B4-056 Restrictive Pulmonary Disease Flashcards
- disorders involving inflammation and fibrosis of the lung parenchyma, particularly the interstitium
- causes reduced diffusion capacity
interstitial lung disease
space between the epithelial and endothelial basement membranes in the alveolar wall
interstitium
fibrosing interstitial lung diseases
just the 3 she said we’re responsible for
- idiopathic pulmonary fibrosis
- non-specific interstitial pneumonia
- pneumoconiosis
insidious onset of dyspnea on exertion
dry cough
hypoxemia, cyanosis, clubbing
inspiratory crackles
idiopathic pulmonary fibrosis
CT findings
- bilateral basilar-predominant reticulation and bronchiectasis
- bilateral subpleural honeycomb cysts
idiopathic pulmonary fibrosis
chronic fibrosing interstitial lung disease with high mortality
idiopathic pulmonary fibrosis
- female
- non-smoker
- dyspnea, cough of several months duration
non specific interstitial pneumonia
- interstitial fibrosis and inflammation
- lung architecture preserved
- no honeycomb/fibroblast foci
non specific interstitial pneumonia
temporal homogenity
non specific interstitial pneumonia
- Patchwork of interstitial fibrosis
- septal distribution
- dense fibrosis/honeycombing
- fibroblast foci
UIP
inflammatory reaction of the lung to inhaled dust/mineral particles
pneumoconiosis
proinflammatory cystalling hydrated silicates
asbestos
pneumoconiosis
golden brown beaded rods with translucent center
ferruginous body
asbestos pneumoconiosis
caused by inhalation of proinflammatory crystalline sillicon dioxide
silicosis
pneumoconiosis
most prevalent occupation disease worldwide
concrete, stone cutting, sand blasting, hard rock mining
silicosis
pneumoconiosis
pneumoconiosis causes activation of
inflammasome
bright crystals under polarized light
silicosis
pneumoconiosis
- alveolar proteinosis
- cholesterol clefts
- eosinophilic globules
acute silicosis
inhaled carbon dust engulfed by alveolar or interstitial macrophages
anthracosis
coal workers pneumoconiosis
coal macules and nodules consisting of carbon laden macrophages and scar
simple coal workers pneumoconiosis
- large scars with dense collagen and black pigment
- progressive massive fibrosis
complicated coal workers pneumoconiosis
granulomatious interstitial lung diseases
2
- sarcoidosis
- hypersensitivity pneumonitis
sarcoidosis
- multi-nucleated giant cells
- epithelioid
- non-necrotizing
- hyalinizing fibrosis
sarcoidosis
- high ACE level
- hypercalcemia
- hypercalciuria
sarcoidosis
from immune reactiong provoked by inhaled antigen in susceptible individuals
hypersensitivity pneumonitis
- increased CD4 and CD8 T lymphocytes
- non-necrotizing granulomas
- antigen specific IgG antibodies
- complement and Ig deposits
hypersensitivity pneumonitis
- insidious onset SOB, cough, weight loss, exercise intolerance
- acute episodes following inhalation of irritant w/ fever, cough, elevated WBC
hypersensitivity pneumonitis
- mosaic perfusion
- BAL fluid >20% lymphocytes
hypersensitivity pneumonitis
interstitial poorly formed non-necrotizing granulomas
hypersensitivity pneumonitis
characterized by proliferation of Langerhans cells
pulmonary langerhans cell histiocytosis
- smoking related
- BRAF or MPK connection
pulmonary langerhans cell histiocytosis
- peribronchial nodules on CT, upper lobe predominate
- bird beak or tennis racket on electron microscopy
pulmonary langerhans cell histiocytosis
eosinophilic granuloma
pulmonary langerhans cell histiocytosis
CD1a, S100, Langerin positive
pulmonary langerhans cell histiocytosis
generally improves after smoking cessation, barring BRAF/MPK mutation
pulmonary langerhans cell histiocytosis
airspace filling lung diseases
2
- pulmonary alveolar proteinosis
- cryptogenic organizing pneumonia
- positive periodic acid stain
- accumulation of surfactant in airspaces
pulmonary alveolar proteinosis
- dyspnea
- productive cough
- gelatinous sputum
pulmonary alveolar proteinosis
CT finding
crazy paving
pulmonary alveolar proteinosis
whorled lamellar bodies in type 2 pneumocytes on electron microscopy
pulmonary alveolar proteinosis
- polypoid branching plugs (Masson bodies)
- mild inflammation
- preserved architecture
organizing pneumonia
- subacute cough with dyspnea
- patchy bilateral ground glass opacities
- idopathic
cryptogenic organizing pneumonia
destruction of bronchial walls by recurrent inflammation
bronchiectasis
irreversible dilation of bronchi from inflammation and destruction of bronchial walls following prolonged infections/obstructions
bronchiectasis
unopposed action of elastases damaging alveolar tissue
emphysema
bronchial mucosa damage associated with eosinophils
bronchial asthma
destruction of the walls of distal airspaces without prominent fibrosis
emphysema
associated with nasal polyps, chronic rhinitis, severe asthma and intolerance to aspirin
drug induced asthma
chronic productive cough without cause for > 3months in a 2 year period
chronic bronchitis
90% of all cases occur in smokers
chronic bronchitis
- hypertrophy of bronchial submucosal glands and hyperplasia of goblet cells
- excessive mucus production and airway obstruction
chronic bronchitis
thickening of the bronchial epithelial basement membrane and hypertrophy of bronchial smooth muscle
atopic asthma
contact with allergen results in degranulation of mast cells and release of histamines
atopic asthma
dilation of respiratory bronchioles
centrilobar emphysema
bronchial dilation with inflammatory destruction
bronchiectasis
most patient clinically diagnosed with emphysema younger than 40
A1AT deficiency
- charcot leyden crystals
- curshmann spirals
- inflammatory cells in mucus
atopic asthma
marked thickening of pulmonary arteries
pulmonary hypertension
will have characteristics of both obstructive and restrictive
pulmonary hypertension
destruction of tissue in alveolar wall
emphysema
fibrosis of alveolar wall
restrictive diseases
non-necrotizing granulomas with multisystem involvement
sarcoidosis
increased susceptibility to mycobacterium
silicosis
most prevalent occupational disease worldwide
silicosis
hypersensitivity pneumonitis caused by inhalation of actinomycete spores
farmer lung
pneumonitis
neoplastic spindle and epitheliod cells that invade adipose tissue
asbestos
polypoid plugs of organizing connective tissue
cryptogenic organizing pneumonia
in-alveolar accumulation of granular eosinophilic material due to build up of surfactant
pulmonary alveolar proteinosis
hyaline membranes lining alveolar space
diffuse alveolar damage
abnormal dilation of distal airspaces
emphysema
temporally heterogenous fibrosis with fibroblast foci and interstitial fibrosis
UIP
firm, tan mass encasing the pleura
malignant meothelioma
diaphragmatic calcified plaques
mesothelioma
localized bronchial dilation due to chronic or recurrent infection
bronchiectasis
immature dendritic cells with grooved, identical nuclei
langerhans
positive stain for S100 and CD1a
langerhans
after quitting smoking, patients with Langerhans cell histiocytosis
usually recover