5 курс / Пульмонология и фтизиатрия / Clinical_Manifestations_and_Assessment_of_Respiratory

TABLE 27.2

Causes of Hypersensitivity Pneumonitis

*HP, Hypersensitivity pneumonitis.

From Selman, M. (2003). Hypersensitivity pneumonitis. In Schwarz, M. I., & Kin, T. E. (Eds.), Interstitial lung disease (4th ed.). Hamilton, Ontario, Canada: 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 27.4). Unfortunately, the lungs are a major target organ 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.

Box 27.4

Medications and Illicit Drugs Associated With the Development of Interstitial Lung Disease

•Antibiotics

•Nitrofurantoin

•Sulfasalazine

•Antiinflammatory agents

•Aspirin

•Gold

•Penicillamine

•Methotrexate

•Etanercept

•Infliximab

•Cardiovascular agents

•Amiodarone

•Tocainide

•Chemotherapeutic agents

•Bleomycin

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•Mitomycin-C

•Busulfan

•Cyclophosphamide

•Chlorambucil

•Melphalan

•Azathioprine

•Cytosine arabinoside

•Methotrexate

•Procarbazine

•Zinostatin

•Etoposide

•Vinblastine

•Imatinib

•Flutamide

•Drug-induced systemic lupus erythematosus

•Procainamide

•Isoniazid

•Hydralazine

•Hydantoins

•Penicillamine

•Illicit drugs (controlled substances)

•Heroin

•Methadone

•Propoxyphene

•Talc as an intravenous contaminant

•Miscellaneous agents

•Oxygen

•Drugs inducing pulmonary infiltrates and eosinophilia: L-tryptophan

•Hydrochlorothiazide

•Radiation

From Camus, P. (2003). Drug-induced infiltrative lung diseases. In Schwarz, M. I., & King, T. E. (Eds.), Interstitial lung disease (4th ed.). Hamilton, Ontario, Canada: BC Decker.

The chemotherapeutics (anticancer agents) are by far the largest group of agents associated with ILD. Bleomycin, mitomycin, busulfan, cyclophosphamide, methotrexate, and carmustine (BCNU) are the major offenders. Nitrofurantoin (an antibacterial drug used in the treatment of urinary tract infections) is also associated with ILD. Gold and penicillamine for the treatment of rheumatoid arthritis also have been shown to cause ILD. The excessive long-term administration of oxygen (oxygen toxicity) is known to cause diffuse pulmonary injury and fibrosis (see Chapter 11, Respiratory Insufficiency, Respiratory Failure, and Ventilatory Management Protocols). As a general rule, the risk for these drugs causing an interstitial lung disorder is directly related to the cumulative dosage. However, drug-induced interstitial disease may be seen as early as 1 month to as late as several years after exposure to these agents.

The precise cause of drug-induced ILD is not known. Diagnosis is confirmed by an open lung biopsy. When interstitial fibrosis is found with no infectious organisms or known industrial exposure, a drug-induced interstitial process must be suspected.

Radiation Therapy.

Radiation therapy in the management of cancer may cause ILD. Radiation-induced lung disease is commonly divided into two major phases: the acute pneumonitic phase and the late fibrotic phase. Acute pneumonitis is rarely seen in patients who receive a total radiation dose of less than 3500 rad. By contrast, doses in excess of 6000 rad over 6 weeks almost always cause ILD in and near the radiated areas. The acute pneumonitic phase develops about 2 to 3 months after exposure. Chronic radiation fibrosis is seen in all patients who develop acute pneumonitis.

The late phase of fibrosis may develop (1) immediately after the development of acute pneumonitis, (2) without an acute pneumonitic period, or (3) after a symptom-free latent period. When fibrosis does develop, it generally does so 6 to 12 months after radiation exposure. Pleural effusion is often associated with the late fibrotic phase.

The precise cause of radiation-induced lung disease is not known. The establishment of a diagnosis is similar to that for drug-induced interstitial disease (i.e., by obtaining a history of recent radiation therapy and confirming the diagnosis with an open lung biopsy).

Irritant Gases.

The inhalation of irritant gases may cause acute chemical pneumonitis and, in severe cases, ILD. Most exposures occur in an industrial setting. Table 27.3 lists some of the more common irritant gases and the industrial settings where they may be found.

TABLE 27.3

Common Irritant Gases Associated With Interstitial Lung Disease

Systemic Diseases

Connective Tissue (Collagen Vascular) Diseases

Scleroderma.

Scleroderma is characterized by chronic hardening and thickening of the skin caused by new collagen formation. It may

occur in a localized form or as a systemic disorder (called systemic sclerosis). Progressive systemic sclerosis (PSS) is a relatively rare autoimmune disorder that affects the blood vessels and connective tissue. It causes fibrous degeneration of the connective tissue of the skin, lungs, and internal organs, especially the esophagus, digestive tract, and kidney.

Scleroderma of the lung appears in the form of ILD and fibrosis. Of all the collagen vascular disorders, scleroderma is the one in which pulmonary involvement is most severe and most likely to cause significant scarring of the lung parenchyma (see Fig. 27.3). The pulmonary complications include diffuse interstitial fibrosis, severe pulmonary hypertension, pleural disease, and aspiration pneumonitis (secondary to esophageal involvement). Scleroderma also may involve the small pulmonary blood vessels and appears to be independent of the fibrotic process involving the alveolar walls. The disease is most commonly seen in women 30 to 50 years of age.

FIGURE 27.3 Reticulonodular pattern of interstitial pulmonary fibrosis in a patient with scleroderma. (From Hansell, D. M., Lynch, D. A., McAdams, H. P., et al. [2010]. Imaging of diseases of the chest [5th ed.]. Philadelphia, PA: Elsevier.)

Rheumatoid Arthritis.

Rheumatoid arthritis is primarily an inflammatory joint disease. It may, however, involve the lungs in the form of (1) pleurisy, with or without effusion; (2) interstitial pneumonitis; (3) necrobiotic nodules, with or without cavities; (4) Caplan syndrome; and (5) pulmonary hypertension secondary to pulmonary vasculitis.

Pleurisy with or without effusion is the most common pulmonary complication associated with rheumatoid arthritis. When present, the effusion is generally unilateral (often on the right side) (see Fig. 27.9). Men appear to develop rheumatoid pleural complications more often than women. Rheumatoid interstitial pneumonitis is characterized by alveolar wall fibrosis, interstitial and intraalveolar mononuclear cell infiltration, and lymphoid nodules. In severe cases, extensive fibrosing alveolitis and honeycombing may develop. Rheumatoid interstitial pneumonitis is also more common in male patients. Necrobiotic nodules are characterized by the gradual degeneration and swelling of lung tissue.

The pulmonary nodules generally appear as well-circumscribed masses that often progress to cavitation. The nodules usually develop in the periphery of the lungs and are more common in men. Histologically, the pulmonary nodules are identical to the subcutaneous nodules that develop in rheumatoid arthritis.

Caplan syndrome (also called rheumatoid pneumoconiosis) is a progressive pulmonary fibrosis of the lung commonly seen in coal miners. Caplan syndrome is characterized by rounded densities in the lung periphery that often undergo cavity formation and, in some cases, calcification. Pulmonary hypertension is a common secondary complication caused by the progression of fibrosing alveolitis and pulmonary vasculitis.

Sjögren Syndrome.

Sjögren syndrome is a lymphocytic infiltration that primarily involves the salivary and lacrimal glands and manifests by dry mucous membranes, usually of the mouth and eyes. Pulmonary involvement frequently occurs in Sjögren syndrome and includes (1) pleurisy with or without effusion, (2) interstitial fibrosis that is indistinguishable from that of other collagen vascular disorders, and (3) infiltration of lymphocytes of the tracheobronchial mucous glands, which in turn causes atrophy of the mucous glands, mucous plugging, atelectasis, and secondary infections. Sjögren syndrome occurs most often in women (90%) and is commonly associated with rheumatoid arthritis (50% of patients with Sjögren syndrome).

Polymyositis-Dermatomyositis.

Polymyositis is a diffuse inflammatory disorder of the striated muscles that primarily weakens the limbs, neck, and pharynx. Dermatomyositis is the term used when an erythematous skin rash accompanies the muscle weakness. Pulmonary involvement develops in response to (1) recurrent episodes of aspiration pneumonia caused by esophageal weakness and atrophy, (2) hypostatic pneumonia secondary to a weakened diaphragm, and (3) drug-induced interstitial pneumonitis.

Polymyositis-dermatomyositis is seen more often in women than men, at about a 2 : 1 ratio. The disease occurs primarily in two age groups: before the age of 10 years and from 40 to 50 years of age. In about 40% of the patients, the pulmonary manifestations are seen 1 to 24 months before the striated muscle or skin shows signs or symptoms.

Systemic lupus erythematosus (SLE) is a multisystem disorder that mainly involves the joints and skin. It also may cause serious problems in numerous other organs, including the kidneys, lungs, nervous system, and heart. Involvement of the lungs appears in about 50% to 70% of cases. Pulmonary manifestations are characterized by (1) pleurisy with or without effusion, (2) atelectasis, (3) diffuse infiltrates and pneumonitis, (4) diffuse ILD, (5) uremic pulmonary edema, (6) diaphragmatic dysfunction, and (7) infections.

Pleurisy with or without effusion is the most common pulmonary complication of SLE. The effusions are usually exudates with high protein concentration and are frequently bilateral. Atelectasis commonly develops in response to the pleurisy,

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effusion, and diaphragmatic elevation associated with SLE. Diffuse noninfectious pulmonary infiltrates and pneumonitis are common. In severe cases, chronic interstitial pneumonitis may develop. Because SLE frequently impairs the renal system, uremic pulmonary edema may occur. SLE also has been found to be associated with diaphragmatic dysfunction and reduced lung volumes. Some research suggests that a diffuse myopathy affecting the diaphragm is the source of this problem. About 50% of cases have a complicating pulmonary infection.

Sarcoidosis.

Sarcoidosis is a relatively common chronic disorder of unknown origin characterized by the formation of tubercles of nonnecrotizing epithelioid tissue (noncaseating granulomas). Common sites are the eyes, lungs, spleen, liver, skin, mucous membranes, and lacrimal and salivary glands, usually with the involvement of the lymph glands. The lung is the most frequently affected organ, with manifestations generally including ILD, enlargement of the mediastinal lymph nodes, or a combination of both (see Fig. 27.7). One of the clinical laboratory hallmarks of sarcoidosis is an increase in all three major immunoglobulins (IgM, IgG, and IgA). Also common is an elevation of the angiotensin converting enzyme (ACE test). The disease is more common among African-Americans and appears most frequently in patients 10 to 40 years of age, with the highest incidence at age 20 to 30. Women are affected more often than men, especially among African-Americans.

Idiopathic Interstitial Pneumonias

Some patients with ILD do not have a readily identified specific exposure, a systemic disorder, or an underlying genetic cause. Such conditions are commonly placed in the idiopathic interstitial pneumonia (ILD) group or the group with other specific pathology.

Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive inflammatory disease with varying degrees of fibrosis and, in severe cases, honeycombing. The precise cause is unknown. Although idiopathic pulmonary fibrosis is the term most frequently used for this disorder, numerous other names appear in the literature, such as acute interstitial fibrosis of the lung, cryptogenic fibrosing alveolitis, Hamman-Rich syndrome, honeycomb lung, interstitial fibrosis, and interstitial pneumonitis.

IPF is commonly separated into the following two major disease entities according to it's predominant histologic appearance: desquamative interstitial pneumonia (DIP) and usual interstitial pneumonia (UIP). In DIP the most prominent features are hyperplasia and desquamation of the alveolar type II cells. The alveolar spaces are packed with macrophages, and there is an even distribution of the interstitial mononuclear infiltrate.

In UIP the most prominent features are interstitial and alveolar wall thickening caused by chronic inflammatory cells and fibrosis. In severe cases, fibrotic connective tissue replaces the alveolar walls, the alveolar architecture becomes distorted, and eventually honeycombing develops. When honeycombing is present, the inflammatory infiltrate is significantly reduced. The prognosis for patients with DIP is significantly better than that for patients with UIP.

Some experts think DIP and UIP are two distinct ILD entities. Others, however, think DIP and UIP are different stages of the same disease process. IPF is most commonly seen in men 40 to 70 years of age. Diagnosis is generally confirmed by an open lung biopsy. Most patients diagnosed with IPF have a more chronic progressive course, and death usually occurs in 4 to 10 years. Death usually is the result of progressive acute ventilatory failure, complicated by pulmonary infection.

Cryptogenic Organizing Pneumonia

Cryptogenic organizing pneumonia (COP) (also known as bronchiolitis obliterans organizing pneumonia [BOOP]) is characterized by connective tissue plugs in the small airways (hence the term bronchiolitis obliterans) and mononuclear cell infiltration of the surrounding parenchyma (hence the term organizing pneumonia). Although most cases have no identifiable cause and therefore are considered idiopathic, COP has been associated with various connective tissue diseases, toxic gas inhalation, and infection. The chest radiograph commonly shows patchy infiltrates of alveolar rather than interstitial involvement. Diagnosis may require a surgical biopsy when the clinical and radiographic data are uncertain. COP is one of the ILDs in which both restrictive and obstructive pathophysiologic findings are present.

Lymphocytic Interstitial Pneumonia

Lymphocytic interstitial pneumonia (LIP) is a diffuse pulmonary disorder characterized by fibrosis and accumulation of lymphocytes in the lungs. It is commonly associated with lymphoma. The diagnosis usually requires a surgical lung biopsy.

Specific Pathology

Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare lung disease involving the smooth muscles of the airways and affects women of childbearing age. It is characterized by the proliferation of disorderly smooth muscle proliferation throughout the bronchioles, alveolar septa, perivascular spaces, and lymphatics. LAM causes the obstruction of small airways and lymphatics. Common clinical features associated with LAM are recurrent pneumothorax and chylothorax. The diagnosis of LAM is confirmed with an open lung biopsy. The prognosis is poor; the disease slowly progresses over 2 to 10 years, ending in death resulting from ventilatory failure.

Pulmonary Langerhans Cell Histiocytosis

Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related ILD characterized by midlung zone star-shaped nodules with adjacent thin-walled cysts. It was once considered a benign condition in adults, but long-term complications such as pulmonary hypertension are becoming increasingly recognized. Diagnosis is confirmed histologically by tissue biopsy.

Pulmonary Alveolar Proteinosis

Pulmonary alveolar proteinosis is a condition of unknown cause in which the alveoli become filled with protein and lipids. The lipoprotein material is similar to the pulmonary surfactant produced by type II cells. In addition, the alveolar macrophages are generally dysfunctional in this disorder. The disease is most commonly seen in adults 20 to 50 years of age. Men are affected twice as often as women. The chest radiograph typically reveals bilateral infiltrates that are most prominent in the perihilar regions (butterfly pattern). It is often indistinguishable from pulmonary edema. Air bronchograms are commonly seen. The diagnosis is confirmed by transbronchial or open lung biopsy or by analysis of fluid removed during bronchial lavage.

Pulmonary Vasculitides

The pulmonary vasculitides (also called granulomatous vasculitides) consist of a heterogeneous group of pulmonary disorders characterized by inflammation and destruction of the pulmonary vessels. The major disorders in this category include Wegener granulomatosis, Churg-Strauss syndrome, and lymphomatoid granulomatosis.

Wegener Granulomatosis.

Wegener granulomatosis is a multisystem disorder characterized by (1) a necrotizing, granulomatous vasculitis, (2) focal and segmental glomerulonephritis, and (3) variable degrees of systemic vasculitis of the small veins and arteries. In the lungs, numerous 1- to 9-cm-diameter nodules are commonly seen in the upper lobes, and cavity formation is often associated with larger lesions (see Fig. 27.8).

Wegener granulomatosis is considered an aggressive and fatal disorder, although the prognosis has significantly improved with the use of cytotoxic agents (e.g., cyclophosphamide). This disorder is most commonly seen in men older than 50 years of age. Diagnosis is confirmed by an open lung biopsy. Histologic examination reveals lesions with marked central necrosis of the pulmonary parenchyma. The area surrounding the necrotizing lesion contains numerous inflammatory white blood cells with some fibroblasts. Inflammatory cell infiltrate and necrotizing vasculitis are seen in the adjacent blood vessels.

Churg-Strauss Syndrome.

Churg-Strauss syndrome is a necrotizing vasculitis that predominantly involves the small vessels of the lungs. The granulomatous lesions are characterized by a heavy infiltrate of eosinophils, central necrosis, and peripheral eosinophilia. Cavity formation is rare in this disorder. Clinically, symptoms of asthma usually precede the onset of vasculitis. In recent years, rapid tapering of oral steroids with substitution of leukotriene inhibitors such as montelukast (Singulair) and zafirlukast (Accolate) have been associated with deaths from fulminant Churg-Strauss syndrome reactions. Neurologic disorders such as mononeuritis multiplex, a simultaneous disease of several peripheral nerves, are frequently associated with this disorder. Diagnosis is usually confirmed with an open lung biopsy, and the disease is often rapidly fatal.

Lymphomatoid Granulomatosis.

Lymphomatoid granulomatosis is a rare necrotizing vasculitis that primarily involves the lungs, although neurologic and cutaneous lesions are sometimes seen. The lesions are usually in the lower lobes, and cavities develop in more than onethird of cases. Pleural effusion is common.

Although the clinical presentation is similar to that of Wegener granulomatosis, there are some distinct differences. For example, more mature lymphoreticular cells are involved in the formation of the granulomatous lesions and no glomerulonephritis is seen. Histologically, the lesions simulate malignant lymphoma. This disorder is most commonly seen in men 50 to 70 years of age. Diagnosis is confirmed by open lung biopsy.

Miscellaneous Diffuse Interstitial Lung Diseases

Goodpasture Syndrome

Goodpasture syndrome is a disease of unknown cause that involves two organ systems: the lungs and the kidneys. In the lungs there are recurrent episodes of pulmonary hemorrhage and hemoptysis and in some cases pulmonary fibrosis, presumably as a consequence of episodes of pulmonary bleeding. In the kidneys there is a glomerulonephritis characterized by the infiltration of antibodies within the glomerular basement membrane (GBM). These circulating antibodies function against the patient's own GBM. They are commonly abbreviated as anti-GBM antibodies. It is thought that the anti-GBM antibodies cross-react with the basement membrane of the alveolar wall and that their deposition in the kidneys and lungs is responsible for producing the pathophysiologic processes of the disease.

Goodpasture syndrome is usually seen in young adults. With appropriate treatment, the 5-year survival rate is almost 80%. An interesting feature of Goodpasture syndrome is that the patient frequently demonstrates an increased pulmonary diffusion capacity (DLCO), which is in direct contrast to most interstitial lung disorders. The increased carbon monoxide uptake commonly seen in this disorder is thought to be caused by the increased amount of retained hemoglobin in the pulmonary tissue.

Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis is an entity of unknown cause that is characterized by recurrent episodes of pulmonary hemorrhage similar to that seen in Goodpasture syndrome. Histologic examination reveals an alveolar hemorrhage with hemosiderin-laden macrophages and hyperplasia of the alveolar epithelium. Unlike in Goodpasture syndrome, however, there is no evidence of circulating anti-GBM antibodies attacking the alveoli or GBMs, and this disorder is not associated with renal disease.

Idiopathic pulmonary hemosiderosis is most often seen in children. As in Goodpasture syndrome, patients commonly demonstrate an increased DLCO, which is in direct contrast to most interstitial lung disorders. Again, the increased uptake of carbon monoxide is thought to be caused by the increased amount of hemoglobin retained in the lungs.

Chronic Eosinophilic Pneumonia

Chronic eosinophilic pneumonia is characterized by infiltration of eosinophils and, to a lesser extent, macrophages into the alveolar and interstitial spaces. Clinically, a unique feature of this disorder is often seen on the chest radiograph, consisting of a peripheral distribution of pulmonary infiltrates. This radiographic pattern is commonly referred to as a photographic negative of pulmonary edema. This is because of the dense peripheral infiltration, with the sparing of the perihilar areas, seen in chronic eosinophilic pneumonia compared with the central pulmonary infiltration with the sparing of the lung periphery seen in pulmonary edema. An increased number of eosinophils is also commonly seen in the peripheral blood. Histologic diagnosis is made by means of an open lung biopsy.

Overview of the Cardiopulmonary Clinical Manifestations Associated With Chronic Interstitial Lung Diseases

The following clinical manifestations result from the pathophysiologic mechanisms caused (or activated) by an increased alveolar-capillary membrane thickness (see Fig. 10.9) and excessive bronchial secretions (see Fig. 10.11)—the major anatomic alterations of the lungs associated with chronic interstitial lung disease (ILD) (see Fig. 27.1).

Clinical Data Obtained at the Patient's Bedside

The Physical Examination

Vital Signs

Increased Respiratory Rate (Tachypnea)

Several pathophysiologic mechanisms operating simultaneously may lead to an increased ventilatory rate:

•Stimulation of peripheral chemoreceptors (hypoxemia)

•Relationship of decreased lung compliance to increased ventilatory rate

•Stimulation of the J receptors

•Pain, anxiety

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Increased Heart Rate (Pulse) and Blood Pressure

Cyanosis

Digital Clubbing

Peripheral Edema and Venous Distention

Because polycythemia and cor pulmonale are associated with chronic ILD, the following may be seen:

•Distended neck veins

•Pitting edema

•Enlarged and tender liver

Nonproductive Cough Chest Assessment Findings

•Increased tactile and vocal fremitus

•Dull percussion note

•Bronchial breath sounds

•Crackles

•Pleural friction rub

•Whispered pectoriloquy

Clinical Data Obtained From Laboratory Tests and Special Procedures

Pulmonary Function Test Findings

Moderate to Severe Interstitial Lung Disease (Restrictive Lung Pathology)

Forced Expiratory Volume and Flow Rate Findings

Lung Volume and Capacity Findings

Decreased Diffusion Capacity

There is an exception to the expected decreased diffusion capacity in the two ILDs Goodpasture syndrome and idiopathic pulmonary hemosiderosis. The DLCO is often elevated in response to the increased amount of hemoglobin retained in the alveolar spaces that is associated with these two disorders.

Arterial Blood Gases

Mild to Moderate Interstitial Lung Disease

Acute Alveolar Hyperventilation With Hypoxemia1 (Acute Respiratory Alkalosis)

Severe Chronic Interstitial Lung Disease

Chronic Ventilatory Failure With Hypoxemia2 (Compensated Respiratory Acidosis)

Acute Ventilatory Changes Superimposed on Chronic Ventilatory Failure3

Because acute ventilatory changes are frequently seen in patients with chronic ventilatory failure, the respiratory therapist must be familiar with—and alert for—the following two dangerous arterial blood gas (ABG) findings:

•Acute alveolar hyperventilation superimposed on chronic ventilatory failure, which should further alert the respiratory therapist to record the following important ABG assessment: possible impending acute ventilatory failure

•Acute ventilatory failure (acute hypoventilation) superimposed on chronic ventilatory failure

1See Fig. 5.2 and Table 5.4 and related discussions for the acute pH, PaCO2, and changes associated with acute

alveolar hyperventilation.

2See Table 5.7, Table 5.8, and Table 5.9 and related discussion for the pH, PaCO2, and changes associated with chronic ventilatory failure.

3See Table 5.7, Table 5.8, and Table 5.9 and related discussion for the pH, PaCO2, and changes associated with acute ventilatory changes superimposed on chronic ventilatory failure.

Oxygenation Indices4

Moderate to Severe Stage Interstitial Lung Disease

5The DO2 may be normal in patients who have compensated to the decreased oxygenation status with (1) an increased cardiac output, (2) an increased hemoglobin level, or (3) a combination of both. When the DO2 is normal, the O2ER is usually normal.

oxygen saturation; V̇O2, oxygen consumption.

Hemodynamic Indices6

(Severe Interstitial Lung Disease)

6CO, Cardiac output; CVP, central venous pressure; LVSWI, left ventricular stroke work index; , mean pulmonary artery pressure; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; RVSWI, right ventricular stroke work index; SV, stroke volume; SVI, stroke volume index; SVR, systemic vascular resistance.

Laboratory Findings

• Increased hematocrit and hemoglobin (polycythemia)

Radiologic Findings

Radiologic findings vary according to the cause.

Chest Radiograph

•Bilateral reticulonodular pattern

•Irregularly shaped opacities

•Granulomas

•Cavity formation

•Honeycombing

•Pleural effusion (see Chapter 24, Pleural Effusion and Empyema)

•Pleural Thickening

As shown in Fig. 27.3, in a patient with severe scleroderma, a bilateral reticulonodular pattern is commonly seen on the radiographs. In patients with asbestosis the opacity is often described as cloudy or as having a ground-glass appearance or pleural thickening and is especially apparent in the lower lobes (Fig. 27.4). Calcified pleural plaques may be seen on the superior border of the diaphragm or along the chest wall (Fig. 27.5). The inflammatory response elicited by the asbestos fibers may produce a fuzziness and irregularity of the cardiac and diaphragmatic borders.

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FIGURE 27.4 Chest x-ray film of a patient with asbestosis. See red arrow for pleural thickening.

FIGURE 27.5 Calcified pleural plaques on the superior border of the diaphragm (arrows) in a patient with asbestosis. Thickening of the pleural margins is also seen along the lower lateral borders of the chest. (A) Anteroposterior view. (B) Lateral view.

Fig. 27.6 shows a diffuse parenchymal ground-glass pattern with some areas of consolidation in a patient with acute farmer lung. The severity of parenchymal opacification in this case is rare.

FIGURE 27.6 Acute farmer lung. Chest radiograph shows diffuse parenchymal ground-glass pattern with some areas of consolidation. The severity of parenchymal opacification in this case is unusual. (From Hansell, D. M., Lynch, D. A., McAdams, H. P., et al.

[2010]. Imaging of diseases of the chest [5th ed.]. Philadelphia, PA: Elsevier.)

In Fig. 27.7 the honeycomb appearance is nicely illustrated in a computed tomography (CT) scan of a patient with

sarcoidosis. Fig. 27.8 shows a patient with Wegener granulomatosis with numerous nodules with a large cavitary lesion adjacent to the right hilus. Fig. 27.9 shows a pleural effusion in a patient with rheumatoid disease.

FIGURE 27.7 Honeycomb cysts in sarcoidosis. High-resolution computed tomography (HRCT) through the right midlung shows perfuse clustered honeycomb cysts. The cysts are larger than the typical honeycomb cysts seen in usual interstitial pneumonia. Cysts are much less extensive in the left lung. (From Hansell, D. M., Lynch, D. A., McAdams, H. P., et al. [2010]. Imaging of diseases of the chest

[5th ed.]. Philadelphia, PA: Elsevier.)

FIGURE 27.8 Wegener granulomatosis. Numerous nodules with a large (6-cm) cavitary lesion adjacent to the right hilus. Its walls are thick and irregular. (From Hansell, D. M., Lynch, D. A., McAdams, H. P., et al. [2010]. Imaging of diseases of the chest [5th ed.]. Philadelphia, PA:

Elsevier.)

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