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Polymyositis/Dermatomyositis

Etiology Immune-mediated muscle inflammation and vascular damage are the hallmarks of polymyositis (PM) and dermatomyositis (DM). In PM the immune system is primed to act against muscle antigens, whereas in DM there is complement-mediated damage to both endomysial vessels and the microvasculature of the dermis. The trigger for the autoimmune malfunction in PM/DM is unknown. As with other connective tissue diseases, B-cell and T-cell autoregulation is…

Systemic Lupus Erythematosus

Etiology The pathogenesis of systemic lupus erythematosus (SLE) involves genetic and environmental factors, hormonal influences, and cell-mediated responses. In SLE, B lymphocytes lose self-tolerance and inappropriately produce autoantibodies. Serologic positivity for antinuclear antibodies (ANAs) is found in nearly all patients, but anti–deoxyribonucleic acid (DNA) antibodies, although less frequent than ANAs, are far more specific for SLE. A high titer of anti–double-stranded DNA (dsDNA) is considered the…

Systemic Sclerosis (Scleroderma)

Etiology Systemic sclerosis (scleroderma) has three cardinal features: excessive collagen production, vascular damage, and inflammation The pathogenesis of systemic sclerosis is obscure, but there are several contributing factors, including genetic, environmental, and autoimmune influences. Familial aggregation for systemic sclerosis, although infrequent (1.2%–1.5% of families), has been established and lays a firmer foundation for genetic studies of its etiopathogenesis. Systemic sclerosis is considered to be an autoimmune…

Rheumatoid Arthritis

Etiology Rheumatoid arthritis (RA) is an autoimmune disorder of unknown cause with variable clinical manifestations. Genetic factors play a major role in disease susceptibility and expression. The B lymphocyte is important in the pathogenesis of RA, and therapeutic B-cell depletion has been shown to be beneficial in active RA. The exact trigger for autoantibody formation is unknown, but in animal models, both B cells and non–B-cell…

Metabolic and Storage Lung Diseases

Metabolic and storage lung diseases are a broad group of diseases and syndromes characterized by underlying biochemical or metabolic dysfunctions. Accurate diagnosis is difficult because these diseases are often indolent, are rarely encountered in clinical practice, and because their manifestations are often vague and nonspecific. These groups of disorders may affect the lung solely or as part of a systemic disorder. Metabolic Pulmonary Diseases Pulmonary Alveolar…

Eosinophilic Lung Diseases

Eosinophilic diseases of the lung include a heterogeneous group of pulmonary disorders that characteristically feature peripheral or tissue eosinophilia. Patients with these illnesses have a variable clinical presentation and may be asymptomatic or may exhibit organ dysfunction or clinical symptoms of severe respiratory illness. Histologically, eosinophilic lung diseases demonstrate infiltration of eosinophils in the pulmonary interstitium and alveolar spaces with preservation of the lung architecture. As…

Idiopathic Pleuroparenchymal Fibroelastosis

Amitani and colleagues first described a unique pattern of upper lobe–predominant, idiopathic pulmonary fibrosis (IPF) in 13 patients in the Japanese literature in 1992. The currently preferred term in the English-language literature, idiopathic pleuroparenchymal fibroelastosis (iPPFE), was coined by Frankel and colleagues in 2004 in a case series of five patients presenting with unique clinical and radiographic findings distinct from any of the previously characterized idiopathic…

Lymphangioleiomyomatosis and Tuberous Sclerosis

Etiology Lymphangioleiomyomatosis (LAM) is a systemic disease of unknown etiology, affecting almost exclusively women. LAM is characterized by idiosyncratic smooth muscle cell proliferation (LAM cells), which leads to lung cysts, systemic lymphatic abnormalities, and abdominal tumors. The main manifestation is a progressive destructive process of the lungs, which may result in respiratory failure. LAM cells can be isolated from peripheral blood, indicating the ability to spread…

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…