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ILD Flashcards

1
Q

What is ILD?

A

Interstitial Lung Disease

Group of lung diseases affecting the ‘interstitium’

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2
Q

What is the lung interstitium?

A

Tissue and space around the air sacs of the lungs

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3
Q

Where is the lung interstitium?

A
  1. Alveolar epithelium
  2. Pulmonary capillary endothelium
  3. Basement Membrane
  4. Perivascular and perilymphatic tissues
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4
Q

Common effect of ILDs?

A 
Decreased lung volumes
Decreased compliance (stiff lungs)
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5
Q

How are ILDs classified?

A
  1. Idiopathic Interstitial Pneumonias (aka unknown cause; about 50% of the time)
  2. Identifiable cause
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6
Q

Common Causes of ILD

A
  1. Inhaled Substances
  2. Connective Tissue Diseases
  3. Radiation
  4. Medications
  5. Infection
  6. Others Diseases
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7
Q

What types of inhaled substances can cause ILD?

A

Mineral Dusts (silica, asbestos)
Antigens (Mold, bacterial)
Gaseous material

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8
Q

What types of Connective Tissue Diseases can cause ILD?

A

Lupus
Rheumatoid Arthritis
Scleroderma

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9
Q

What types of Medications can cause ILD?

A 
Bleomycin
Amiodarone
Methotrexate
Gold
Nitrofurantoin
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10
Q

What types of infections can cause ILD?

A

Recurrent bactrial pneumonia
TB
Viral Infection

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11
Q

What types of other diseases can cause ILD?

A

Sarcoidosis
Alveolar Proteinosis
Histiocytosis

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12
Q

What is IPF?

A

Idiopathic Pulmonary Fibrosis

Defined as a specific form of chronic progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with histopathologic and/or radiographic pattern of usual interstitial pneumonia (UIP)

***Requires the exclusion of other forms of Interstitial Pneumonia

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13
Q

How common is IPF?

A 
25-35% of cases
More common in old people >60 years old
50% die within 5 years
Incidence: 7-16/100k
Prevalence: 14-43/100k
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14
Q

How do you diagnose IPF?

A
  1. Exclusion of other known diseases of ILD
  2. Presence of UIP pattern on HRCT in patients subjected to surgical lung biopsy
  3. Classic signs and symptoms
  4. Lung function abnormalities (PFTs)
  5. CXR and CT Scan
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15
Q

What is a chronic progressive form of IPF?

A

Extensive deposition of collagen leading to widespread fibrosis

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16
Q

What is acute exacerbation in IPF?

A

Acute (Within 30 days) worsening of symptoms with worsening radiological findings

  • Dx of IPF
  • Unexplained worsening of development of dyspnea within 30 days
  • New bilateral ground-glass abnormality and/or consolidation super imposed on a background reticular or honeycomb!!!
  • No evidence of Pulm infection
  • Exclusion of alternative causes
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17
Q

Signs and Symptoms of IPF

A
  1. Dyspnea (at rest or with exertion)
  2. Cough: usually dry
  3. Decreased tolerance for activity
  4. Fatigue, fever, weight loss
  5. Clubbing
  6. Cyanosis
  7. Tachypnea
  8. Inspiratory dry fine crackles at lung bases
  9. Signs of right ventricular failure (edema, increased JVD)
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18
Q

What is the effect on Lung Function in IPF?

A
  1. Impaired Oxygenation due to scarring and damage
    - Early: increased A-a gradient
    - Late: Hypoxemia
  2. Restriction of lung volumes, decreased lung compliance
  3. Decreased diffusion capacity
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19
Q

PFTs of IPF patients will show

A
  1. Restrictive Impairment
  2. Decreased DLCO
  3. Hypoxemia worse on exercise
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20
Q

How will a CXR or CT show up in IPF?

A
  1. Diffuse Interstitial Infiltrate
  2. Peripheral, SUBPLEURAL, bibasilar infiltrate
  3. Can be patchy
  4. HONEY COMB Changes in later stage
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21
Q

Classic Usual Interstitial Pneumonia (UIP) Pattern

A
  1. Extensive Honeycombing
  2. Basal Predominance
  3. Peripheral Predominant Reticular abnormality with multiple layers of honeycombing
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22
Q

A patient that fills these requirements would be at a _______ prognosis with IPF:

  • Younger age
  • Female gender
  • Milder dyspnea (less functional impairment)
  • Response to therapy
  • Cigarette smoker at time of diagnosis
A

Better Prognosis

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23
Q

A patient that fills these requirements would be at a _______ prognosis with IPF:

  • Older Age
  • Presence of Pulmonary Hypertension
  • Advanced degree of Fibrosis on HRCT
  • Decreased Diffusing Capacity
  • Hypoxemia requiring O2 Supplementation
A

Worse Prognosis

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24
Q

How do you treat a patient with IPF?

A
  1. Antiinflammatory drugs
  2. O2 supplementation
  3. Pulmonary rehab
  4. Treatment of Asymptomatic GERD
  5. Lung tx for younger patients with advanced IPF
  6. Newer Pharmacotherapeutic Modalities so far are unsuccessful
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25
Q

Inflammation of the lungs due to breathing in a foreign substance, usually certain types of dust like fungus, or molds leading to acute lung disease, that may turn into long-lasting (chronic) lung disease.

A

Hypersensitivity Pneumonitis

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26
Q

What can cause Hypersensitivity Pneumonitis?

A
  1. Repeated or intense exposure to dust from moldy, hay, straw, and grain
  2. Fungus present in humidifiers, heating systems, and air conditioners found in homes and offices
  3. Birds
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27
Q

________ has a protective effect on Hypersensitivity Pneumonitis.

A

Smoking

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28
Q

Signs and Symptoms in the Acute Stage of Hypersensitivity Pneumonitis

A
  1. Cough
  2. Fever, Chills
  3. SOB
  4. Malaise
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29
Q

Signs and Symptoms in the Subacute Stage of Hypersensitivity Pneumonitis

A

Wheeze with SOB

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30
Q

Signs and Symptoms in the Chronic Stage of Hypersensitivity Pneumonitis

A
  1. SOB
  2. Cough, often dry
  3. Loss of appetite –> Weight loss
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31
Q

Diagnostic Tests for Hypersensitivity

A
  1. CXR
  2. PFTs
  3. Anitbody Panels
  4. High resolution CT Chest/Expiratory
  5. Bronchoscopy (lung lavage; transbronchial biopsy)
  6. Video-assisted or open lung biopsy
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32
Q

Lung disease after you get chest radiation, commonly occurring 4-6 weeks

A

Radiation Induced Lung Dz

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33
Q

Treatment of Radiation Induced Lung Dz

A

Steroids

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34
Q

What is the treatment for Hypersensitivity Pneumonitis?

A
  1. Identify the causative agent and avoid it

2. Corticosteroids are effective in the acute and subacute form of the dz

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35
Q

What is the prognosis of Hypersensitivity Pneumonitis?

A
  • Most symptoms go away by avoiding or limiting exposure

- Chronic form of this disease may lead to pulmonary fibrosis

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36
Q

A disease of unknown cause characterized by abnormal inflammatory masses (granulomas) in certain organs of the body.

A

Sarcoidosis

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37
Q

This can affect almost any organ in the body, although it most commonly affects the lungs and lymph nodes

A

Sarcoidosis

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38
Q

Causes of Sarcoidosis

A
  1. Hypersensitivity to environmental factors
  2. Genetics
  3. Extreme immune response to infection
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39
Q

Incidence of Sarcoidosis

A
  1. More common in AA than Caucasians
  2. Females&raquo_space; Males
  3. Starts 20s-40s
  4. Very rare in children
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40
Q

Non-caseating granuloma is a

A

Fundamental lesion of sarcoidosis

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41
Q

Signs of Sarcoidosis

A

Commonly found in asymptomatic patients with an abormal CXR

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42
Q

Symptoms of Sarcoidosis

A
  1. Fever, shortness of breath cough
  2. Visual symptoms
  3. Neurological symptoms
  4. Enlarged lymph nodes
  5. Hepatomegaly/ splenomegaly
  6. Erythema nodosum
  7. Hypercalcemia, Kidney stones
  8. Dry mouth
  9. Weight loss
43
Q

First rule in trying to Diagnose Sarcoidosis

A

Rule out TB and fungal infection!!

44
Q

Diagnostic Testing for Sarcoidosis after ruling out TB or a fungal infection

A
  1. CBC
  2. ACE levels, Calcium
  3. CXR
  4. CT Scan
  5. Biopsy (lymph node from skin or lung)
45
Q

What would you be looking for on a CXR or CT Chest if suspecting Sarcoidosis?

A

Hilar or Mediastinal Lymphadenopathy
Interstital Infiltrates
Varying degree of lung fibrosis

46
Q

Acute presentation of Sarcoidosis is called

A

Lofgren’s Syndrome

47
Q

What is presented in Lofgren’s Syndrome?

A
  1. Erythema Nodosum
  2. Arthritis (mainly ankles)
  3. Hilar Adenopathy
  4. Uveitis
48
Q

Rare presentation of Sarcoidosis

A

Heerfordt’s Syndrome

49
Q

What is presented in Heerfordt’s Syndrome

A
  1. Parotitis (Inflammation of the Parotid Gland)
  2. Fever
  3. Facial Nerve Palsy
50
Q

How do you treat Sarcoidosis?

A
  1. Spontaneous remission is common even without treatment
  2. Corticosteroids is indicated for severely affected patients
  3. Therapy may continue for one or two years
  4. Some of the most severely affected patients may require life-long therapy
  5. Immunosuppressive agents: Methotrexate, Azathioprine, and Cyclophosphamide, are sometimes used in addition to corticosteroids
  6. Irreversible organ failure require an lung transplant
51
Q

50% of the sarcoidosis pts:

A. Die within three years of assumed onset
B. Receive lung damage within three years after assumed onset
C. Resolve without treatment in three years
D. Will develop lung damage if lung involvement in the patients

A

C. Resolve without treatment in three years

52
Q

20% of the sarcoidosis pts:

A. Die within three years of assumed onset
B. Receive lung damage within three years after assumed onset
C. Resolve without treatment in three years
D. Will develop lung damage if lung involvement in the patients

A

D. Will develop lung damage if lung involvement in the patients

53
Q

T/F: It is rare for patients with sarcoidosis to die.

54
Q

Some complications that can arise with sarcoidosis includes:

A
  1. Diffuse ILD and hypoxemia
  2. Pulmonary HTN
  3. Cardiac involvement (arrhythmia and heart failure)
  4. Eye disease (anterior uveitis)
  5. CNS Involvement
  6. Kidney Stones and Renal Failure
55
Q

Cryptogenic Organizing Pneumonia (COP) is sometimes referred to as

A

Bronchiolitis Obliterans

56
Q

How does COP present in clinic?

A
  1. Mild flu-like symptoms with fever, cough, and malaise
  2. Progressively mild dyspnea ensues
  3. Anorexia and weight loss is common
  4. Hemoptysis (uncommon and rarely severe)
  5. CP, night sweats and mild arthalgia are also uncommon
  6. Physical Examination usually discloses focal sparse crackles
57
Q

What would you see in a CXR/CT of TYPICAL COP?

A
  1. Multiple alveolar opacities
  2. Usually bilateral and peripheral
  3. Often migratory
  4. Size varies from a few centimeters to a whole lobe
  5. An air bronchogram is often present in consolidated opacities
  6. On HRCT the density of opacities ranges from ground glass to consolidation
58
Q

What would you see in a CXR/CT of FOCAL COP?

A
  1. Solitary opacity
  2. The lesions are often located in the upper lobes, and may be cavitary.
  3. May be totally asymptomatic and discovered by routine chest radiographs
  4. False-positive PET Scan
  5. Spontaneous regression has been reported
  6. Usually does not relapse after surgical excision
59
Q

What would you see in a CXR/CT of INFILTRATIVE COP?

A
  1. Interstitial and superimposed small alveolar opacities
  2. Infiltrative pattern may consist of a poorly defined arcade-like or polygonal appearance defining a perilobular pattern
  3. Often associated with other opacities, especially consolidation.
60
Q

What would PFTs show in a patient with COP?

A
  1. Mild or moderate restrictive ventilatory defect is the most common abnormality
  2. DLCO is reduced in proportion to restriction
  3. Hypoxemia at rest and/or during exercise is usually mild
61
Q

After performing a transbronchial biopsy with a patient with suspected COP, what would you see on pathology?

A

Presence of buds of granulation tissue consisting of fibroblasts-myofibroblasts embedded in connective tissues is the hallmark!!!!

“Butterfly Pattern” - Extension from one alveolus to another

Budding that extends into the bronchioles may obstruct the lumen

62
Q

Treatment of COP

A

Steroids/Methylprednisolone

Initial Dose: 0.75-1.5 mg/kg/day (with further boluses for the first few days)

Then you taper to lower doses, and the duration is 6 months to 1 year.

63
Q

Corticosteroid treatment results in ______ clinical improvement and clearing of the opacities on chest imaging without significant sequelae in COP patients

64
Q

Despite the good, fast treatment of COP by Corticosteroids, what is a common occurrence?

A

Relapses are common upon stopping or reducing corticosteroids

65
Q

What are common Occupational Lung DILD

A

Coal Worker Pneumoconiosis
Asbestosis
SIlicosis
Berylliosis

66
Q

What can cause Coal Worker’s Pneumoconiosis (CWP)?

A

Breating in dust from coal, graphite, or man-made carbon for long periods of time.

Why? Lung inflammation and scarring can occur.

67
Q

What two forms can manifest in CWP?

A
  1. Simple (1-2mm nodules)

2. Complicated/Progressive Massive Fibrosis) (>1 cm masses on fibrotic areas with cavities)

68
Q

CWP is most common in people over the age of?

A

50 years old

69
Q

True/False:

Smoking increases your risk of developing CWP.

A

FALSE

But it can add more harmful damage to the lungs.

70
Q

Symptoms of CWP

A
  1. SOB
  2. Chronic Cough
  3. Crackles
71
Q

Diagnostic Testing for CWP

A
  1. CXR

2. HRCT Chest

72
Q

If a Simple CWP is presented on a CXR/CT, what would you see?

A

Small rounded opacities

73
Q

If a Complicated CWP is presented on a CXR/CT, what would you see?

A

Large Conglomerate Masses

74
Q

Treatment of CWP

A
  1. No specific treatment for this disorder
  2. Avoid future exposure to the dust
  3. Supportive care (O2 therapy)
75
Q

What is the prognosis for CWP

A

Simple: Usually good
Complicated: May lead to progressive pulmonary fibrosis with restrictive ventilatory impairment.

76
Q

Complications of CWP

A
  1. Cor Pulmonale

2. Pulmonary TB

77
Q

Chronic inflammatory condition affecting the lung parenchyma after long-term, heavy inhalational exposure to asbestos.

A

Asbestosis

78
Q

What is asbestos?

A

Naturally occurring fibrous silicates

79
Q

What does the severity of asbestosis depend on?

A

Duration of exposure to asbestos and the amount inhaled

80
Q

True/False:

Smoking increases your risk of developing Asbestos.

81
Q

Signs and Symptoms of Asbestosis

A
  1. SOB
  2. Cough
  3. Chest Tightness/Pain
  4. Clubbing
  5. Lung crackles
82
Q

Diagnostic Tests for Asbestosis

A
  1. CXR (will show small lungs with hazy infiltrates. Lower lung zones are more affected.)
  2. CT Chest
  3. PFTs (will show restrictive lung disease)
83
Q

Treatment of Asbestosis

A
  1. No effective medical treatment
  2. Stopping further exposure to asbestos is essential
  3. Postural Drainage, chest percussion, and vibration can help remove secretions from the lungs.
  4. O2 Therapy
  5. Lung Transplantation
84
Q

Diffuse fibrotic reaction of the lungs to inhalation of free crystalline silica (sand, quartz)

85
Q

Intense exposure to silica can cause this disease to occur as soon as ____ year(s), but most commonly takes _______ year(s).

86
Q

What are the three types of Silicosis?

A
  1. Simple Chronic Silicosis
  2. Accelerated Silicosis
  3. Acute Silicosis
87
Q

Results from long-term exposure (more than 20 years) to low amounts of silica dust

A

Simple Chronic Silicosis

88
Q

Occurs after exposure to larger amounts of silica over a shorter period of time (5 - 15 years).

A

Accelerated Silicosis

89
Q

Results from short-term exposure to very large amounts of silica .

A

Acute Silicosis

90
Q

Symptoms of Silicosis

A
  1. Chronic Cough
  2. SOB with exertion (commonly in people with progressive massive fibrosis)
  3. Fever, Cough, Weight Loss, Severe Breathing Difficulty
91
Q

Diagnostic Tests for Silicosis

A
  1. CXR
  2. PFTs
  3. PPD Skin Test
92
Q

True or False:

People with Silicosis are at high risk for developing TB

93
Q

How do you treat Silicosis?

A
  1. No specific treatment for it
  2. Removing the source of silica exposure
  3. Supportive treatment includes cough medicine, bronchodilators, and O2 if needed
  4. Antibiotics for assc resp. infection
  5. Limiting exposure to irritants, quit smoking, and having routine TB skin tests
94
Q

Cause of Drug-Induced Parenchymal Lung Disease

A

Could be any drug, but commonly:

  1. Methotrexate
  2. Nitrofurantoin
  3. Bleomycin: Avoid O2
  4. Amiodarone
  5. Gold
95
Q

What are some common extrinsic causing restrictive lung dz?

A
  1. Obesity
  2. Diaphragmatic Paralysis
  3. Chest Wall Abnormality
96
Q

What is Obesity Hypoventilation Syndrome?

A

Obesity that causes decrease in expiratory reserve volume, decreased ventilation of basilar portions of lungs, and hypoxemia.

97
Q

How do you diagnose this Obesity Hypoventilation Syndrome?

A
  1. BMI >30

2. Hypercapnea

98
Q

How do you treat Obesity Hypoventilation Syndrome?

A
  1. Weight Loss

2. Check TSH

99
Q

What is Obesity Hypoventilation Syndrome often confused with?

A

Ondine’s Curse

100
Q

What is diaphragmatic paralysis?

A

Unilateral diaphragmatic paralysis in absence of other diseases

101
Q

Symptoms of diaphragmatic paralysis

A
  1. Asymptomatic

2. Encroachment of abdominal contents on thoracic cavity causes symptoms to worsen when a patient is supine.

102
Q

Performing a test to evaluate diaphragmatic paralysis would show

A

Most patients retain normal vital capacity, despite functional loss of hemidiaphragm.

103
Q

Any disease that restricts motion of chest wall, distorts its symmetry, or interferes with neuromuscular function that can lead to hypoventilation

A

Chest Wall Disease

104
Q

What can Chest Wall Disease Cause?

A

Total lung and vital capacities are decreased
Residual volume is normal

Hypoventilation produces hypercapnia and progressive atelectasis

Pulmonary Medicine (14 decks)

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