Life-Threatening Hemoptysis.

Life-threatening hemoptysis (LTH) is any amount of hemoptysis that causes significant hemodynamic decompensation or respiratory distress which may lead to death if left untreated. While the amount of hemoptysis that qualifies as massive hemoptysis has continued to be debated, any amount between 100 to 1,000 mL/day is considered significant. Up to 15% cases of hemoptysis are LTH and need urgent life-saving intervention. Understanding of pulmonary vascular anatomy is of paramount importance to manage LTH. The goal of treatment lies in airway protection, appropriate oxygenation, and prevention of exsanguination. Once the airway is stabilized, a quick diagnosis and control of bleeding site is targeted. This chapter highlights current practices and approach to LTH including medical management, bronchoscopic approach, and advanced therapies such as bronchial artery embolization and surgical resection. We review situations, such as bronchiectasis, vascular malformation, diffuse alveolar hemorrhage, and tracheostomy bleed and specific approach to management of these conditions in a systematic and evidence-based manner.

Comparison of Clinical Measures Among Interstitial Lung Disease (ILD) Patients with Usual Interstitial Pneumonia (UIP) Patterns on High-Resolution Computed Tomography.

PURPOSE: Idiopathic Pulmonary Fibrosis is a progressive and fatal interstitial lung disease (ILD) characterized by a typical radiographic or histologic usual interstitial pneumonia (UIP) pattern. In 2018, diagnostic categories of UIP based on computed tomography patterns were revised by the Fleischner Society. The study aimed to describe differences in comorbidities and spirometry in ILD patients that were characterized by high-resolution computed tomography (HRCT) images as having a typical, probable, indeterminate, and alternative diagnosis of UIP. METHODS: We retrospectively studied 80 ILD patients from 2017 to 2019. Typical UIP was defined using the Fleischner Society diagnostic criteria for IPF. Atypical UIP was reached by consensus after a multidisciplinary clinical-radiological-pathological review of patient data. Baseline characteristics, comorbidities, and spirometry were compared among the four subgroups. RESULTS: Among 80 patients, 59% were male, 61% had a history of smoking, and the mean age was 67.7 ± 10 years (SD). A typical UIP pattern was more frequently observed among patients with chronic obstructive pulmonary disease (COPD) (p < 0.001) and pulmonary hypertension (p = 0.03). Of 30 patients with COPD, 14 had emphysema, while 10 had IPF. After adjusting for forced expiratory volume in one second (FEV1) in liters, change of FEV1% from baseline to 6-12 months, age, and sex, only COPD remained significantly associated with typical UIP (p = 0.018). Tobacco use was not significantly associated with any radiographic type (p = 0.199). CONCLUSION: Typical UIP was prevalent among COPD/emphysema patients. Although smoking has a strong association with IPF, we did not find a significant association with smoking and typical UIP in our cohort.

Spontaneous pneumothorax in a single lung transplant recipient-a blessing in disguise: A case report.

BACKGROUND: End-stage chronic obstructive pulmonary disease (COPD) is one of the common lung diseases referred for lung transplantation. According to the international society of heart and lung transplantation, 30% of all lung transplantations are carried out for COPD alone. When compared to bilateral lung transplant, single-lung transplant (SLT) has similar short-term and medium-term results for COPD. For patients with severe upper lobe predominant emphysema, lung volume reduction surgery is an excellent alternative which results in improvement in functional status and long-term mortality. In 2018, endobronchial valves were approved by the Food and Drug Administration for severe upper lobe predominant emphysema as they demonstrated improvement in lung function, exercise capacity, and quality of life. However, the role of endobronchial valves in native lung emphysema in SLT patients has not been studied. CASE SUMMARY: We describe an unusual case of severe emphysema who underwent a successful SLT 15 years ago and had gradual worsening of lung function suggestive of chronic lung allograft dysfunction. However, her lung function improved significantly after a spontaneous pneumothorax of the native lung resulting in auto-deflation of large bullae. CONCLUSION: This case highlights the clinical significance of native lung hyperinflation in single lung transplant recipient and how spontaneous decompression due to pneumothorax led to clinical improvement in our patient.

Advances in Targeted Therapy for Progressive Fibrosing Interstitial Lung Disease.

Progressive fibrosing interstitial lung disease (PF-ILD) has been redefined as a new clinical syndrome that shares similar genetics, pathophysiology, and natural history to idiopathic pulmonary fibrosis (IPF). IPF is the most common form of idiopathic interstitial pneumonias, which is progressive in nature and is associated with significant mortality. Therapies targeting an inflammatory and/or immune response have not been consistently effective or well tolerated in patients with IPF. The two antifibrotic drugs approved for IPF treatment, nintedanib and pirfenidone, have been shown to reduce lung function decline in PF-ILD. Novel uses of antifibrotic therapy are emerging due to a paucity of evidence-based treatments for multiple ILD subtypes. In this review, we describe the current body of knowledge on antifibrotic therapy and immunomodulators in PF-ILD, drawing from experience in IPF where appropriate.

A review of endovascular stenting for superior vena cava syndrome in fibrosing mediastinitis.

Fibrosing mediastinitis (FM) is a rare disorder of inflammation and fibrosis involving the mediastinum. The formation of fibroinflammatory mass in the mediastinum can lead to obstruction of mediastinal structures and cause severe debilitating and life-threatening symptoms. Superior vena cava syndrome (SVCS) is a dreaded complication of FM with no medical therapy proven to be efficacious. Spiral vein grafting has long been utilized as first-line therapy for SVC syndrome due to FM. Endovascular repair with stents and angioplasty for malignant causes of SVC syndrome is well established. However, there are limited data on their utility in SVC syndrome due to FM. We present two cases of SVC syndrome due to FM treated with endovascular stenting and a detailed review of current literature on its utility in SVCS due to benign causes.

A Blueprint for Success: Design and Implementation of an Ideal Bronchoscopy Suite.

Bronchoscopy is essential to the practice of pulmonary medicine. It is an important diagnostic and therapeutic tool for many disease processes. Bronchoscopy can be performed in a variety of clinical settings, from the bedside to an operating room. Although bronchoscopy has been practiced for more than a century, consensus recommendations from stakeholders have yet to be developed for the planning, implementation, and construction of a bronchoscopy suite. A wide range of procedures can be performed via bronchoscopy; therefore, the required tools and the procedure area must be aligned with the needs of the facility. Designing a bronchoscopy suite is by no means a "one size fits all" process. We present an overview of critical features to be considered in the planning for an ideal bronchoscopy suite. We use the term "ideal" because it represents a subjective conception of what is perfect and does not convey a rigid, universal blueprint.

Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease.

Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytic neoplasm. The rarity of the disease and heterogenous clinical presentations often leads to delayed diagnosis. Historically, ECD lacked effective treatment and the prognosis was poor. Following the recent discovery of frequent BRAF-V600E mutation in patients with ECD, vemurafenib, a selective BRAF V600 kinase inhibitor has been approved for BRAF-mutated ECD patients. The prognosis of ECD has dramatically improved with early recognition of the disease and available treatment. ECD affects nearly every organ system. Cardiac involvement with pericardial effusion is common but rarely with constrictive physiology or requiring pericardiectomy. We present a case of a 56-year-old woman with recurrent pericarditis with constrictive physiology along with pleural effusion and interstitial lung disease that was diagnosed with ECD 3 years after initial presentation. The patient's symptoms were relieved with pericardiectomy and targeted therapy.

Forbearance With Bronchoscopy: A Review of Gratuitous Indications.

Advanced technologies such as endobronchial ultrasound and electromagnetic navigation have revolutionized the field of bronchoscopy. Its indications as a diagnostic as well as a therapeutic tool continue to expand at a rapid pace. This growth also has led to the emergence of a new subspecialty of interventional pulmonology and more than 40 fellowship training programs. However, with increasing popularity and accessibility, there is a high impetus for performing the procedure when it may be of limited value. On the basis of a literature review and our own experience, we produced a list of conditions for which bronchoscopy is of limited value yet is being performed frequently. Conditions such as idiopathic pulmonary fibrosis, massive hemoptysis, cystic fibrosis, smear-negative pulmonary TB, and stage I sarcoidosis may be approached best in a more prudent fashion, with the bronchoscopic approach reserved for exceptional cases. We present an overview of conditions for which the expectations for bronchoscopy exceed the evidence in the literature, and we coined the term "forbearance with bronchoscopy" for situations in which this popular tool may not be the most appropriate initial approach.