Etiology, Prevalence, and Epidemiology Goodpasture syndrome, also known as anti–basement membrane antibody disease, is an autoimmune disorder characterized by repeated episodes of pulmonary hemorrhage, usually associated with glomerulonephritis and the presence of anti–glomerular basement membrane (anti-GBM) antibodies. Goodpasture syndrome is…
Etiology, Prevalence, and Epidemiology Pulmonary vasculitis is traditionally characterized by size of vessel involvement as defined by the Chapel Hill nomenclature. Within the small-vessel vasculitides, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis represents a diverse group of entities, and although the inciting…
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…
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…
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…
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…
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…
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…
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…
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…