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The practice questions listed below correlate well with Egan’s Chapter 26, so you can use this information to help prepare for your exams.

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Interstitial Lung Disease Practice Questions:

1. What does ILD stand for?
ILD stands for Interstitial Lung Disease. We needed to clear that up in question #1 so that now your will know when reference ILD throughout the rest of this study guide. đŸ™‚

2. ILDs are a diverse group of illnesses that can be organized into groups based on what?
Based on related causes.

3. ILDs generally cause what?
They cause chronic, progressive dyspnea on exertion and cough.

4. Findings on examination of ILD are often limited to the chest in the form of what?
Fine, inspiratory crackles.

5. The most common chest radiograph finding in ILD is what?
Diffuse reticular or reticulonodular infiltrates with reduced lung volumes.

6. What are the PFTs like for Interstitial Lung Diseases patients?
Pulmonary function testing usually reveals restrictive physiology and decreased diffusion capacity; however, other patterns can be seen.

7. What are the causes of the ILDs?
The causes are diverse but are most frequently from exposure (tobacco, hypersensitivity pneumonitis antigens, silica, asbestos), autoimmune dysfunction (sarcoidosis, connective tissue disease associated), and abnormal injury healing (IPF).

8. Nonspecific treatment may be considered in all ILD patients (e.g., supplemental oxygen, pulmonary rehabilitation), but specific ILD treatment depends on what?
It depends on the underlying disease and may consist of immunosuppressive.

9. What is the most common treatment for interstitial lung disease?
A lung transplant.

10. What is a common physical finding with ILD?
Bibasilar inspiratory dry crackles in the bases.

11. What is the classification of ILD based on?

12. What is the most effective way to diagnose ILD?
Open lung biopsy.

13. What type of lung disease is usually seen in smokers?

14. What form of ILD can present with warm, red, and raised lesions on the lower extremities and a chest x-ray showing bilateral hilar lymphadenopathy?

15. What population is sarcoidosis usually seen in?
African American.

16. How would stage 2 sarcoidosis look on a chest x-ray?
Bilateral hilar LA with infiltrates.

17. What changes occur in stage 3 sarcoidosis?
The infiltrates are still present but the hilar LA disappear.

18. What form of ILD is typically seen in men 50-70 years old with progressive dyspnea and bibasilar crackles?
Idiopathic Pulmonary Fibrosis.

19. What form of ILD presents with intermittent episodes of dyspnea and is seen in people who live on farms?
Hypersensitivity pneumonitis.

20. What is the most important thing to find when diagnosing hypersensitivity pneumonitis?
The patient’s history and work history.

21. What does the diagnosis of drug-induced interstitial disease rely on?
The exclusion of all other processes.

22. A young patient comes in with a butterfly rash on their face, progressive dyspnea, and there is ANA antibody present. What is their diagnosis?
CTD (connective tissue disease).

23. What type of ILD is commonly seen in coal miners, potters, and drill workers?

24. What is the main goal of ILD treatment?
Preventing the irreversible damage of the lung.

25. The term Interstitial Lung Disease (ILD) refers to a broad group of inflammatory lung disorders. More than 180 disease entities are characterized by what?
Acute, subacute, or chronic inflammatory infiltration of alveolar walls by cells, fluid, and connective tissue.

26. What are some other names for Interstitial Lung Diseases?
Diffuse interstitial lung disease, fibrotic interstitial lung disease, pulmonary fibrosis, or pneumoconiosis.

27. If left untreated, the inflammatory process can progress to what?
Irreversible pulmonary fibrosis.

28. What are the major pathologic or structural changes of the lungs associated with interstitial lung diseases?
Destruction of the alveoli and adjacent pulmonary capillaries, Fibrotic thickening of the respiratory bronchioles, alveolar ducts, and alveoli, Granulomas, Honeycombing and cavity formation, Fibrocalcific pleural plaques (with asbestos), Bronchospasm, and Excessive bronchial secretions.

29. How is the acute stage of ILD generally characterized?
Edema and the infiltration of a variety of white blood cells in the alveolar walls and interstitial spaces.

30. How is the chronic stage of ILD generally characterized?
In addition to the inflammation and infiltration of numerous white blood cells, some fibroblasts may be present in the alveolar walls and interstitial spaces.

31. What could follow the chronic stage of ILD?
Further interstitial thickening, fibrosis, granulomas, and in some cases honeycombing and cavity formation.

32. What are some examples of inorganic particulate (dust) exposures?
Asbestos, Coal dust, Silica, Beryllium, Aluminum, Barium, Clay, Iron, and Certain talcs.

S33. What causes Asbestosis?
Exposure to asbestos fibers, which are composed of magnesium, sodium, and iron.

34. Asbestosis may affect only one lung, a lobe, or a segment which is usually in the lower lobes. What is also common with Asbestosis?
Pleural calcification, and symptoms that may not appear until years later.

35. Coal Workers Pneumoconiosis (CWP) is caused by the pulmonary exposure to coal dust. What does it look like, and what are some other names?
Coal miners lungs, or black lung has the appearance of black spots (coal macules) on the lungs.

36. Silicosis is caused by what?
The exposure to silica, which is the main component of more than 95% if rocks on the earth.

37. Simple silicosis is characterized by small rounded nodules scattered throughout the lung, what happens during complicated silicosis?
The nodules coalesce and form large masses of fibrosis tissue. Worsening can lead to tissue necrosis and cavity formation.

38. What causes Berylliosis?
The exposure to beryllium, which is a steel-gray, lightweight metal found in certain plastics, ceramics, rocket fuels, and x-ray tubes.

39. The inhalation of beryllium causes what?
The development of granulomas and diffuse interstitial inflammation in more complex cases.

40. What are some examples of organic exposures that cause ILD?
Moldy hay, Silage, Moldy sugar cane, Mushroom compost, Barley, Cheese, Wood pulp, bark, dust, Cork dust, Bird droppings, and Paints.

41. What are the antigens that cause hypersensitivity pneumonitis?
Bacteria, Fungi, Amoeba, Animal protein, and Chemicals.

42. What are some occupational, environmental, and therapeutic exposures that can cause ILD?
Medications and illicit drugs such as Antibiotics, Anti-inflammatory agents, Cardiovascular agents, Chemotherapy agents, Illicit drugs, and Oxygen therapy, Radiation therapy, and Irritant gases such as Chlorine, Ammonia, and Nitrogen dioxide.

43. What are some examples of connective tissue diseases that lead to ILD?
Scleroderma, Rheumatoid arthritis, Sjogrens syndrome, Systemic lupus erythematosus, and Sarcoidosis.

44. What is scleroderma?
Chronic hardening and thickening of the skin caused by new collagen formation.

45. What is rheumatoid arthritis?
Inflammatory joint disease.

46. What is Sjogrens Syndrome, which is commonly associated with rheumatoid arthritis?
Lymphocytic disorder that destroys the salivary and exocrine glands, which causes dry mucus membranes and possible mucus plugging.

47. What is systemic lupus erythematous?
Autoimmune disease that mainly involves the joins and skin but can also affect the lungs, heart and kidneys.

48. What is sarcoidosis?
Chronic disorder of unknown origin, which involves abnormal collections of inflammatory cells that can form as nodules in multiple organs.

49. Which connective tissue disease is most common in African Americans age 10-40?

50. What is idiopathic pulmonary fibrosis (IPF)?
Progressive inflammatory disease of an unknown cause that causes varying degrees of fibrosis and honeycombing in severe cases.

51. IPF is most commonly seen in which patients?
Caucasian men age 40 to 70.

52. Idiopathic pulmonary fibrosis affects over 128,000 people in the US, how many die each year?

53. IPF is how many times more common than CF and ALS?
5 times more common.

54. What are the 2 types of Idiopathic Pulmonary Fibrosis?
(1) Desquamative Interstitial Pneumonia (DIP), and (2) Usual Interstitial Pneumonia (UIP).

55. Nonspecific Cryptogenic-Organizing Pneumonia (COP), also known as Bronchiolitis Obliterans Organizing Pneumonia (BOOP), is characterized as what?
Connective tissue plugs and mononuclear cell infiltration of the surrounding lung parenchyma. Although the cause is unknown, it has been linked to connective tissue disease, toxic gas inhalation, and infection.

56. What is Lymphocytic Interstitial Pneumonia (LIP)?
Diffuse pulmonary disorder characterized by fibrosis and accumulation of lymphocytes in the lungs, commonly associated with lymphoma.

57. What are the two most rare diseases that lead to ILD?
(1) Lymphangioleiomyomatosis (LAM), and (2) Pulmonary Langerhans cell histiocytosis (PLCH).

58. The pulmonary vasculitides consist of a group of pulmonary disorders characterized by inflammation and destruction of the pulmonary vessels. What are some of those disorders?
Wegner’s granulomatosis, Churg-Strauss syndrome, and Lymphomatoid granulomatosis.

59. Which two miscellaneous diffuse interstitial lung diseases involve recurrent pulmonary hemorrhage?
(1) Goodpasture’s syndrome, and (2) Idiopathic pulmonary hemosiderosis.

60. While all other diseases cause a decrease in DLCO, miscellaneous diffuse interstitial lung diseases cause what?
An increase in DLCO.

61. What would be found during the physical examination of a patient with ILD?
Increased heart rate and blood pressure, Increased respiratory rate, Cyanosis, Digital clubbing, Peripheral edema, and venous distention, and a Nonproductive cough.

62. What are some chest assessment findings that would be expected with ILD?
Increased tactile and vocal fremitus, Dull percussion note, Bronchial breath sounds, Crackles, Pleural friction rub, and Whispered pectoriloquy.

63. Mild to moderate ILD causes the ABG to look like what?
A decreased CO2 and an increased pH.

64. What would severe ILD cause the ABG to look like?
An increased CO2 with a normal pH, due to an increased HCO3.

65. What could be found on a chest radiograph associated with ILD?
Bilateral reticulonodular pattern, Irregularly shaped opacities, Granulomas, Cavity formation, Honeycombing, and Pleural Effusion.

66. Why is the management of ILD so difficult?
Because there is often no specific cause.

67. Which drugs are commonly administered with reasonably good results, but varying benefits from one patient to the next?
Corticosteroids, used to reduce inflammation.

68. What are the respiratory care treatment protocols for ILD?
Oxygen Therapy, Bronchopulmonary hygiene therapy, Mechanical ventilation, Pulmonary rehabilitation, Research trials, Lung transplantation, Palliative care, and Anti-fibrotic agents currently being studied for IPF only.

69. What is plasmapheresis?
Plasma exchange which directly removes the anti-GBM antibodies from the circulation

70. What is Asbestosis?
A restrictive lung disease caused by prolonged exposure to asbestos fibers. Associated with a high incidence of malignant lung tumors and pleural abnormalities.

71. What are Connective Tissue Diseases?
A group of acquired disorders that have in common diffuse immunologic and inflammatory changes in small blood vessels and connective tissue. The cause of most of these diseases is unknown.

72. What is Hypersensitivity Pneumonitis?
It is an Inflammatory form of interstitial pneumonia that results from an immunologic reaction in a hypersensitive person. The reaction may be provoked by various inhaled organic dust, often containing fungal spores. The disease can be prevented by avoiding contact with causative agents, also called extrinsic allergic alveolitis.

73. What is Idiopathic Pulmonary Fibrosis?
The formation of scar tissue in the connective tissue of the lungs without known cause resulting in severe chronic restrictive lung disease.

74. What is the definition of Interstitial Lung Disease?
A respiratory disorder characterized by a dry, unproductive cough and dyspnea on exertion. The chest radiograph usually shows fibrotic infiltrates in the lung tissue, usually in the lower lobe.

75. What is Lymphangioleiomyomatosis?
It is a lung abnormality most commonly observed in women and characterized by abnormal proliferation of smooth muscle cells in the interstitium, dyspnea, abnormal radiographic findings, and commonly pneumothorax.

76. What is occupational ILD?
Interstitial lung disease resulting from occupational exposure. Asbestosis is a common example.

77. What is Organizing Pneumonia?
It is the new term for bronchiolitis obliterans organizing pneumonia, that generally occurs in the setting of connective tissue disease.

78. What is Pulmonary Langerhans Cell Histiocytosis?
A condition characterized by abnormal proliferation of Langerhans cells, accompanied by interstitial markings on the chest film and dyspnea.

79. What is Silicosis?
A lung disorder caused by continued, long-term exposure to the dust of an inorganic compound, silicon dioxide, which is found in sands, quartzes and many other stones. Chronic silicosis is marked by widespread fibrotic nodular lesions in both lungs.

80. Which of the following are considered part of the lungs’ interstitium?
Alveolar walls and lumens; Cilia; and Fibroblasts. That is because the interstitium of the lung comprises the alveolar walls and lumens, pulmonary microvasculature, interstitial macrophages, fibroblasts, myofibroblasts, and matrix components of the lungs.

81. Which of the following is required in most cases in order to make a definitive diagnosis of interstitial lung disease (ILD)?
Lung biopsy; because to make a diagnosis of interstitial lung disease (ILD) physicians must consider the patient’s clinical presentation, radiographic findings, pulmonary function test results, laboratory values, and lung biopsy findings. In order to make a definitive diagnosis of which of the more than 200 interstitial lung diseases the patient has, a lung biopsy is usually required.

82. Which of the following are key diagnostic features of interstitial lung disease (ILD)?
Dyspnea at rest and/or with exertion; and Histopathologic features of inflammation and/or fibrosis of the pulmonary parenchyma. The key diagnostic features of interstitial lung disease (ILD) include dyspnea at rest and/or with exertion, bilateral diffuse interstitial infiltrates on the chest radiograph, physiologic abnormalities of a restrictive lung defect, and histopathologic features of inflammation or fibrosis of the pulmonary parenchyma.

83. Which of the following interstitial lung diseases has an unknown cause?

84. Which of the following is the most important tool in the identification of the etiology of interstitial lung disease (ILD)?
Medical history. Physicians perform many different tests to determine whether the patient has interstitial lung disease (ILD). However, the patient’s medical history is most valuable in determining the type and etiology of a specific patient’s ILD.

85. A physician is examining a patient whom she suspects has a form of interstitial lung disease (ILD). The patient is a smoker and presents with dyspnea, cough, fever, chills, and night sweats. The physical exam reveals digital clubbing. Which of the following diseases might the patient have?
Idiopathic pulmonary fibrosis. Patients with interstitial lung disease present with many different symptoms. The most likely diagnosis is idiopathic pulmonary fibrosis (IPF).

86. A physician is examining the chest radiograph of a patient she suspects has a form of interstitial lung disease (ILD). The radiograph shows eggshell calcification. Which of the following diseases might the patient have?
Silicosis. The appearance of eggshell calcification on a chest radiograph is almost unique to silicosis.

87. Which of the following are indications for surgical lung biopsy for patients in whom physicians suspect interstitial lung disease (ILD)?
Patient younger than 65 years; and Normal chest radiograph despite clinical signs. Indications for surgical lung biopsy include a patient younger than 65 years; a history of fevers, weight loss, and sweats; a history of hemoptysis; symptoms and signs of peripheral vasculitis; a history of pneumothorax; a normal chest radiograph; and rapidly progressing disease.

88. Which of the following tests can help physicians determine the needed biopsy sites for patients with suspected interstitial lung disease (ILD)?
High-resolution computed tomography. Physicians use high-resolution computed tomography (HCRT) to help them determine the best sites to biopsy in patients they suspect have interstitial lung disease (ILD).

89. Which of the following is the primary objective of therapy in patients with interstitial lung disease (ILD)?
Removing the agent of injury. The most important tenet of therapy for patients with interstitial lung disease (ILD) is to remove the agent of injury, if possible. If the agent cannot be found and removed, suppressing inflammatory and cellular immune responses is the next priority.

Final Thoughts

So there you have it! Thank you so much for making it all the way to the end of our study guide on Interstitial Lung Disease. I hope you found this information to be helpful.

Use this study guide effectively and you’ll ace your exams — there’s no doubt about it. Thanks again for reading and as always, breathe easy my friend.