Interstitial lung disease Flashcards by Jason S

What is the definition of interstitial lung disease?

lung parenchymal disorders with common clinical, radiologic, physiologic and pathologic features; hallmark-involvement of interstitium

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What is infiltrative lung disease?

infiltration of cellular and non-cellular elements within alveolar septa and alveoli

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What is diffuse parenchymal lung disease?

DLPD

type of interstitial lung disase

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What is restrictive lung disease?

characterized by reduced total lung capacity in presence of a normal or reduced expiratory flow rate

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]What are the chronic interstitial lung disease?

fibrosing lung disorder (pneumoconioses)
granulomatous disorder (sarcoidosis)
idiopathic interstitial pneumonias (IIPs)

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What is alveolitis?

damage to pneumocytes and endothelial cells

leads to leukocytes releasing cytokines which mediate and stimulate interstitial fibroisis

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What are the symptoms of interstital fibrosis?

decreased lung compliance and elasticitiy leading to

decreased lung expansion during inspiration

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What are the clinical and laboratory findings?

Dry cough and dyspnea
late inspiratory crackles, bibasilar (VELCRO CRACKLES)
Cor pulmonale
chest radiography-bilateral reticulonodular infiltrates

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What is pneumoconioses?

non-neoplastic lung diseases in response to inhalation of mineral dusts inhaled in the workplace

now expanded to include disease induced by organic and inorganic particulate matter

coal dust, silica, asbestos. beryllium 25% cases of chronic interstitial lung disease

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What is the general pathogenic principles of pneumoconioses?

development

amount of dust retained in lung parenchyma
size, shape and buoyancy of particles
- 1-5 micrometers reach bifurcation of respiratory bronchioles and alveolar duts
-<0.5 micrometers reach alveoli and phagocytosed by alveolar macrophages
particle solubility and phsicochemical reactivity
possible additional effects of other irritants

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What is coal workers’ pneumoconiasosis?

antithracotic pigment- coal mines, urban centers, tobacco

pulmonary anthracosis

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What is anthracosis?

asymptomatic

anthracotic pigment in interstitial compartment and lymph nodes

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Simple coal workers’ pneumoconiosis gross morphology?

fibrous opacities <1cm
upper lobes and upper portions of lower lobes
characterized by coal dust deposits adjacent to respiratory bronchioles

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What is complicated coal workers’ pneumoconiosis?

progressive massive fibrosis
fibrous opacities >1cm
w/ or w/o central necrosis
massive fibrosis
complication-Cor pulmonale
Caplan syndrome-CWP with rheumatoid nodules in lung
no increased incidence of TB or ccancer

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What is silicosis?

most comon occupational disease worldwide
crystalline silicon dioxide
quartz activates alveolar macrophages after engulfment ->cytokine release -> fibrogenesis

complications -cor pulmonale; association with Caplan syndrome
increased risk for TB and cancer

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What are asbestos-related disease appearances?

deposition sites-respiratory bronchioles, alveolar ducts and alveoli

Ferruginous bodies–macropahges and phagocytose asbestos fibers and coat them with ferritin

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What are the pathogenesis of asbestos-related disease?

benign pleural plaques -- not a prcursor of mesothelioma
diffuse interstitial fibrosis
bronchogenic carcinoma
-additional risk with smoking
-20 yrs after first exposure

Mesothelioma
-no relationship to smoking
-arises from lining mesothelial cells of pleura
-25 to 40 yrs after 1st exposure
no increased risk for TB
complications
-Cor pulmonale
-Caplan syndrome

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What is berylliosis?

granulomatous inflammation – TB and sarcoidosis

complications -cor pulmonale and lung cancer

What is sarcoidosis?

multisystem granulomatous disease of unkonwn etiiology

highest incidence in AA

disorder of immune regulation

What are the lab and radiologic findings of sarcoidsosis?

ACE levels
-marker of disease activity and response to steroids
hypercalcemia
polyclonal grammopathy
cutaneous anergy due to consumption fo CD4 Th cells
CXR
-bilateral hilar adenopathy
-reticulonodular shadows in lungs

What is HSN pneumonitis?

inhaled antigen producing granulomatous interstitial pneumonitis

Type III hsn rxn

first exposure – IgG antibodies
second exposure – antibodies combine with inhaled antigens to form immune complexes
Chronic exposure –granuloma formation (Type 4 HSN response)

What is HSN pneumonitis?

important types

farmers lung –moldy hay
-silos fillers disase
-byssinosis

What is idiopathic pulmonary fibrosis?

15% cases of chroonic lung disase
idiopathic interstitial pneumonias
usual interstitial pneumonia ~idiopathic interstitial fibrosis

What are the clinical manifestations of idiopathic pulmonary fibrosis?

dyspnea; non-specific constitutional symptoms such as fever, weight loss, fatigue

repeated injury to the lung –> interstitial fibrosis

interstitial fibrosis –> honeycomb lung

What is collagen vacular disease causes?

``` 10% of interstitial lung disease SLE -interstitial lung disase in 50% of pts RA -wide spectrum of pulmonary changes ``` ``` Systemic sclerosis (scleroderma) -interstitial fibrosis with pulmonary vascular hypertrophy ```

What is atelectasis?

lung in whole or part is collapsed or without air | -loss of lung volume due to inadequate expansion of airspaces

What are the types of atelectiasis?

resorption compression loss of surfacant contraction

What is resportion atelectasis?

consequence of complete airway obstruction obstruction in bronchi, subsegmental bronchi prevents air from reaching alveoli resorption of air in distal airspace through pores of kohn lack of air in distal space collapse

What are teh common causes of obstruction in resorption atelectasis?

mucus/mucopurlent plug following surgery aspiration of foreing material bronchial asthma, bronchitis, bronchiectasis bronchial neoplasm (caveat- total obstruction)

What are the clinical findings in resportion atelectasis?

fever and dyspnea (24-36 hours following surgery) ipsilateral deviation of trachea ipsilateral diaphragmatic elevation absent breath sounds and absent vocal vibratory sensation collapsed lung doesnt expand on inspiration

What is compression atelectasis?

air or fluid accumulation in pleural cavity --increased pressure--collapses underlying lung trachea and mediastinum shift away from atelectatic lung

What direction does trachea and mediastinum shift in - tension pneumothorax - pleural effusion?

compression atelectasis | shifts away from collapsed lung

What is loss of surfacant(neonatal atelactassis)?

``` lipoportein -phosphatidylcholine -phosphatidylglycerol proteins --SPA and D innate immunity --Surfacant protein B and C-- reduction of surface tension at air liquid barrier in alveoli ``` synthesized by ttype 2 pneumocytes - synthesis begins by 28th week of gestation - stored in lamellar boies

Neonatal atelactasis histology?

collapsed alveoli are lined by hyaline membranes

What are the clinical finidings of neonatal atelactasis?

respiratory distress within a few hours of birth hypoxemai and respiratory acidosis ground glass appearance on CXR

What are the complications of neonatal atelactasis?

``` intraventricular hemorrhage patenet ductus arteriosus necrotizing enterocolitis hypoglycemia O2 therapy-damages to lungs and cataracts ```

What is contraction atelectasis?

fibrotic changes in lung or pleura prevent full expansion (not reversible)

What is acute lung injury?

endothelial or epithelial injury initiated by numerous factors non-heritable and heritable mediators-cytokines, oxidants, growth factors manifestations - Pulmonary edema, diffuse alveolar damage (ARDS)

What is the cause of pulmonary edema in acute lung injury?

edema to alterations in starling pressure -microvascular or alveolar injury -- increase in capillary permeability -undetermined origin therapy and outcome depend on underlying etiology

What is acute respiratory distress syndrome?

noncardiogenic pulmonary edema resulting from acute alveolar capillary damage - -direct lung injury - -indirect lung injury (systemic disease)

What are teh conditions associated with development of majority of ARDS cases?

sepsis diffuse lung infections gastric aspiration physical injury/trauma

What are the clinical findings of acute respiratory distress syndrome?

dyspnea severe hypoxemia not responsive to O2 therapy respiratory acidosis

What is the pathogensis acute respiratory distress syndrome?

acute injury to alveolar epithelial or endothelial cells alveolar macrophages and other cells release cytokines -neutrophilic chemotaxis -transmigration of neutrophils from capillaries into alveoli -leakage of protein rich exudate forming hyaline membranes damage to pneumocytes causing surfacant deficiency atelectasis repair by type 2 pneumocytes progressive interstitial fibrosis