B4.056 - Restrictive Lung Diseases: Interstitial Lung Disease COPY Flashcards
cryptogenic organizing pneumonia
note: polypoid plugs
most prevalent chronic occupational disease
silicosis
what are other interstitial lung diseases
pulmonary alveolar proteinosis
langerhans cell histiocytosis/eosinophilic granulom
characterize idiopathic pulmonary fibrosis
chronic progressive fibrosing interstitial lung disease without an identifiable cause and with a high mortality rate
complicated CWP
progressive massive fibrosis
what leads to disease in pneumonconiosis
the particles inhaled stimulate an immune resonse that leads to disease
UIP in IPF
what is characteristic of the distribution of idiopathic pulmonary fibrosis
spatially and temporally hetoerogenous distribution: i.e. normal and abnormal areas, early fibrosis (fibroblast foci) and established fibrosis
describe how asbestos relates to malignancy
tumor initiator and promoter
asbestos fibers generate reactive free radicals in distal lung, close to mesothelium. toxic chemicals absorbed into asbestos fibers
loose granulomas in HS pneumonitis
most likey microscopic finding of HS P?
granuloma
asbestos body
brown indicates iron from macrophage ferritin
where does malignant mesothelioma arise
in pleura
cryptogenic organizing pneumonia
lab values in sarcoidosis
elevated serum ACE
hypercalcemia or hypercalciuria leading to nephrolithiasis and renal dysfunction can occur
enzyme 1a - hydroxylase in activated macrophages that convert 25-hydroxyvitamin D to 1,26 -mdihydroxyvitamin D, the active form of the vitamin. this results in increased gut absorption
Diseases affecting the pulmonary interstitium result in
Effective loss of lung tissue
decrease in lungs ability to expand
decrease in the lungs ability to transfer oxygen or CO2 within the blood
course of IPF
chronic progressive, downhill course
may have acute exacerbations
progressive lung disease, end stage lung and/or pulmonary hypertension
increased risk of lung cancer
respiratory failure most frequent cause of death
what are the symptoms of cryptogenic organizing pneumonia
variable onset
cough
dyspnea
may sponteneously resolve
prognosis for mesothelioma and treatment
50% die in 12 months of Dx
chemo, radiaton, extrapleural pneumonectomy may help
what happens when coal is inhaled
inhaled carbon pigment is engulfed by alveolar or intesrtitial macrophages, which then accumulate in connective tissue along lymphatics or in lympoid tissue
sarcoidosis
treatment for cryptogenic organizing pneumonia
steroid treatment for most patients for complete recovery
sarcoidosis
Schaumann body
describe the presentation of idiopathic pulmonary fibrosis
insidious onset of worsening dyspnea and dry couhg
5th - 6th decade at presentation
fine bibasilar inspiratory crackles in >80% of patients, with progression upward as teh disease advances
subsequent hypoxemia, cyanosis and clubbing
honeycomb lung
what other lesions are associated with sarcoidosis
eye and skin lesions
pulmonary langerhans histiocytosis
what is asbestos
crystalline hydrated silicates that form fibers
what is the differential for organizing pneumonia pattern
viral and bacterial
inhaled toxins
drugs
collagen vascular diseases
GVHD
BAL of hypersenstivity pneumonitis
increased CD4 and CD8 T lymphocytes
specific antibodies against the causative antigen in serum
complement and immunoglobulins
noncaseating granulomas in 2/3rd of patients: T cell mediated (type 4) HS againts antigens
clinicla presentation of HS pneumonities
acute episodes follow inhalation of antigenic dust in a previously sensitized patient usually 4-6 hrs after exposure. Recurrent episodes of dyspnea, fevver, cough and increased white cells
what happens in asbestos related disease
asbestos fibers are phagocytosed by macrophages acivate the inflammasone and stimulate the release of proinflammatory and fibrogenic factors
what is an anthracosis
innocuous coal induced pulmonary lesion in coal miners, all urban dwellers and tobacco smokers
what are fibrosisng interstitial lung diseases
- idiopathic pulmonary fibrosis
- non specific interstitial pneumonia
- cryptogenic organizing pneumonia
- drugs
- connective tissue diseases
- pneumoconiosis
- radiation
24 to African American woman presents with fatigue, ahces and pains, blurry vision, dry eyes and SOB. Small cutaneous bumps, uveitis and enlarged liver. Abnormal X rays restrictiv lung findings, increased ACE and 24 hr Ca++ excretion. Bronchioloalveolar lavage revealed CD4/8 ratio of >5
sacroidosis
pulmonary hypertension
what is pulmonary langerhans cell histocytosis/eosinophilic granuloma and who is it seen in
interstitial lung disease with proliferation of langerhans cells (immature dendritic cells)
adults
smoking
nodules or nodules with cystic change
recurrent pleural effusions in patients with asbestos exposrue
not common, think mesothelioma
what is this and when is it seen
Left arrow - established (late) fibrosis
Right arrow - uninvolved, note subpleural area more affected
Usual interstitial pneumonia
seen in idiopathic pulmonary fibrosis
sarcoidosis
clinical syndrome of HS pneumonitis
bird fanciers disease, (protein in serum, feathers, droppings)
Farmers lung (thermophilic actinomycetes in moldy hay)
Bagassosis (moldy sugar cane)
Humidifier lung (thermophilic bacteria in heated water reservoirs)
sarcoidosis
note: multinucleated giant cells
epitheliod histiocytes
CWP
dense scars with collagen and pigment
describe the way sarcoidosis looks on imagin
bilateral hilar lymphadenopathy or lung involvement is visible on chest imaging
what are characterstics of cryptogenic organizing pneumonia
polypoid plugs of loose organizing connective tissue within small airways, alveolar ducts, and the surrounding alveoli