Role of rituximab as neoadjuvant therapy in a difficult case of relapsing pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is a rare disease which involves the accumulation of insoluble lipoproteinaceous material in the alveoli leading to impaired gas exchange and even respiratory failure. Autoimmune PAP is the most common type and is characterized by the presence of anti-granulocyte-monocyte colony stimulating factor (anti GM-CSF) antibody. Whole lung lavage has been traditionally used as first-line management of PAP but there is a lack of clarity especially in the treatment of relapsing cases of PAP. Rituximab is an anti Cluster of Differentiate 20 (CD 20) monoclonal antibody that has been tried as salvage therapy for relapsing cases of PAP. We present a case of 35 years old female patient who was diagnosed as a case of relapsing PAP who was managed initially with neoadjuvant rituximab. This is a retrospective observational report showing novel use of neoadjuvant rituximab in a difficult case of relapsing PAP.

Development of pneumatocele and spontaneous pneumothorax as a late complication of COVID-19 pneumonia.

SARS-COV2 pandemic has spread like wildfire and has affected all the countries worldwide. The virus mainly affects the lungs and has numerous manifestations. The development of spontaneous pneumatocele and pneumothorax has rarely been reported in the literature, especially in spontaneously breathing patients. We report two cases of COVID-19 patients who developed these complications after discharge from our hospital. These complications are uncommon but can be potentially fatal and the treating physician should keep these complications as differential while managing such cases.

A very rare case of lingular sequestration.

Pulmonary sequestration also called as bronchopulmonary sequestration is a rare congenital anomaly. It is defined as a mass of dysplastic lung tissue which has no connection with the main bronchopulmonary tree and is supplied by a branch of systemic artery and drainage by the separate venous system. It can be classified into intralobar and extralobar variety, with intralobar being more common. Its incidence is around 1 per 8300 to 35000, and it constitutes about 0.15-6.4% of all congenital lung anomalies. It generally involves lower lobes with the left lobe being more common than the right. It is an uncommon entity and rarely reported in literature for lingula. Its distribution is equal in gender distribution except for extralobar variety which has a male preponderance. It generally presents with recurrent pneumonia and hemoptysis. Here, we describe a very rare case of intralobar lingular sequestration in a patient who presented with recurrent chest infections and was managed with segmentectomy.

Real-world experience of monoclonal antibodies in mild-to-moderate COVID-19 patients at a tertiary care center.

Background: Neutralizing antibodies cocktail (casirivimab and imdevimab) has received emergency use authorization recommendation by Food and Drug Administration (FDA) and WHO for mild-to-moderate COVID-19 infection in specific high-risk groups. Antibodies cocktail has shown promising results in preventing progression to severe disease, but the real-world experience is still evolving. Herein, we present a retrospective analysis of 22 patients who were administered the antibodies cocktail between August 2021 and March 2022 at our tertiary care center. Methods: We conducted an observational retrospective analysis of clinicoradiological, inflammatory parameters, progression of the disease, and outcome among 22 mild and moderate COVID-19 patients treated with antibodies cocktail. Results: The mean age was 67.7 years (SD ± 18.3) and comprised of 13 males (59%), while 9 were females (40.9%). Nine (40.9%) patients were fully vaccinated with two doses, nine (40.9%) were partially vaccinated with one dose while four patients (18.2%) were unvaccinated, and the rest were unvaccinated. Diabetes and hypertension were the commonest comorbidities; hematological and solid organ malignancies were other comorbidities. Eight patients had radiological opacities consistent with COVID-19 pneumonia and had shown significant regression in four patients after the therapy. None of our patients required supplemental oxygen or progressed to severe acute respiratory distress syndrome. All patients were discharged in a stable condition within 6 days of the therapy. Conclusions: The neutralizing antibodies cocktail has shown encouraging results in our analysis in preventing progression to severe disease in patients with high-risk conditions.

Domiciliary noninvasive ventilation for chronic respiratory diseases.

Patients with chronic respiratory diseases, including chronic obstructive pulmonary disease (COPD), neuromuscular diseases, kyphoscoliosis and obstructive sleep apnoea-obesity hypoventilation syndrome (OSA-OHS), are at a higher risk of decompensation in the form of hypercapnic respiratory failure leading to intensive care unit (ICU) admission and increased mortality. This article reviews the evidence of role of domiciliary noninvasive ventilation (NIV) in patients with diseases with chronic ventilatory failure, including the mechanism of the effect of (NIV).

Pulmonary embolism in coronavirus disease 2019: the silent killer.

BACKGROUND: Pulmonary embolism (PE) has been identified as one of the deadliest complications of coronavirus disease 2019 (COVID-19), especially in patients admitted to the intensive care unit (ICU). Western literature reminds us of the high prevalence of PE in COVID. Here, we report a series of 13 cases of PE diagnosed and managed at our hospital. METHODS: Retrospective analysis of medical records of 13 cases of PE admitted at our hospital from February 1, 2020, to September 31, 2020, were done. Their clinical, laboratory, and radiologic data were assessed in detail. RESULTS: Computed tomography pulmonary arteriography was used to make the diagnosis in eight patients (61.53%), and clinical findings with corroborative ultrasound and laboratory parameters were used to label PE in five patients (38.46%). Five patients were hemodynamically unstable, requiring thrombolysis with recombinant tissue plasminogen activator, and four patients (30.76%) suffered a fatal outcome. CONCLUSION: COVID-19 is a highly prothrombotic state, and all physicians should keep a high vigilance for PE. All hospitalized patients with COVID-19, especially those admitted in ICU, should be on prophylactic anticoagulation and, if there is any worsening, should be started on therapeutic regimen. Patients at the time of discharge should be switched to oral anticoagulation, which should be continued for at least 3-6 months.

A case of thymoma presenting as pyrexia of unknown origin.

ABSTRACT: Pyrexia of unknown origin can be caused due to numerous infective and noninfective causes. It poses a diagnostic dilemma to the clinicians and requires a myriad of investigations for the confirmation of diagnosis. Thymomas are rare mediastinal tumors that present as anterior mediastinal mass; however, thymomas presenting as pyrexia of unknown origin has rarely been reported in the literature. We report an interesting case of a middle-aged male who presented as pyrexia of unknown origin due to thymoma.

Intrapleural Fibrinolysis with Urokinase versus Alteplase in Complicated Pleural Effusions and Empyema: A Prospective Randomized Controlled Trial.

BACKGROUND: Intrapleural fibrinolytic therapy (IPFT) has been used as an effective agent since 1949 for managing complicated pleural effusion and empyema. Several agents, such as streptokinase, urokinase (UK), and recombinant tissue plasminogen activator (rt-PA), have been found to be effective with variable effectiveness. However, a head-tohead controlled trial comparing the efficacy of the most frequently used agents, i.e., UK and rt-PA (alteplase) for managing complicated pleural effusion has rarely been reported. METHODS: A total of 50 patients were randomized in two intervention groups, i.e., UK and rt-PA. The dose of rt-PA was 10 mg, and that of UK was 1.0 lac units. UK was given thrice daily for 2 days, followed by clamping to allow the retainment of drugs in the pleural space for 2 hours. rt-PA was instilled into the pleural space twice daily for 2 days, and intercostal drainage was clamped for 1 hour. RESULTS: A total of 50 patients were enrolled into the study, of which 84% (n=42) were males and 16% (n=8) were females. Among them, 30 (60%) patients received UK, and 20 (40%) patients received alteplase as IPFT agents. The percentage of mean± standard deviation changes in pleural opacity was -33.0%±9.9% in the UK group and -41.0%±14.9% in the alteplase group, respectively (p=0.014). Pain was the most common adverse side effect, occurring in 60% (n=18) of the patients in the UK group and in 40% (n=8) of the patients in the alteplase group (p=0.24), while fever was the second most common side effect. Patients who reported early (within 6 weeks of onset of symptoms) showed a greater response than those who reported late for the intervention. CONCLUSION: IPFT is a safe and effective option for managing complicated pleural effusion or empyema, and newer agents, such as alteplase, have greater efficacy and a similar adverse effect profile when compared with conventional agents, such as UK.

Early experience of delamanid in extensively drug-resistant pulmonary tuberculosis.

Tuberculosis is a leading cause of death in our country. Multidrug-resistant tuberculosis increases the morbidity and mortality due to severe manifestations and difficult and prolonged medications. Newer antitubercular drugs like delamanid have been approved by WHO in management of these cases, but the real-world experience of this drug is lacking in our country. We present our early experience of use of delamanid in extensively drug-resistant pulmonary tuberculosis.

A cross sectional observational study on uncontrolled bronchial asthma and inhaler technique among out patients in a tertiary care centre in Western Maharashtra.

Background: The 2014 Asia Pacific - Asthma Insight and Management (AP-AIM) study showed that none of the Indian asthmatics had controlled asthma when evaluated objectively. Our study aims to detect through objective measurements, uncontrolled asthma in OPD patients, the adequacy of their inhaler technique, and their awareness regarding the importance of inhaler technique. Method: The asthma Control Test (ACT) score was used to screen for uncontrolled asthma. A validated objective questionnaire and scoring were used to objectively assess the correctness and awareness of the inhalation technique. Result: The study was carried out over 2 month period and 80 patients meeting the inclusion criteria were enrolled. Out of 80 patients; 47 (58.75%) had well-controlled asthma and 33 (41.25%) had uncontrolled asthma. 64 (80%) patients had good, and 16 (20%) had poor inhalation techniques. Among the 64 patients with good inhalation technique 43 (67.18%) had well-controlled asthma and 21 (32.82%) had uncontrolled asthma. Among the 16 patients with poor inhalation technique 4 (25%) had well-controlled asthma and 12 (75%) had uncontrolled asthma. There was a statistically significant association between asthma control and inhaler technique (P value 0.002). In the study population 56 (70%) patients were aware of the inhalation technique and 24 (30%) were unaware of the inhalation technique. Out of the 56 patients who were aware of the inhalation technique 53 (94.64%) had good inhaler technique and 3 (5.36%) had poor inhalation technique. Among the 24 patients who were unaware of the inhaler technique 11 (45.83%) had good inhaler technique and 13 (54.17%) had poor inhaler technique. There was a statistically significant association between awareness of inhaler technique and good inhaler technique (P value 0.001). Conclusion: There is a statistically significant association between asthma control and inhaler technique and between inhaler technique and inhaler awareness. Improving the awareness regarding inhaler technique among bronchial asthma patients is the way forward to improve asthma control in our country.

Anti-Jo1 Syndrome: Understanding a Rare Cause of Interstitial Lung Disease.

Background: Anti-Jo1 syndrome is one of the most common amongst the various anti synthetase syndromes (ASS), which forms a subgroup of the idiopathic inflammatory myositis (IIM). It is characterised by myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanic's hands; associated with the presence of anti-Jo1 antibodies in serum. Being an orphan disease, the clinical diagnosis is often delayed. Materials and methods: In this retrospective study, all patients diagnosed as Anti-Jo1 syndrome, from two tertiary care hospitals in Western Maharashtra, between 01 January 2019 - 31 December 2020, were enrolled. The parameters studied included demographic data, clinical features at presentation, laboratory parameters, spirometry, and radiographic findings, along with treatment instituted. Result: A total of 17 patients (8 males, 9 females) qualified for inclusion in the study. The mean age of diagnosis was 40 (±13) years with mean time to diagnosis being 2 years (± 0.6 years), from first clinical presentation. The most common presenting symptoms encountered were arthritis (n = 12, 70.5%), fever (n = 16, 70.5%), myositis (n=11, 64.7%) and breathlessness (n=10, 58.8%).10 patients had ILD at presentation on high resolution computerised tomography of chest (n=10, 58.8%) with restrictive lung defect on spirometry. Six patients required induction of immunosuppression using pulse methylprednisolone (n=6) and Rituximab (n=6), while 11 were managed with oral steroids. Mycophenolate mofetil (n=10) and Azathioprine (n=7) were used as maintenance immunosuppression. Conclusion: Anti-Jo1 syndrome is a myositis syndrome, presenting with a multitude of clinical features. Steroids and disease modifying anti rheumatic drugs form mainstay of therapy.

A very unusual case of interstitial lung disease.

Cotton dust exposure has been implicated in causing diseases like byssinosis and obstructive airway diseases like COPD and asthma. Long-term exposure to cotton dust causing interstitial lung disease and pulmonary fibrosis has been sparsely reported in the literature. Here, we report a case of an individual with long-term cotton dust exposure who presented with typical symptoms of interstitial lung disease and was managed conservatively.

Cement Factory Worker Presenting With Raynaud Phenomenon, Breathlessness, and Digital Ulcers.

CASE PRESENTATION: A 54-year-old man who had worked in a cement factory for the past 30 years, presented to the chest clinic with complaints of insidious onset, gradually progressive breathlessness with intermittent dry cough of three years' duration. The symptoms were associated with bluish discoloration of fingers on exposure to cold. He also gave a history of digital ulcers at the fingertips of the same duration. These ulcers used to heal, leaving behind pitted scars. There was also an associated history of progressive tightening of skin involving the face, extremities, and trunk. He also complained of food getting stuck in the throat, and he had to take frequent sips of water while eating, along with a feeling of early satiety. There was also a history of skin pruritus. There was no history of arthritis, rash, or alopecia. He had been treated 15 years ago for pulmonary TB, with 9 months of anti-tubercular therapy. He denied any similar illness in the family. On eliciting his occupational history, he revealed that other coworkers in his workspace had complained of a similar illness. He was a nonsmoker and teetotaller with no known addictions or exposure to chemicals.

Early experience of nintedanib in COVID-19 ARDS-related pulmonary fibrosis: a case series.

The current COVID-19 pandemic has spread like wildfire worldwide and has affected millions of people. The novel corona virus mainly affects the lungs leading to life threatening disease like acute respiratory distress syndrome (ARDS). The aftermath of the disease in form of pulmonary fibrosis is upcoming cause of further increase in morbidity and mortality. Nintedanib is an oral antifibrotics with proven role in idiopathic pulmonary fibrosis, however its use in COVID-19 related pulmonary fibrosis has not been studied. We report our early experience of use of nintedanib in COVID-19 related pulmonary fibrosis.

Diagnostic Yield and Safety of Bronchoscopic Lung Cryobiopsy in Evaluation of Lung Mass.

Background A mass lesion in the lung is a common finding seen on chest radiology. The prognosis of patients with mass lesions in the lung is capricious as malignancy is a consideration. It is essential to diagnose the underlying aetiology at the earliest with minimally invasive procedures for prompt treatment of the case. Bronchoscopic lung cryobiopsy (BLC) is a newer interventional technique in pulmonary medicine for the diagnosis of mass lesions in the lung. Materials and methods This is a retrospective study of patients reporting to a tertiary care centre who were radiologically (by computed tomography scan of the chest) diagnosed with a mass lesion of the lung and who underwent BLC during the period from January 2018 to January 2021. We analysed the diagnostic yield of the technique defined as a positive tissue diagnosis after the histopathological examination (HPE) along with the safety of the procedure. Results During the above period, we evaluated 70 patients who were diagnosed radiologically with mass lesions of the lung and underwent BLC. We obtained tissue diagnoses for 66 cases and the result of four cases was inconclusive. The diagnostic yield of the BLC procedure was 94.29%. There was no mortality and complications were minimal bleeding and small pneumothorax. Conclusion BLC is a newer technique for obtaining lung tissue via a flexible bronchoscope obviating the need for open lung biopsy. The main advantage of the technique is providing larger tissue samples with minimal or no side effects without undergoing multiple procedures as compared to other bronchoscopic or surgical methods for obtaining a diagnosis from lung tissue. BLC is a safer and promising technique in diagnosing mass lesions of the lung with better yield.

Diagnosis of Achondroplasia at Birth: A Case Report.

Autosomal dominant mutations in fibroblast growth factor receptor 3 cause achondroplasia, the most common form of dwarfism in humans. Achondroplasia is a genetic disorder causing rhizomelic shortening of limbs. Head is often large with prominent forehead causing vaginal delivery difficult. A twenty-one years old multipara mother gave birth to a baby with achondroplasia via spontaneous vaginal delivery with episiotomy without any complication. Achondroplasia, in this case, was diagnosed on the basis of antenatal ultrasonography finding, clinical features and radiological finding of the baby. He was admitted in the special baby care unit for observation and discharged on the next day as no complications were noted. Keywords: achondroplasia; dwarfism; ultrasonography.