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Smoking-Related Interstitial Lung Disease

Currently, the worldwide number of tobacco smokers is estimated at 1.1 billion people, with the World Health Organization estimating that tobacco use is responsible for approximately 6 million premature deaths each year. In the United States 42 million adults are still smokers despite overall decreasing rates of smoking in the United States. Despite a majority of the morbidity and mortality of smoking being related to lung…

Pulmonary Langerhans Cell Histiocytosis

Etiology Langerhans cell histiocytosis (LCH) is an uncommon disease. Several synonyms, including histiocytosis X, eosinophilic granuloma, and Langerhans cell granulomatosis, have been used in the past, but the term Langerhans cell histiocytosis is now preferred. The previously used term histiocytosis X included several entities with similar morphologic but different clinical manifestations. These entities included Letterer-Siwe disease, which is often fatal and occurs in children and infants…

Hypersensitivity Pneumonitis

Etiology Hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis, is an immune-mediated inflammatory form of diffuse interstitial pulmonary disease caused by inhalation of various antigens that affect susceptible patients. Occasionally, an HP reaction pattern may be seen in association with drug toxicity. Bacterial, mycobacterial, fungal, animal protein, and chemical compound causative antigens have been identified, with common examples listed in Table 32.1 . TABLE 32.1…

Sarcoidosis

Sarcoidosis is a systemic inflammatory disorder of unknown etiology that affects multiple organs and is characterized by the formation of noncaseating granulomas. Intrathoracic lymph node and pulmonary parenchymal involvement occurs in more than 90% of cases. The granulomas have a characteristic distribution along the lymphatics in the pleura, interlobular septa, and bronchovascular bundles. They may resolve spontaneously or with treatment or progress to fibrosis. Etiology The…

Acute Interstitial Pneumonia

Acute interstitial pneumonia (AIP) is a severe acute disease of unknown etiology that usually occurs in a previously healthy individual and produces histologic findings of diffuse alveolar damage. The clinical, radiologic, and pathologic manifestations are identical to those of acute respiratory distress syndrome (ARDS); the only distinction is that no etiology is found. AIP is essentially idiopathic ARDS. Prevalence and Epidemiology AIP is rare, has no…

Cryptogenic Organizing Pneumonia/Secondary Organizing Pneumonia

Organizing pneumonia (OP) is a histologic pattern characterized by the presence of intraluminal granulation tissue polyps within alveolar ducts and surrounding alveoli associated with chronic inflammation of the surrounding lung parenchyma. Because the granulation tissue polyps frequently also involve the bronchioles, the pattern was previously known as bronchiolitis obliterans organizing pneumonia (BOOP). However, given the confusion of this entity with bronchiolitis obliterans (synonym: obliterative or constrictive…

Nonspecific Interstitial Pneumonia

Etiology, Prevalence, and Epidemiology Nonspecific interstitial pneumonia (NSIP) is a chronic interstitial lung disease characterized by homogeneous expansion of the alveolar walls by inflammation or fibrosis or both. NSIP is the second most common chronic interstitial pneumonia, after usual interstitial pneumonia (UIP), accounting for 14% to 35% of cases. NSIP may be idiopathic but more commonly occurs as a manifestation of connective tissue disease, hypersensitivity pneumonitis,…

Usual Interstitial Pneumonia/Idiopathic Pulmonary Fibrosis

Etiology, Prevalence, and Epidemiology Idiopathic pulmonary fibrosis (IPF) has been defined as “a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic and/or CT appearance of usual interstitial pneumonia (UIP).” IPF is the most common idiopathic interstitial lung disease. Data from the late 1980s estimated a prevalence of 20 per 100,000 for men and 13 per 100,000 for women,…

Leukemia

Etiology Leukemias are a group of malignancies in which abnormal cells, usually abnormal white blood cells (leukocytes), are produced in the bone marrow. Leukemias may be classified as myeloid, also known as myelogenous or myeloblastic, or lymphoid, also known as lymphocytic or lymphoblastic, depending on the types of abnormal cells that are produced. In myeloid leukemias, myeloid stem cells, which normally develop into erythrocytes, platelets, and…

Hodgkin Lymphoma

Etiology, Prevalence, and Epidemiology Hodgkin lymphoma (HL) is a neoplasm of B lymphocytes characterized by the presence of Reed-Sternberg cells. HL accounts for 10% of all cases of lymphoma and approximately 0.6% of all cancers diagnosed annually, with an annual incidence of 2 to 3 per 100,000 in Europe and the United States. Peak incidence occurs in two main age groups: young adults in the third…

Non-Hodgkin Lymphoma

Overview Non-Hodgkin lymphoma (NHL) is a heterogeneous group of diseases that accounts for 90% of lymphoma diagnoses, with the other 10% being Hodgkin lymphoma (HL). NHL arises during lymphocyte differentiation in either the humoral or cell-mediated immunity lineages of the immune system. Advances in molecular genetics and immunohistochemistry have increased the ability to differentiate distinct types of lymphoma, although not all of them are well understood.…

Pulmonary Lymphoid Hyperplasia and Lymphoid Interstitial Pneumonia (Lymphocytic Interstitial Pneumonia) *

Pulmonary Lymphoid Hyperplasia (Follicular Bronchiolitis) Etiology, Prevalence, and Epidemiology Pulmonary lymphoid hyperplasia, also known as follicular bronchiolitis or hyperplasia of bronchus-associated lymphoid tissue, is a benign condition characterized histologically by the presence of polyclonal lymphoid aggregates along the bifurcation of the bronchioles and along the pulmonary lymphatics and is mainly differentiated from lymphocytic interstitial pneumonia (LIP) by its typical distribution limited mainly to the airways. It…

Pulmonary Metastases

Etiology, Prevalence, and Epidemiology Pulmonary metastases are common—present at autopsy in 20% to 54% of patients with extrapulmonary malignancy. The most common primary sites associated with pulmonary metastases in biopsy series are the breast, colon, kidney, uterus, bladder, melanoma, and head and neck. Clinical Presentation Most pulmonary metastases occurring as single or multiple nodules are asymptomatic. When present, symptoms are nonspecific and include cough, hemoptysis, and…

Inflammatory Pseudotumor

Etiology, Prevalence, and Epidemiology Inflammatory pseudotumor, also known as inflammatory myofibroblastic tumor, is a quasineoplastic lesion that clinically and radiologically tends to mimic a malignant neoplasm and that histologically consists of a mixture of inflammatory cells, myofibroblastic spindle cells, and plasma cells. The proportion of the various cells varies considerably from pseudotumor to pseudotumor. Pseudotumors with a predominance of plasma cells are commonly referred to as…

Pulmonary Hamartoma

Etiology, Prevalence, and Epidemiology Pulmonary hamartomas are benign neoplasms, probably derived from bronchial wall mesenchymal cells. Hamartomas are the most common benign pulmonary neoplasm and account for about 8% of primary lung tumors. Although they may be seen in adolescents and young adults, most cases occur in patients older than 40 years; the peak incidence is in the seventh decade of life. Men are affected two…

Neuroendocrine Hyperplasia, Pulmonary Tumorlets, and Carcinoid Tumors

Neuroendocrine Hyperplasia and Pulmonary Tumorlets Etiology, Prevalence, and Epidemiology Normal lung tissue contains scattered neuroendocrine (Kulchitsky) cells within the bronchial and bronchiolar epithelium. These cells play a role in the detection of hypoxia as well as fetal lung development and may be involved in local epithelial cell growth and regeneration. Hyperplasia of these cells can be seen as a response to chronic airway inflammation, such as…

Pulmonary Carcinoma Staging

Schemes for Staging The most widely used scheme for staging non–small cell lung cancer (NSCLC) is the TNM classification. A variety of alterations in this scheme have been made to better group patients with similar prognosis and treatment options. The seventh edition of the TNM classification of lung cancer, published in 2009, was based on the analysis of a retrospective international database collected from 1990 to…

Lung Cancer: Radiologic Manifestations and Diagnosis

Etiology Lung cancer arises when cells lining the bronchi or peripheral airways undergo neoplastic change in response to an inciting agent or agents. The most common and well-recognized cause of lung cancer is cigarette smoking, which has been unequivocally linked with this disease through case-control studies since the 1950s. A dose-response relationship between cigarette consumption and the development of lung cancer has been well established. Although…

Screening for Lung Cancer

Background Lung cancer is the third most common cancer and the leading cause of cancer-related death in the United States. The most important risk factor for lung cancer is smoking, which results in approximately 85% of all US lung cancer deaths. It is estimated that 224,390 new cases and 158,080 deaths from lung cancer occurred in the United States in 2016. Deaths from lung cancer exceed…

Human Immunodeficiency Virus Infection

Etiology Since the recognition of human immunodeficiency virus (HIV) infection as the cause of acquired immune deficiency syndrome (AIDS) in the 1980s, many changes have occurred in the demographics, complications, and treatment of this disease. AIDS is the most advanced stage of HIV infection, where patients have a CD4 count of less than 200 cells/mm 3 or an AIDS-defining condition, such as recurrent bacterial pneumonia, Kaposi…