Interstitial Lung Diseases
After reading this chapter, you will be able to:
• List the anatomic alterations of the lungs associated with chronic interstitial lung disease.
• Describe the causes of chronic interstitial lung disease.
• List the cardiopulmonary clinical manifestations associated with chronic interstitial lung disease.
• Describe the general management of chronic interstitial lung disease.
• Describe the clinical strategies and rationales of the SOAPs presented in the case study.
• Define key terms and complete self-assessment questions at the end of the chapter and on Evolve.
Anatomic Alterations of the Lungs
The anatomic alterations of ILD may involve the bronchi, alveolar walls, and adjacent alveolar spaces. In severe cases the extensive inflammation leads to pulmonary fibrosis, granulomas, honeycombing, and cavitation. During the acute stage of any ILD, the general inflammatory condition is characterized by edema and the infiltration of a variety of white blood cells (e.g., neutrophils, eosinophils, basophils, monocytes, macrophages, and lymphocytes) in the alveolar walls and interstitial spaces (see Figure 25-1, A). Bronchial inflammation and thickening and increasing airway secretions may be also present.
The major pathologic or structural changes associated with chronic ILDs are as follows:
• Destruction of the alveoli and adjacent pulmonary capillaries
• Fibrotic thickening of the respiratory bronchioles, alveolar ducts, and alveoli
• Honeycombing and cavity formation
• Fibrocalcific pleural plaques (particularly in asbestosis)
• Excessive bronchial secretions (caused by inflammation of airways)
Etiology and Epidemiology
Because there are over 180 different pulmonary disorders classified as ILD, it is helpful to group them according to their occupational or environmental exposure, disease associations, and specific pathology. Table 25-1 provides an overview of common ILD groups. A discussion of the more common ILDs follows.
Table 25-1
Interstitial Lung Diseases of Known Causes or Associations
Occupational, Environmental and Therapeutic Exposures
Inorganic particulate (dust) exposure
Asbestos
Exposure to asbestos may cause asbestosis—a common form of ILD. Asbestos fibers are a mixture of fibrous minerals composed of hydrous silicates of magnesium, sodium, and iron in various proportions. There are two primary types: the amphiboles (crocidolite, amosite, and anthophyllite) and chrysotile (most commonly used in industry). Asbestos fibers typically range from 50 to 100 µm in length and are about 0.5 µm in diameter. The chrysotiles have the longest and strongest fibers. Box 25-1 lists common sources associated with asbestos fibers.
As shown in Figure 25-1, B, asbestos fibers can be seen by microscope within the thickened septa as brown or orange baton-like structures. The fibers characteristically stain for iron with Perls’ stain. The pathologic process may affect only one lung, a lobe, or a segment of a lobe. The lower lobes are most commonly affected. Pleural calcification is common and diagnostic in patients with an asbestos exposure history.
Silica
Complicated silicosis is characterized by nodules that coalesce and form large masses of fibrous tissue, usually in the upper lobes and perihilar regions. In severe cases the fibrotic regions may undergo tissue necrosis and cavitate. Box 25-2 lists common occupations associated with silica exposure.
Organic materials exposure
Hypersensitivity pneumonitis
Hypersensitivity pneumonitis (also called allergic alveolitis or extrinsic allergic alveolitis) is a cell-mediated immune response of the lungs caused by the inhalation of a variety of offending agents or antigens. Such antigens include grains, silage, bird droppings or feathers, wood dust (especially redwood and maple), cork dust, animal pelts, coffee beans, fish meal, mushroom compost, and molds that grow on sugar cane, barley, and straw. The immune response to these allergens causes production of antibody and an inflammatory response. The lung inflammation, or pneumonitis, develops after repeated and prolonged exposure to the allergen. The term hypersensitivity pneumonitis (or allergic alveolitis) is often renamed according to the type of exposure that caused the lung disorder. For example, the hypersensitivity pneumonitis caused by the inhalation of moldy hay is called farmer’s lung. Table 25-2 provides common causes, exposure sources, and disease syndromes associated with hypersensitivity pneumonitis.
Table 25-2
Causes of Hypersensitivity Pneumonitis
Antigen | Exposure Source | Disease (Syndrome) |
Bacteria, Thermophilic | ||
Saccharopolyspora rectivirgula | Moldy hay, silage | Farmer’s lung |
Thermoactinomyces vulgaris | Moldy sugarcane | Bagassosis |
Thermoactinomyces sacchari | Mushroom compost | Mushroom worker’s lung |
Thermoactinomyces candidus | Heated water reservoirs | Air conditioner lung |
Bacteria, Nonthermophilic | ||
Bacillus subtilis, Bacillus cereus | Water, detergent | Humidifier lung, washing powder lung |
Fungi | ||
Aspergillus species | Moldy hay | Farmer’s lung |
Water | Ventilation pneumonitis | |
Aspergillus clavatus | Barley | Malt worker’s lung |
Penicillium casei, | Cheese | Cheese washer’s lung |
Penicillium roqueforti | ||
Alternaria species | Wood pulp | Woodworker’s lung |
Cryptostroma corticale | Wood bark | Maple bark stripper’s lung |
Graphium, Aureobasidium pullulans | Wood dust | Sequoiosis |
Merulius lacrymans | Rotten wood | Dry root lung |
Penicillium frequentans | Cork dust | Suberosis |
Aureobasidium pullulans | Water | Humidifier lung |
Cladosporium species | Hot tub mist | Hot tub HP* |
Trichosporon cutaneum | Damp wood and mats | Japanese summer-type HP* |
Amebae | ||
Naegleria gruberi | Contaminated water | Humidifier lung |
Acanthamoeba polyphaga | Contaminated water | Humidifier lung |
Acanthamoeba castellani | Contaminated water | Humidifier lung |
Animal Protein | ||
Avian proteins | Bird droppings, feathers | Bird-breeder’s lung |
Urine, serum, pelts | Rates, gerbils | Animal handler’s lung |
Chemicals | ||
Isocyanates, trimellitic anhydride | Paints, resins, plastics | Chemical worker’s lung |
Copper sulfate | Bordeaux mixture | Vineyard sprayer’s lung |
Phthalic anhydride | Heated epoxy resin | Epoxy resin lung |
Sodium diazobenzene sulfate | Chromatography reagent | Pauli’s reagent alveolitis |
Pyrethrum | Pesticide | Pyrethrum HP* |
*HP, Hypersensitivity pneumonitis.
From Selman M: Hypersensitivity pneumonitis. In Schwarz MI, Kin TE, eds: Interstitial lung disease, ed 4, Hamilton, 2003, BC Decker.
Medications and illicit drugs
As the list of medications and illicit drugs continues to grow, so does the list of possible side effects (Box 25-4). Unfortunately, the lungs are major target organs affected by these side effects. Although it is impossible to discuss in detail the various lung-related side effects of every drug, it is possible to describe some of the general concerns related to drug-induced lung disease and to list some of the pharmacologic agents that may be responsible.