Chapter 18- Interstital Lung Diseases Flashcards

1
Q

What are 3 common symptoms of ILD?

A

dyspnea on exertion
dry cough
constitutional symptoms sometimes

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2
Q

What does PFT show with ILD?

A

restriction and gas-exchange abnormalities

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3
Q

Lymphangioleiomyomatosis commonly presents in what people?

A

women of childbearing age

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4
Q

Langerhans cell histiocytosis commonly presents in what people?

A

young male smokers

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5
Q

What is the most common Idiopathic interstitial pneumonia?

A

cryptogenic fibrosing alveolitis (IPF)

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6
Q

What is IPF (cryptogenic fibrosing alveolitis)?

A

progressive fibrosis of the lungs resulting in nonproductive cough and SOB that worsens with exertion and eventually causes hypoxemic respiratory failure

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7
Q

What age is assoc. w/ IPF?

A

50-70y/o

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8
Q

What will be found on PE, PFT, and X-ray with IPF?

A

crackles

PFT- decreased lung volumes, poor oxygenation, infiltrates on Xray

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9
Q

What is the treatment for IPF?

A

Corticosteroids + azathioprine

Case by case decision

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10
Q

For acute exacerbation of IPF, what is the treatment?

A

corticosteroids

Poor prognosis

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11
Q

What interstitial pneumonia is assoc. with connective tissue disorders and hypersensitivity pneumonitis?

A

Nonspecific interstitial pneumonia (NSIP)

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12
Q

What are S/S of NSIP?

A

slowly progressive dyspnea and bilateral interstitial infiltrates?

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13
Q

What is treatment for NSIP?

A

immunosuppressants

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14
Q

What population is desquamative interstitial pneumonia (DIP) assoc. with?

A

young individuals that smoke

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15
Q

What are the S/S of DIP?

A

progressive SOB and bilateral infiltrates on chest radiograph

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16
Q

How do you diagnose DIP?

A

biopsy (shows macrophages in alveolar spaces)

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17
Q

How do you treat DIP/

A

avoid tobacco

immunosuppressants

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18
Q

What is acute interstitial pneumonitis (AIP)?

A

dyspnea that progresses from days to weeks and leads to respiratory failure

usually fatal

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19
Q

What are s/s of Cryptogenic organizing pneumonia (COP)?

A

subacute or chronic dyspnea first noted on exertion and sometimes triggered after acute illness, like URI

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20
Q

Treatment of COP

A

immunosuppressants

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21
Q

Who is lymphocytic interstitial pneumonia (LIP) common in?

A

women

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22
Q

What are s/s of lymphocytic interstitial pneumonia (LIP)?

A

gradual onset dyspnea and cough

occasionally fever, CP, arthralgias, weight loss

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23
Q

How to you treat lymphocytic interstitial pneumonia (LIP)?

A

corticosteroids

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24
Q

What is the most common granulomatous disorder?

A

sarcoidosis

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25
Q

What are the associated risks of sarcoidosis?

A

Northern European
10-40 y/o
Women > men

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26
Q

Characterized by formation in tissues of noncaseating granulomas that organize in an inner core of epithelioid cells, macrophages, and giant cells, surround by a rim of lymphocytes, fibroblasts, and connective tissue

A

sarcoidosis

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27
Q

What organs are affected by sarcoidosis?

A

virtually any organ,

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28
Q

What are two problems assoc. with acute sarcoidosis?

A

Lofren syndrome

Uveoparotid fever

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29
Q

Lofren syndrome

A

erythema nodosum, arthritis, hilar adenopathy

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30
Q

Uveoparotid fever

A

uveitis, parotitis, facial nerve palsy

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31
Q

How is sarcoidosis treated?

A

corticosteroids

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32
Q

What are the usually s/s and PFTs for interstitial lung diseases related to connective tissue disorders?

A

extertional dyspnea and dry cough or asymptomatic
Bibasilar crackles
PFTs: restrictive pattern with decreased diffusion capacity

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33
Q

What is the treatment for interstitial lung diseases related to connective tissue disorders?

A

immunosuppressants

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34
Q

2 causes of drug induced lupus

A

procainamide or hydralazine

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35
Q

Drug that causes dyspnea, hemoptysis, pulmonary infiltrates

A

crack cocaine (“crack lung”)

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36
Q

Characterized by disruption of alveolar capillary membranes resulting in bleeding into alveolar spaces

A

Pulmonary vasculitis and diffuse alveolar hemorrhage

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37
Q

S/S: Pulmonary vasculitis and diffuse alveolar hemorrhage

A

abrupt onset of cough, fever, dyspnea, hemoptysis

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38
Q

What are 3 abnormalities associated with systemic vasculitis?

A

ocular, nasopharyngeal, cutaneous

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39
Q

What do you hear on auscultation with Pulmonary vasculitis and diffuse alveolar hemorrhage

A

inspiratory crackles, systolic murmur

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40
Q

What are 3 histologic patterns of Pulmonary vasculitis and diffuse alveolar hemorrhage

A

bland pulmonary hemorrhage
diffuse alveolar damage
pulmonary capillaritis

41
Q

Define pneumoconiosis

A

accumulation of mineral dusts in the lungs usually leading to fibrosis

42
Q

What professions can lead to silicosis?

A

mining, stone cutting, carving, polishing, sandblasting

43
Q

What are s/s of silicosis?

A

dyspnea or asymptomatic

44
Q

What can chronic silicosis result in besides fibrosis?

A

simple nodular silicosis

45
Q

Formation of pigmented lesions surrounded by emphysema (coal macules)

A

coal worker’s pneumoconiosis

46
Q

What s/s are associated with coal worker’s pneumoconiosis?

A

chronic productive cough

diffuse small rounded opacities on Xray

47
Q

Where can you be exposed to abestos?

A

fibrous silicate used for insulation,, friction bearing surfaces or to strengthen materials

48
Q

Characterized by pleural plaques, effusion, fibrosis, gradual onset of dyspnea

A

Asbestosis

49
Q

Leads to acute chemical bronchitis and pneumonitis

A

berylliosis

50
Q

How to treat berylliosis?

A

corticosteroids

51
Q

What pneumoconiosis is associated with RA?

A

coal worker’s pneumoconiosis

52
Q

What pneumoconiosis has elevated risk of TB?

A

silicosis

53
Q

What pneumoconiosis has increased incidence of malignancy?

A

asbestosis

54
Q

Fever, chills, cough, dyspnea, malaise that occurs several hours after inhaling organic antigens. treated with corticosteroids

A

acute hypersensitivity pneumonitis

55
Q

What are the s/s of subacute or chronic hypersensitivity pneumonitis?

A

chronic dyspnea and cough

56
Q

What can be heard and seen on CT for hypersensitivity pneumonitis

A

Heard: diffuse crackles and wheezes

CT: ground glass opacities, centrilobular nodules, mosaic attenuation patterns

57
Q

“eosinophilic granuloma” seen in young to middle aged adults that are mainly white male smokers. Associated with dyspnea on exertion, cough, possible hemoptysis

A

Pulmonary Langerhans Cell Hisitocytosis

58
Q

What does imaging and PFT show for Pulmonary Langerhans Cell Hisitocytosis?

A

PFT: obstructive pattern, impaired diffusion capacity
Imaging: micronodular lesions, cysts in mid and upper lung zones

59
Q

Treatment for Pulmonary Langerhans Cell Hisitocytosis

A

stop smoking

corticosteroids

60
Q

Abnormal proliferation of smooth muscle cells

A

Lymphangioleiommyomatosis

61
Q

What are S/S of Lymphangioleiommyomatosis?

A

dyspnea, PNEUMOTHORAX, possible chylous pleural effusions and hemoptysis

62
Q

How to treat Lymphangioleiommyomatosis?

A

manage pleural complications, bronchodilator, O2 therapy, avoid estrogen

63
Q

Presence of pulmonary infiltrates and eosinophilia of peripheral blood or lung

A

eosinophilic lung disease

64
Q

Acute eosinophilic lung disease

A

Fever, nonproductive cough, dyspnea <7 days leading to respiratory failure

Healthy Men 20-40 y/o

65
Q

What do diagnostics show for Acute eosinophilic lung disease?

A

imaging: bilateral infiltrates

abundant eosinophils in lavage fluid

66
Q

Treatment of Acute eosinophilic lung disease

A

corticosteroids

67
Q

Productive cough, dyspnea, malaise, weight loss, NIGHT SWEATS, fever. Predominantly middle aged women. Treated with corticosteroids

A

Chronic eosinophilic lung disease

68
Q

Simple pulmonary eosinophilia (Loffler syndrome)

A

Transient migratory infiltrates that last <1 month
Causes: Idiopathic, drugs, PARASITES
Treatment: remove cause, treat parasites, corticosteroids if idiopathic

69
Q

When aspergillosis colonizes the airways in patients with asthma or CF

A

Allergic bronchopulmonary aspergillosis

70
Q

Allergic bronchopulmonary aspergillosis S/S

A

fever, malaise, cough productive of THICK BROWN MUCUS PLUGS, some hemoptysis

71
Q

Diagnostics for Allergic bronchopulmonary aspergillosis

A

Xray: pulmonary infiltrates, central bronchiectasis

Antibodies to aspergillus present

72
Q

Treatment of Allergic bronchopulmonary aspergillosis

A

corticosteroids, itraconazole

73
Q

Lipoproteinaceous material accumulates in alveoli. Caused by congenital or disorders with decreased macrophages. Can also be idiopathic.

A

Pulmonary Alveolar proteinosis

74
Q

S/S of Pulmonary Alveolar proteinosis

A

progressive dyspnea on exertion, malaise, low-grade fever, cough

75
Q

Pulmonary Alveolar proteinosis: findings of chest X-ray, CT, lavage

A

XRAY: bilateral perihilar opacities
CT: thickening of interlobular and interlobular septa
LAVAGE: milky, opaque fluid, looks foamy

76
Q

Treatment of Pulmonary Alveolar proteinosis if hypoxia or severe dyspnea

A

whole lung lavage

77
Q

What are the clinical manifestations of the pulmonary system in sarcoidosis?

A

Dyspnea, cough, wheezing, hemoptysis, endobronchial lesions

78
Q

What are the clinical manifestations of the dermatological system in sarcoidosis?

A

Erythema nodosum, papules, plaques

79
Q

What are the clinical manifestations of the otolaryngologic system in sarcoidosis?

A

Saddle nose deformity, sinusitis, laryngeal lesions, parotitis

80
Q

What are the clinical manifestations of the ocular system in sarcoidosis?

A

Uveitis, choriorectinitis, lacrimal gland enlargement, chronic tearing, optic neuritis

81
Q

What are the clinical manifestations of the Neurologic system in sarcoidosis?

A

Cranial nerve palsy, headache, diabetes insipidus, mass lesions, seizures, meningitis, encephalitis

82
Q

What are the clinical manifestations of the rheumatologic system in sarcoidosis?

A

Arthralgias, arthropathy, myopathy

83
Q

What are the clinical manifestations of the gastrointestinal system in sarcoidosis?

A

Elevated transaminases, abdominal pain, jaundice

84
Q

What are the clinical manifestations of the cardiologic system in sarcoidosis?

A

Arrhythmias, conduction abnormalities, sudden cardiac death, pulmonary hypertension, congestive heart failure

85
Q

What are the clinical manifestations of the hematologic system in sarcoidosis?

A

Lymphadenopathy (especially hilar), hypersplenism

86
Q

What are the clinical manifestations of the Endocrine system in sarcoidosis?

A

Hypercalcemia, hypercalciuria, epididymitis

87
Q

What are the clinical manifestations of the renal system in sarcoidosis?

A

Renal calculi, interstitial nephritis, renal failure

88
Q

What are the syndromes associated with sarcoidosis?

A
Lofgren syndrome
Heerfordt syndrome (uveoparotid fever)
89
Q

What does lofgren syndrome consist of?

A

Fever, arthralgias, bilateral hilar adenopathy, erythema nodosum

90
Q

What does Heerfordt syndrome consist of?

A

Fever, swelling of parotid gland and uveal tracts, cranial nerve VII palsy

91
Q

What connective tissue disorders are involved in pleural effusion?

A

RA (5-40%)

Lupus (30-40%)

92
Q

What connective tissue disorders are involved in necrobiotic nodules?

A

RA

93
Q

What connective tissue disorders are involved in fibrosis?

A
RA (20--60%)
Lupus (3%)
Systemic Sclerosis (15-90%)
Polymyotisis/dermatomyotisis (10-40%)
Sjogren syndrome (33%)
94
Q

What connective tissue disorders are involved in bronchiolitis?

A

RA
Lupus
Sjogren syndrom

95
Q

What connective tissue disorders are involved in pulmonary arthropathy?

A

RA
Lupus
Systemic sclerosis
Polymyotisis/dermatomyotisis

96
Q

What connective tissue disorders are involved in pneumonitis hemorrhage?

A

Lupus

97
Q

What connective tissue disorders are involved in diaphragm dysfunction?

A

Lupus

98
Q

What connective tissue disorders are involved in aspiration?

A

Systemic sclerosis

Polymyotisis/dermatomyotisis (14%)

99
Q

What connective tissue disorders are involved in secondary carcinoma?

A

Systemic sclerosis