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CRRAB II Week 7 - WLB > Pulmonary Pathology Part 3 - Witrak > Flashcards

Pulmonary Pathology Part 3 - Witrak Flashcards

1
Q

What is Idiopathic Pulmonary Fibrosis?

A

most lethal common ILD with 70-80% mortality at 5 years post Dx = slowly progressive pulmonary fibrosis with gradually worsening dyspnea and associated non-productive cough.
- age typically >40 years, most commonly
males, and majority (70%) are smokers or
former smokers.
(Witrak says it is worse than cancer)

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2
Q

What do the PFT’s show in Idiopathic Pulmonary Fibrosis?

A

restrictive pattern with decreased TLV

and decreased FVC.

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3
Q

What does the CXR show in Idiopathic Pulmonary Fibrosis?

A

diffuse interstitial opacities.

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4
Q

What does the high resoluation CT show in Idiopathic Pulmonary Fibrosis?

A

characteristic peripheral/subpleural and bibasilar reticulonodular opacities and architectural/cystic distortion – may be sufficient for Dx (50%).

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5
Q

What does the histology show in Idiopathic Pulmonary Fibrosis?

A

usual interstitial pneumonia (UIP): patchy interstitial fibrosis alternating with normal/near-normal lung parenchyma: both old (fibrotic) and young (fibroblastic) areas of scarring – with honeycomb change.
NOTE: inflammation not a significant feature.

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6
Q

What is the theory of cause of Idiopathic Pulmonary Fibrosis?

A

abnormal alveolar healing response (fibrogenesis) to miscellaneous toxic/injurious stimuli (exogenous/endogenous) – TGF beta-related?; association with GERD

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7
Q

What is the clinical course of Idiopathic Pulmonary Fibrosis?

A

Usually relentlessly progressive with periodic acute exacerbations: can have DAD-like flare.
- typically death with hypoxemia, cyanosis,
digital clubbing - associated with
pulmonary hypertension/cor pulmonale.
- minority of patients develop bronchogenic
carcinoma: typically adenocarcinoma.
- heart failure/ischemic heart disease causes
death in 1/3 of patients.
- may co-exist with emphysema

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8
Q

What is the treatment for Idiopathic Pulmonary Fibrosis?

A

oxygen supplementation, newer medications inhibiting fibrogenesis, and lung transplant

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9
Q

What is the most common causes of Classic Inorganic Pneumoconioses?

A

Silicosis: due to inhalation of silica (silicon
dioxide) = most abundant mineral on earth.
- typically chronic lung injury from usually
long-standing (10-30 years) inhalational
exposure: esp. mining/sandblasting
workers.
- progressive respiratory insufficiency with
CXR showing multiple small (

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10
Q

Occupational/environmental ILD can develop from what types of inhaled organic/antigenic dusts?

A

hypersensitivity pneumonitis due to:
- thermophilic actinomycetes: Farmer’s
lung, grain handler’s lung, humidifier/air
conditioner lung
- bacteria (humidifier lung)
- true fungi (aspergillus)
- animal proteins (e.g. bird fancier’s lung)

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11
Q

What are the histological findings in Silicosis?

A

can see cavitary necrosis and hilar adenopathy with calcification.

  • increased risk of tuberculosis.
  • acute silicosis: following a massive inhalational exposure.
  • histology shows fibrocellular parenchymal nodules; bronchoalveolar lavage – silica in macrophages as per energy-dispersive x-ray analysis; acute silicosis can resemble alveolar proteinosis.
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12
Q

How is Silicosis diagnosed?

A

usually a clinical one = history of exposure sufficient to cause degree of illness present, compatible chest imaging, and absence of another diagnosis to explain pulmonary findings

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13
Q

What is Asbestos-related lung disease due to?

A
  • due to inhalation of naturally-occurring mineral fibers (hydrated magnesium silicates).
    • USA: typically due to exposure to asbestos insulation products.
    • two main asbestos fiber types: serpentine (90%:less toxic) and amphibole (more toxic).
    • direct toxic effects to: pulmonary
      parenchyma and pleura.
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14
Q

What is Asbestosis?

A

pulmonary interstitial lung disease beginning in lower lobes with asbestos bodies
= subtype of ferruginous body.
Benign pleural disease: parietal pleural calcified plaques (50%), pleural effusions, diffuse visceral pleural fibrosis.
Malignant pleural disease – mesothelioma: may be delayed several decades post exposure.
Lung cancer risk: increased (without smoking) and very increased (with smoking).

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15
Q

What are the key CXR findings in Asbestosis?

A

calcified pleural plaques (over dome of diaphragm or pleural lining over the rib cage)

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16
Q

How is Asbestosis diagnosed?

A

If exposure history fits symptoms and chest
imaging studies: especially calcified pleural
plaques – lung biopsy not needed.

If Bx needed for confirmation: look for
asbestos bodies and confirm with scanning
electron microscopy/x-ray analysis.

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17
Q

What is Coal worker’s pneumoconiosis?

A
  • blackening of lungs = anthracosis: due to
    coal dust pigment in lymphatics - formation of
    small nodules/macules: esp. upper lung fields.
  • 90% of cases relatively benign: little to no
    effect on pulmonary function.
  • 10% affected with progressive massive
    fibrosis: likely secondary to co-existent silica
    inhalation during mining process.
  • some association with chronic
    bronchitis/emphysema.
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18
Q

What is hypersensitivity pneumonitis?

A

Immunologically mediated lung disease with mixed interstitial/alveolar features.
- due to antibody reaction to inhaled
antigen (usually organic).
- over 300 etiologies reported.
- if offending agent not removed from the
patient’s environment: can have eventual
severe pulmonary fibrosis.

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19
Q

What are the clinical features of hypersensitivity pneumonitis?

A

*Acute attacks 4-6 hours post exposure with
fever, dyspnea, cough, leukocytosis.
HRCT: characteristic mid/upper zone ground glass or nodular opacities.
PFT: can be restrictive, obstructive, or mixed patterns.

20
Q

What are some common etiologies of hypersensitivity pneumonitis?

A

Farmer’s lung: up to 7% of farming population.
Pigeon-breeder’s lung: 6-21% of breeders.
Humidifier lung

21
Q

What does the pathology show in hypersensitivity pneumonitis?

A

typically bronchiole-centric pattern with interstitial pneumonitis, poorly formed granulomas in 2/3; late stage disease – interstitial fibrosis with honeycombing, obliterative bronchiolitis.

22
Q

How is hypersensitivity pneumonitis diagnosed?

A
  • inhalation history
  • characteristic clinical and imaging features
  • BAL with lymphocytosis
  • transbronchial Bx.
23
Q

What is the treatment for hypersensitivity pneumonitis?

A

Avoid antigen exposure.
- course of corticosteroids for more severely
symptomatic patients.
- early disease reversible

24
Q

What is the classical presentation of Sarcoidosis?

A

presenting Sx: cough, dyspnea, chest pain.
- additional Sx: fatigue, malaise, fever, weight
loss ± skin, joint, eye, other organ disease.
- majority of patients: 10-40 years old.
- USA: 3-4 times more common in African
Americans: 2% lifetime risk.

25
Q

What does CXR show in Sarcoidosis?

A

bilateral hilar adenopathy ± reticular pulmonary opacities with mid to upper zone predominance:
-Stages I (mild) to IV (severe)

26
Q

What are the pathologic features of Sarcoidosis?

A

non-caseating granulomas along bronchovascular lymphatics and involving alveoli - with parenchymal fibrosis in advanced cases;
hilar lymph nodes: granulomatous lymphadenitis

27
Q

What does the BronchoAlveolar Lavage (BAL) show in Sarcoidosis?

A

increased lymphocytes with CD4/CD8 >4:1
- with typical transbronchial Bx findings = 80+%
predictive of sarcoidosis

28
Q

What is the clinical course of Sarcoidosis?

A
  • majority of patients recover with minimal to no residual symptoms (± corticosteroid Rx).
  • can be incidental CXR discovery: bilateral hilar adenopathy.
  • minority of patients: progressive pulmonary
    fibrosis, restrictive lung function, and cor pulmonale
29
Q

How is Sarcoidosis diagnosed?

A

By exclusion - always first rule out granulomatous infection

  • may be associated with hypercalcemia (enzymatic production of active Vitamin D which leads to increased calcium absorption)
  • **Biopsy to confirm.
30
Q

What are the forms of Pulmonary Vascular Disease?

A

THROMBOEMBOLISM, pulmonary hypertension, diffuse pulmonary hemorrhage syndromes/vasculitis

31
Q

Where do fat embolisms come from?

A

Bone fractures or orthopedic surgeries

32
Q

What are the types of acquired hypercoagulability?

A
  • immobilization/post-surgical.
    - pregnancy/contraceptive hormones.
    - obesity.
    - cardiac disease.
    - cancer*
    - anti-phospholipid antibody syndromes.
    - heparin-induced thrombocytopenia (HIT).
    - multiple additional disease states
33
Q

What are the types of congenital hypercoagulability?

A

Factor V Leiden, Prothrombin gene mutation, deficiencies of factors C, S or antithrombin III, fibrinolytic system deficiency.

34
Q

What does the EKG show in pulmonary embolism?

A

may show right heart strain (acute
cor pulmonale) or electromechanical
dissociation: NSR but without pulse.

35
Q

How is pulmonary hypertension defined?

A

mean pulmonary artery pressure > 25 mmHg at rest (normal 8-20)

36
Q

What are the causes of pulmonary hypertension?

A

1) Chronic hypoxemic vasoconstriction: chronic lung disease (COPD/ILD), obstructive sleep apnea syndrome.
2) Acquired left heart disease: e.g. mitral stenosis: increased L atrial pressure then increased pulmonary venous pressure then increased pulmonary arterial pressure.
3) Recurrent thromboemboli
4) Primary pulmonary arterial hypertension – PCWP (pulmonary venous pressure)

37
Q

What is the pathology of pulmonary hypertension?

A
  • Arterioles/small arteries with medial hypertrophy/intimal fibrosis: some patients have significant vasospastic component
  • PVOD: venule sclerosis
  • R ventricular hypertrophy
  • If organizing thrombi present: suggests chronic recurrent pulmonary emboli
38
Q

What are the clinical features of pulmonary hypertension?

A
  • associated underlying disease may be
    dominant clinically (COPD, ILD).
    • cardiac exam: increased intensity of pulmonic
      component of 2nd heart sound.
    • exertional dyspnea, lethargy, fatigue
      • inability to increase cardiac output.
    • RV hypertrophy with RV failure/cor
      pulmonale: angina, syncope, peripheral
      edema, passive hepatic congestion/abdominal
      pain, elevated JVP
39
Q

What are the most common causes of Pulmonary Hemorrhage resulting in hemoptysis?

A

Commonest causes (70%):

   *Bronchogenic carcinoma
   *Bronchiectasis
    Bronchitis
    Bacterial pneumonia
  *Tuberculosis
  * = can cause massive hemoptysis

Less commonly: pulmonary embolism
L ventricular failure
Mycetoma (fungus ball)
Pulmonary abscess
Rare causes of hemoptysis: miscellaneous diseases to
include: alveolar hemorrhage syndromes.

40
Q

What are diffuse pulmonary hemorrhage syndrome due to?

A

bleeding into alveolar spaces: diffuse alveolar hemorrhage (DAH)

41
Q

What are the symptoms of pulmonary hemorrhage syndrome?

A

usually abrupt/rapidly evolving symptom
onset with: cough, hemoptysis (may be
delayed), fever, dyspnea/hypoxemia.

42
Q

What are the chest imaging findings in pulmonary hemorrhage syndrome?

A

CXR/CT: diffuse alveolar opacities.

43
Q

What etiologies are associated with Diffuse Alveolar Hemorrhage?

A

Associations:
- drug/toxic injury to alveolar capillaries/wall - e.g. amiodarone, crack cocaine.
- ARDS/DAD.
- anticoagulation.
- mitral stenosis: now rare.
- pulmonary capillaritis (autoimmune).
- pulmonary infection/bone marrow
transplantation.
Mortality rates: up to 50%.
Long term risk: recurrent hemorrhage with pulmonary fibrosis.

44
Q

What are some diseases that may result with Diffuse Alveolar Hemorrhage?

A
  1. ) Goodpasture’s Syndrome
  2. ) Wegener’s granulomatosis
  3. ) Systemic lupus erythematosus
  4. ) Idiopathic pulmonary hemosiderosis/hemorrhage
45
Q

99% of lung tumors are what type?

A

carcinoma (ordinary lung cancer)

46
Q

What are the Clinicopathologic Lung Tumor classifications?

A

Non-small cell carcinomas (85%):
Adenocarcinoma (40-50%) - female dominant.
- a subset of cases have a weak to no
apparent relationship to smoking.
Squamous carcinoma (20-30%).
Large cell undifferentiated carcinoma (5-15%).
Misc. non-small cell types: sarcomatoid,
pleomorphic, adenosquamous, combined
histologies.
Small cell carcinoma (15%)

CRRAB II Week 7 - WLB (12 decks)

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