Pathology Of Restrictive Lung Diseases Flashcards

1
Q

PFT values in restrictive diseases

A

FEV is reduced

FEV1 is reduced

FEV1/FVC ratio is either normal or increased

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2
Q

2 general categories of restrictive lung diseases

A

1) chest wall disorders w/ normal lungs
- obesity, pleural diseases, neuromuscular disorders

2) disorders of the lung parenchyma w/ normal chest wall
- ARDS
- pneumonconioses, interstital fibrosis
- sarcoidosis

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3
Q

What are the end-stage conditions of restrictive lung diseases?

A

Respiratory failure, pulmonary HTN and cor pulmonale

causes ventilation- perfusion ratio mismatches and hypoxia

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4
Q

Idiopathic pulmonary fibrosis

A

Fibrosis category of restrictive lung disease

Unknown etiology that is characterized by patchy, progressive bilateral interstitial fibrosis

Most commonly affects males, but can affect females

Almost always occurs after age 50

  • Requires usual interstitial pneumonia pattern to diagnose*
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5
Q

Cellular senescence and IPF

A

It is hypothesized that repeated injury and defective repair of alveolar epithelium eventually causes germ line mutations that’s leads to a loss of telomerase.
- causes premature cellular senescence and prevents mitosis

Also is hypothesized that exuberant fibroblastic foci is developed, leading to mass fibrosis

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6
Q

IPF morphology

A

Pleural surfaces of the lung look cobble-stoned and hard
- also possess rubbery white areas of fibrosis, mostly in the lower lobe of the lungs

Histologically shows honeycomb fibrosis
- formed by hyperplastic type 2 pneumocystis and causes alveolar wall collapse

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7
Q

IPF clinical features

A

Nonproductive (no sputum) coughing w/ progressive dyspnea

Velcrolike crackles (dry rales)

Shows cyanosis, peripheral edema in late stages

Honeycombing on xrays and CTs

Treatments

  • NO anti-inflammatory
  • anti-fibrotic therapies are good, but only if caught early and most are not
  • lung transplant is the only definitive treatment (however this is really high risk)
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8
Q

Pneumoconioses

A

Lung disorders caused by inhalation of organic/inorganic dusts/particles

Three most common (not in order) are:

1) coal dust * not reactive*
2) silica reactive
3) asbestos reactive

Note: this is especially problematic in cystic fibrosis patients since mucocillary movement is primary mover in getting these particles out

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9
Q

Pathogenesis of pneumoconioses

A

Dependent on the following characteristics

1) size
- > 5 microns in size = get lodged in upper airways
- <0.5 microns in size = move into the alveoli and come out normally
- 1-5 microns in size = get larger in alveoli and are the MOST dangerous

2) shape
3) reactivity of the particles

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10
Q

Pneumoconioses pathogenesis

A

Following phagocytosis by macrophages, many particles activate the inflammasome and produce IL-1
- cause fibroblast proliferation and collagen deposition

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11
Q

Coal workers pneumoconiosis

A

Includes 3 sub types

1) asymptomatic Anthracosis
2) simple coal workers pneumoconiosis
3) complicated CWP or progressive massive fibrosis (PMF)

All 3 produce black lung disease due to carbon pigmented macrophages getting trapped in the vasculature

Symptoms/signs

  • usually asymptomatic and benign (until it hits #3)
  • in PMF, cause spulmonary HTN, dyspnea, chest pain, peripheral edema and cor pulmonale
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12
Q

Silicosis

A

THE MOST PREVALENT CHRONIC OCCUPATIONAL DISEASE

Caused by inhalation of silica
- causes activation of inflammasomes and produces increased fibrogenic cytokines

Morphology

  • shows tiny and discrete pale/black nodules in upper zones of the lungs only
  • lots of collagen fibers surrounding the tissues

Clinical features:

  • fine nodules in the upper lung zones and the outlining of the lungs on xrays
  • most symptoms are not present until PMF is present
  • increased susceptibility to TB* and often shows granulomas

Treatment:

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13
Q

Asbestos

A

Includes a wide group of crystalline silicate particles

Causes some of the following

1) parenchyma interstital fibrosis
2) localized fibrous plaques
3) pleural effusions
4) lung cancers
5) mesothelioma (malignant lung cancers)

Signs/symptoms

  • progressively worsening dyspnea (usually diagnostic 10 yrs after exposure)
  • productive cough
  • disease usually remains static, but can progress to CHF/cor pulmonale
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14
Q

Asbestosis unique pathogenesis

A

Functions as both a tumor initator and promoter

  • generates reactive free radicals, destroying mesothelium in the distal lungs/lower lobes
  • spreads to middle and upper as disease progresses

Also increases absorption of potentially toxic chemicals that may be inhaled
- amplifies toxic chemical effects

Shows honeycombing as well (similar to IPF)

Also can develop calcium plaques on the diaphragm or base of the lungs

morpholgoy shows unique golden brown staining asbestosis bodies

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15
Q

Asbestosis and cancer relationship

A

Risk for developing lung carcinomas
- 5x increase

Mesothelioma
- 1000x increased

note both values increase even more if you are smoking also

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16
Q

Sarcoidosis

A

Multi-system disease with unknown etiology

  • causes noncaseating granulomatous inflammation in tissues/organs
  • mimics mycobacterial/fungal infections

Prevalence in women is greater and blacks vs whites are greater
- very rare in Chinese and southeastern Asians

Signs/symptoms:

  • most common is bilateral hilar lymphadenopathy on xray
  • eye irritation, lacrimal gland inflammation and glaucoma
  • will also often show paratracheal and axillary lymphadenopathy
  • skin lesions (only 25% patients)
  • spleen granulomas
  • hypercalcemia/calciuria
  • serum angiotensin converting enzyme levels is increased

Diagnosis is a diagnosis of exclusion

17
Q

Sarcoidosis pathogenesis

A

Hypothesized to be immune regulation disorders

  • suggested CD4+ helper T0cells drive antibody production against unidenfited antigens, producing granulomas
  • Is a type 4 hypersensativity reaction most often

no evidence that shows what the antigen is

Morphology shows giant cells and granulomas
- overtime the granulomas devolpment pink collagen outlines

18
Q

Schaumann bodies and asteroid bodies

A

Two morphological appearances in sarcoidosis

Schaumann bodies
- large laminated concretions of calcium and proteins (look like a black hole)

Asteroid bodies
- stellate inclusions enclosed within giant cells