaturation (NOD), while 28 (28%) had OSA. The 28 cases of OSA were distributed as 15 mild OSA (53.57%), 10 moderate OSA (35.71%), and 3 severe OSA (10.71%). The patients were divided into the following four groups: total study Group (A), patients with OSA (Group B), patients with NOD without OSA (Group C), and no SDB (Group D). The mean forced vital capacity values predicted in the four groups were 53.67%, 50%, 45.56%, and 57.87%, respectively. The mean body mass index in the four groups was 24.56, 27, 26.98, and 24.89 kg/m2, respectively. The mean 6-min walk distance in the four groups was 280.7, 250, 256.65, and 311.4 m, respectively. The mean partial pressure of oxygen in the four groups was 65.65, 60, 62.10, and 75.66 mmHg, respectively. The mean apnea-hypopnea index in the study group was 2.98/h, 8.6/h with mild OSA, 21.69/h with moderate OSA, 48.78/h with severe OSA, 3.89/h in patients having NOD without OSA, and 2.54/h in patients with no SDB. CONCLUSION: SDB in ILD is associated with a significant impact on the cardinal determinants of functional capacity, lung function, and quality of life.

"Esophagomediastinal fistula presenting as drug resistant tuberculosis".

Esophageal tuberculosis is one of the rarest forms of tuberculosis with higher incidence in immunocompromised cases. In majority of cases it is seen associated with esophagomediastinal and esophagotracheal fistulas. Diagnosis is established with the help of esophagoscopy followed by histopathology and microbiological analysis of biopsy sample. Treatment with antituberculous therapy alone is sufficient in majority of cases, however surgical management is mandatory in severe and non resolving cases. We thereby report an interesting case of esophagomediastinal fistula presenting as drug resistant tuberculosis with retroviral disease.

Management of interstitial lung diseases: A consensus statement of the Indian Chest Society (ICS) and National College of Chest Physicians (NCCP).

BACKGROUND: Interstitial lung disease (ILD) is a complex and heterogeneous group of acute and chronic lung diseases of several known and unknown causes. While clinical practice guidelines (CPG) for idiopathic pulmonary fibrosis (IPF) have been recently updated, CPG for ILD other than IPF are needed. METHODS: A working group of multidisciplinary clinicians familiar with clinical management of ILD (pulmonologists, radiologist, pathologist, and rheumatologist) and three epidemiologists selected by the leaderships of Indian Chest Society and National College of Chest Physicians, India, posed questions to address the clinically relevant situation. A systematic search was performed on PubMed, Embase, and Cochrane databases. A modified GRADE approach was used to grade the evidence. The working group discussed the evidence and reached a consensus of opinions for each question following face-to-face discussions. RESULTS: Statements have been made for each specific question and the grade of evidence has been provided after performing a systematic review of literature. For most of the questions addressed, the available evidence was insufficient and of low to very low quality. The consensus of the opinions of the working group has been presented as statements for the questions and not as an evidence-based CPG for the management of ILD. CONCLUSION: This document provides the guidelines made by consensus of opinions among experts following discussion of systematic review of evidence pertaining to the specific questions for management of ILD other than IPF. It is hoped that this document will help the clinician understand the accumulated evidence and help better management of idiopathic and nonidiopathic interstitial pneumonias.

Role of GeneXpert in the diagnosis of mycobacterium tuberculosis.

INTRODUCTION: GeneXpert (GX) is a novel, integrated, cartridge-based, nucleic acid amplification test with an established role for rapid diagnosis of Mycobacterium tuberculosis and detection of rifampicin resistance. AIM: To evaluate the role of GX in pulmonary and extrapulmonary tuberculosis (TB) cases. MATERIAL AND METHODS: A prospective study was conducted in the pulmonary medicine department of a tertiary care hospital after the Ethics Comittee permission. Data of 257 presumptive TB patients was retrieved for GX, acid fast bacilli smear and cul-ture (AFB smear and culture) and drug susceptibility test (DST). Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) of GX in diagnosis and determination of rifampicin resistance in pulmonary and extrapulmonary TB cases were calculated and compared with culture and DST results. RESULTS: Our study included 132 pulmonary and 125 extrapulmonary cases. On the basis of clinicoradiological and microbiological correlation, diagnosis of TB was confirmed in 104 pulmonary and 103 extrapulmonary cases. Out of a total of 104 pulmonary TB cases, 73 were rifampicin-sensitive and 31 were rifampicin-resistant cases. 103 extrapulmonary TB patients included 66 rifampicin-sensitive and 37 rifampicin-resistant cases. The sensitivity, specificity, PPV, NPV of GX in diagnosis and detection of rifampicin resistance in pulmonary TB was 95%, 93%, 98%, 84% and 96%, 100%, 100%, 96%, respectively. The sensitivity, specificity, PPV, NPV of GX in diagnosis and detection of rifampicin resistance in extrapulmonary TB cases was 79%, 86%, 96%, 47% and 97%, 95%, 97%, 95%, respectively. CONCLUSIONS: GX results are superior to smear microscopy and comparable to culture with shorter turnaround time.We recom-mend using it in routine TB diagnosis as this will expedite the management of patients with presumptive TB.

Tuberculosis in 2019.

Tuberculosis (TB) updates and guidelines have been published rapidly in last few years. The WHO and RNTCP have recommended suggestions that have changed the diagnostics and therapeutics paradigm in 2019. The rapid nature of these changes need to be appraised at the pulmonologist end. We conducted a google survey to study these gaps and subsequently review TB in 2019 focusing on the gaps in the survey. We narrate a short review covering the important diagnostic and therapeutic aspects in brief. We discuss the results of our google survey to address the knowledge gaps. Diagnosis, principles and rationale of therapy and treatment of drug sensitive and drug resistant tuberculosis including the shorter regimen and regrouping of drugs are important considerations of our review.

Percutaneous lung abscess drainage: revisiting the old gold standard.

Lung abscess is defined as the necrosis of lung tissue with cavity formation due to varied etiology. The treatment of lung abscesses is medical involving antibiotics and chest physiotherapy. The failure of medical line of management requires an invasive surgical or percutaneous approach for drainage and control of infection. While the literature is ample regarding the surgical approach, it is rather scarce on the percutaneous approach. The percutaneous drainage has been most studied with computed tomography guidance. With our case series we describe to the treatment of lung abscesses non-responsive to medical management, by a bedside minimally invasive ultrasound or fluoroscopy guided percutaneous drainage approach.

The Medical Concrete Cancer of the Lung: A Unique Case Report.

A 53-year-old 10 pack year smoker and concrete worker for 12 years presented to us with a lung mass proven to be small cell lung cancer on lung biopsy. An industrial terminology of concrete cancer already exists defining the wear and tear in concrete due to various interactions. The interactions of concrete and smoke on lung, predisposed and increased the risk of lung cancer probably as an additive effect which we label as the medical concrete cancer of the lung.