Interstitial Lung Diseases

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Interstitial Lung Diseases

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.

During the chronic stage the general inflammatory response is characterized by the infiltration of numerous white blood cells (especially monocytes, macrophages, and lymphocytes), and some fibroblasts may also be present in the alveolar walls and interstitial spaces. This stage may be followed by further interstitial thickening, fibrosis, granulomas, and, in some cases, honeycombing and cavity formation. Pleural effusion also may be present. In the chronic stages the basic pathologic features of interstitial fibrosis are identical in any interstitial lung disorder (so-called end-stage pulmonary fibrosis).

As a general rule, the interstitial lung disorders produce a restrictive lung disease. However, because bronchial inflammation and excessive airway secretions also can develop in the small airways, the clinical manifestations associated with an obstructive lung disorder may also be seen. Therefore the patient with ILD may demonstrate a restrictive disorder, an obstructive disorder, or a combination of both.

The major pathologic or structural changes associated with chronic ILDs are as follows:

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.

Radiation Therapy Irritant Gases

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Interstitial Lung Diseases of Known Causes or Associations

Occupational, Environmental and Therapeutic Exposures

Inorganic particulate (dust) exposure

Asbestos

Exposure to asbestos may cause asbestosisa 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.

Coal dust

The pulmonary deposition and accumulation of large amounts of coal dust causes what is known as coal worker’s pneumoconiosis (CWP). CWP also is known as coal miner’s lung and black lung. Miners who use cutting machines at the coal face have the greatest exposure, but even relatively minor exposures may result in the disease. Indeed, cases have been reported in which coal miners’ wives developed the disease, presumably from shaking the dust from their husbands’ work clothes.

Simple CWP is characterized by the presence of pinpoint nodules called coal macules (black spots) throughout the lungs. The coal macules often develop around the first- and second-generation respiratory bronchioles and cause the adjacent alveoli to retract. This condition is called focal emphysema.

Complicated CWP or progressive massive fibrosis (PMF) is characterized by areas of fibrotic nodules greater than 1 cm in diameter. The fibrotic nodules generally appear in the peripheral regions of upper lobes and extend toward the hilum with growth. The nodules are composed of dense collagenous tissue with black pigmentation. Coal dust by itself is chemically inert. The fibrotic changes in CWP are usually caused by silica.

Silica

Silicosis (also called grinder’s disease or quartz silicosis) is caused by the chronic inhalation of crystalline, free silica, or silicon dioxide particles. Silica is the main component of more than 95% of the rocks of the earth. It is found in sandstone, quartz (beach sand is mostly quartz), flint, granite, many hard rocks, and some clays.

Simple silicosis is characterized by small rounded nodules scattered throughout the lungs. No single nodule is greater than 9 mm in diameter. Patients with simple silicosis are usually symptom-free.

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*

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*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.

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