Abstract
This chapter discusses six common entities of respiratory disease: obstructive and restrictive disorders of gas exchange, infectious and inflammatory diseases, immunologic disorders, vascular diseases of the lung, tumors of the lung and pleura, and miscellaneous other diseases of the respiratory tract.
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Keywords
- Chronic Obstructive Pulmonary Disease
- Pulmonary Hypertension
- Idiopathic Pulmonary Fibrosis
- Pulmonary Hypoplasia
- Pulmonary Alveolar Proteinosis
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Introduction
This chapter discusses six common entities of respiratory disease: obstructive and restrictive disorders of gas exchange, infectious and inflammatory diseases, immunologic disorders, vascular diseases of the lung, tumors of the lung and pleura, and miscellaneous other diseases of the respiratory tract.
Chronic Obstructive Lung Diseases
Chronic obstructive pulmonary disease (COPD) comprises a number of different respiratory diseases, with pathologic obstruction of pulmonary air flow as the common pathogenetic mechanism. Major examples of such diseases include chronic bronchitis, bronchiolitis and asthma, cystic fibrosis (CF), bronchiectasis, and α1-antitrypsin deficiency. COPD causes progressive and destructive emphysema and recurrent inflammation with destruction of lobular tissues and small vessels and finally may lead to cor pulmonale secondary to reduced intrapulmonary blood flow, pulmonary hypertension, and right heart insufficiency.
There are different types of emphysema, with a different extent of the lesion (centriacinar, panacinar, bullous) or different pathogenesis (acinar, paraseptal, interstitial). In radiology (and partly in pathology), the term lobular is used more commonly than acinar, although they are not completely congruent (acini being smaller units than lobes). The pathophysiology of COPD as determined by spirometric function tests is defined by a normal or increased total lung capacity (TLC) and forced vital capacity (FVC) in combination with decreased forced expiratory volume (FEV). COPD follows either increased resistance to air flow (e.g., luminal narrowing of air ducts) or the loss of elastic recoil (passive widening of air spaces). The figures present gross features of the most common examples of COPD.
Chronic Restrictive Lung Diseases
Restrictive pulmonary diseases (RPDs) include a number of disorders that have in common a progressive limitation of respiratory expansion of the lungs. Both acute and chronic forms are defined: Acute RPDs include adult respiratory distress syndrome (ARDS) and acute hypersensitivity pneumonitis, whereas chronic RPDs include idiopathic pulmonary fibrosis (IPF), sarcoidosis, collagen vascular diseases affecting the lung (see the section on Immunologic Diseases), and pneumoconioses. Chronic diseases progress to extensive pulmonary fibrosis with honeycombing, and (as with COPD) pulmonary hypertension and cor pulmonale may develop. Spirometric function tests in RPDs show significantly reduced respiratory compliance (reduced FVC with normal or reduced FEV).
Pulmonary Infectious Diseases
Pathologic features of pulmonary infections are quite variable, depending on the etiologic organism and its toxicity, replication, and persistence, as well as on the quality and intensity of host defense mechanisms. Bacterial pneumonias (bronchogenic or hematogenous) commonly are suppurative, abscessing, necrotizing, or hemorrhagic. Viral pneumonias (through immune T-cell stimulation) are preferentially lymphocytic and, in severe cases, are necrotizing and hemorrhagic (e.g., classic influenza). Fungal infections in the lung stimulate macrophages and frequently cause granulomas (also an immune T-cell reaction); depending on their toxicity, they may cause additional necroses and vascular invasion with thromboses. Parasitic infestations and mycobacterial infections also stimulate phagocytosis and may cause granulomas. Only acute pneumonias without significant tissue necrosis can show complete resolution and healing. Longer-lasting or chronic pneumonitides will resolve incompletely with residual scarring. Chronic, persistent infections consequently also present a risk of the development of chronic restrictive lung diseases.
Bacterial Pneumonias
Tuberculosis
Viral Pneumonitis
Fungal Pneumonia
Protozoal Infections
Immunologic Diseases of the Lung
Some lung diseases already mentioned—asthma (COPD) and acute hypersensitivity pneumonitis (an RPD)—have an immunologic pathogenesis. Similarly, for several forms of IPF (fibrosing alveolitis), autoimmune mechanisms are considered to be contributory in their pathogenesis or course. In addition, collagen vascular diseases such as systemic lupus erythematosus (SLE), primary systemic sclerosis (scleroderma), and rheumatoid arthritis frequently involve the lungs. Patients with rheumatoid arthritis may show features of silicosis (Caplan’s syndrome), and several forms of systemic vasculitides also affect the lungs, including Goodpasture’s syndrome, Wegener’s pneumogenic granulomatosis, and Churg–Strauss disease. Although gross features of these disorders are usually nonspecific, the figures show a few examples as a reminder for the differential diagnosis. All are thought to have an autoimmune pathogenesis, although the etiology remains obscure. Many of these diseases have genetic traits. (See also the figures on asthma and IPF under Sect. “Chronic Obstructive Pulmonary Diseases.”)
Vascular Diseases of the Lung
Vascular lung diseases include primary or secondary changes of the structure of blood vessels. Most common are plexiform vessel disease (the end stage of pulmonary hypertension) and primary pulmonary glomangiosis (Masshoff and Röher disease [MRD]). Another group of vascular diseases in the lung occurs secondary to clotting disorders, with occlusion of intrapulmonary blood vessels by thromboembolism or local thrombosis (e.g., with the use of hormonal contraceptives). Structural vascular alterations, including extensive thromboses of small vessels, are commonly accompanied by severe pulmonary hypertension and cause cor pulmonale. Thromboembolic and major thrombotic disorders may cause pulmonary hemorrhagic infarction (in cases of left ventricular heart failure) and ultimately also pulmonary hypertension. Acute massive thromboembolism of major pulmonary arteries will cause acute right heart failure and sudden death.
A related condition is “shock lung,” although it is caused by systemic circulatory failure rather than being a primary disease of the lung vasculature. Shock lung not infrequently determines the final outcome of the patient’s clinical course.
(See also the figures on Wegener’s granulomatosis under “Immunologic Diseases of the Lung.”)
Tumors of the Lungs and Pleura
Tumors of the lungs essentially comprise epithelial neoplasms (carcinomas), mesenchymal tumors (sarcomas), and metastases. All are classified according to the cell of origin (e.g., squamous cell carcinoma [SCC] of the bronchus, adenocarcinoma of the bronchial glands, or the alveolar pneumocytes). Sarcomas may include angiosarcomas, neurosarcomas, and lymphosarcomas. Other tumors include large cell or small cell carcinomas or tumors of specialized neuroendocrine cells in the lung, identified as carcinoids. Finally, the lungs are frequent targets for tumor metastases from cancers of the breast, pancreas, testes, bone, skin (e.g., malignant melanoma), and essentially from other sarcomas. Mesenchymal tumors of the pleura identified as mesothelioma can mimic epithelial adenoid structures.
Miscellaneous Lung Diseases
Additional images in this chapter depict some entities not covered in the earlier paragraphs: lipid pneumonia (LP), alveolar proteinosis, pulmonary fluid overload syndrome (PFOS), pulmonary hypoplasia, and sequestration of the lung. LP may be either exogenous or endogenous. Most reported cases of exogenous LP have resulted from inhalation of mineral oil or petrolatum or occupational exposure to oil mist (steel industry, airline engine maintenance). Endogenous LP is rare; it is independent of exogenous lipid exposure, and its etiology is often unclear. It has been described as accompanying diseases of airway obstruction (“resorptive pneumonitis”) as well as in certain infections or parasitic infestations. The latter may cause overproduction of surfactant by type II pneumocytes, with accumulation of related substances in lipid-filled macrophages.
Pulmonary alveolar proteinosis (PAP) is a rare disease with excessive accumulation of granular phospholipoproteinaceous materials in air spaces. Patients suffer from progressive dyspnea and are treated with repeated bronchopulmonary lavage. The etiology is unknown; primary (idiopathic) forms have been described, as well as secondary PAP occurring after inhalation of substances such as silicates, beryllium, aluminum, and insecticides. PAP also occurs more frequently in patients with hematologic malignancies or in immunosuppressed persons.
Pulmonary fluid overload syndrome is a serious complication of hyperinfusion and poor electrolyte balance. The lungs show a massive alveolar and interstitial edema, with fibroplasia if the disorder persists over a prolonged period. PFOS may be further complicated by bronchospasm and acute respiratory distress syndrome, in which antibodies against plasma proteins and neutrophils initiate anaphylactic reactions.
Pulmonary hypoplasia and pulmonary sequestration are developmental disorders. Pulmonary hypoplasia is frequently related to urogenital malformations of the fetus, such as urethral aplasia (prune belly syndrome). Pulmonary sequestration consists of abnormal development of a lung segment (or lobule) that is separated from the normal lung (located either within or outside it). This segment usually receives its arterial blood supply directly from the aorta and its venous drainage via the inferior pulmonary vein or azygos or hemiazygos veins. Impaired bronchial drainage will cause changes previously described as cystic alveolar dysplasia.
Reference
Leslie KO, Wick MR. Practical pulmonary pathology: a diagnostic approach. Philadelphia: Churchill Livingstone; 2005.
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Krueger, G.R.F., Wagner, M., Oldham, S.A.A. (2013). Pathology of the Respiratory Tract. In: Krueger, G., Buja, L. (eds) Atlas of Anatomic Pathology with Imaging. Springer, London. https://doi.org/10.1007/978-1-4471-2846-5_3
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