Pulmonary Pathology 3 Flashcards
What are three interstitial lung diseases that are fibrotic in nature?
usual interstitial PNA, non-specific interstitial PNA, and cryptogenic organizing PNA (COP)
how do you make the diagnosis of idiopathic pulmonary fibrosis?
diagnosis requires classic findings on high-resolution CT scan or pulmonary biopsy
what does pulmonary biopsy show if a clinician diagnoses a patient with idiopathic pulmonary fibrosis?
usual interstitial pneumonia (UIP)
what four things make up UIP?
normal areas, inflammation, fibroblast foci, peripheral honeycombing
Why do people get idiopathic pulmonary fibrosis?
it’s not quite well understood, but their are contributing factors such as environmental factors (industrialized societies, and smoking), genetic factors, and increasing age
what are the clinical findings associated with idiopathic pulmonary fibrosis?
shortness of air, crackles on exam (velcro), restrictive pattern on PFTs, basilar infiltrates that progress to honeycomb sign
how is honeycombing different from emphysema?
the spaces in honeycombing are surrounded by a think rhine
what is the prognosis of idiopathic pulmonary fibrosis?
bad; it has a progressive course- most patients die from the respiratory disease 3-5 years after diagnosis
what are the potential therapies for idiopathic pulmonary fibrosis?
lung transplantation or medication to arrest fibrosis such as tyrosine kinase inhibitors or TGF-beta inhibitors
what is non-specific interstitial pneumonia?
idiopathic, but it is NOT non-specific
what is the histologic features of non-specific interstitial PNA?
uniform infiltrates and fibrosis, no heterogeneity, no fibroblast foci, no granulomata
why is the interstitium thickened in NSIP?
there’s a bunch of inflammatory cells here- most of which are lymphocytes and some plasma cells
what is the predominant pattern of cryptogenic organizing pneumonia?
fibroblastic foci (Masson bodies)
what is the presentation of cryptogenic organizing pneumonia?
it occurs superimposed on a prior infection or inflammatory process; presents with pneumonia-like consolidation in the 5th/6th decades
what type of diagnosis is cryptogenic organizing pneumonia?
a diagnosis of exclusion- you have to make sure you aren’t dealing with an active infection, drug- or toxin-induced, or related to a connective tissue disorder
how do you treat cryptogenic organizing pneumonia?
patients tend to have a full recovery with oral steroids
what if a patients presents with symptoms of an idiopathic pulmonary fibrosis but they also have an underlying autoimmune/connective tissue disease (RA, systemic sclerosis, lupus erythematous), what would you call it?
a connective tissue associated ILD
what are two ILDs that are considered to be granulomatous diseases?
sarcoidosis and hypersensitivity pna
what is sarcoidosis?
a systemic disease manifesting non-caseating (non-necrotizing) granulomata in various organs
what is the clinical presentation of sarcoidosis?
incidental abnormal radiograph; dyspnea
what cells for the granuloma found in sarcoidosis?
epithelioid histiocytes
what is the most identifiable cell in a granuloma?
the multinucleated giant cell
how can you tell if someone has sarcoidosis based on chest radiograph?
there are different stages, but hilar lymphadenopathy is one of the common ways that sarcoidosis can present
what are the histologic features of sarcoidosis?
wavy collagen showing dense fibrosis; tight and well circumscribed
what are the two granuloma inclusions that should make you think sarcoidosis?
asteroid bodies, and schaumann bodies (both found in the giant cells)
What is the common demographic of sarcoidosis?
young, african american
what is a common lab finding associated with sarcoidosis?
elevated serum ACE levels
what is hypersensitivity pneumonitis?
an ill-defined granulomata; immune reaction to an inhaled antigen
what are 3 examples of hypersensitivity pneumonitis?
pigeon-breeder’s lung, farmer’s lung, and hot tub lung
what are you having an immune reaction to in cases of farmer’s lung?
actinomycetic spores in hay
What are you having an immune reaction to in cases of hot tub lung?
reaction to mycobacterium avium complex (MAC)
what is the classic characteristic of hypersensitivity pneumonitis?
airway centered granulomata with associated lymphocytes- you’ll see a very specific airway concentric granuloma
how do you diagnose hypersensitivity pneumonitis?
HISTORY
what are three interstitial lung diseases that are smoking-related diseases?
desquamative interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease, langerhans cell histiocytosis
how does desquamative interstitial pneumonia present?
smokers in their 4th-5th decade of life, restrictive lung disease presentation
what are the histologic features of desquamative interstitial pneumonia?
very characteristic “stuffed” alveolar spaces full of macrophages
what is the prognosis of desquamative interstitial pneumonia? Treatment?
good prognosis; stop smoking and corticosteroids
what is respiratory bronchiolitis-interstitial lung disease?
part of a spectrum with DSIP, but less symptomatic and earlier presentation
what is the presentation of respiratory bronchiolitis- interstitial lung disease?
3rd-4th decades, dose dependent; radiographic abnormalities prompt biopsy for diagnosis
what are the biopsy findings suggestive of RB-ILD?
macrophages present to a lesser extent, “peribronchiolar” metaplasia (abnormally located ciliated cells), may have fibrosis in advanced cases
what is langerhans cell histiocytosis (LCH)?
young smokers with stellate lung lesions; progressive scarring leads to cysts
what happens if the peripheral cysts seen in LCH rupture?
it can cause a pneumothorax
what are the histologic features associated with LCH?
eosinophils, langerhans cells (immature dendritic cells), varying fibrosis and cysts
how do you treat LCH?
can reverse with smoking cessation
how do you make the diagnosis of LCH?
there will be lots of eosinophils and when you stain it with CD1a it confirms it
what is pulmonary alveolar proteinosis?
impairment of surfactant metabolism
what causes pulmonary alveolar proteinosis?
a defect of granulocyte-macrophage colony stimulating factor (GM-CSF)
what is the common demographic of pulmonary alveolar proteinosis?
young to middle aged females who have an autoimmune phenomenon occurring
what is pneumoconiosis?
a reaction by the lungs to inhaled mineral or organic dust or vapors- occupational exposure or air pollution
what worsens cases of pneumoconiosis?
if exposure is high and repetitive, the size of particles is small, and smoking
what is the spectrum of coal workers’ pneumoconiosis?
anthracosis, coal macules/nodules, progressive massive fibrosis
most patients with coal workers’ pneumoconiosis will not have progressive disease; what is the exception to this?
the exception if the coal is bituminous coal (used to heat homes/cabins)
what is silicosis?
disease resulting from inhaled silicon dioxide
who is at risk for silicosis?
mining/quarry work; concrete repair/demolition
how does silicosis present?
insidious onset, can occur after exposure is no longer present; may progress to massive pulmonary fibrosis
what could be a complication associated with silicosis?
patients with silicosis are at two-fold risk of developing cancer (as opposed to CWP except bituminous coal-associated pneumoconiosis which is also linked to cancer)
what are the histologic features of silicosis?
dense collagenous nodules
what are the radiographic findings associated with silicosis?
eggshell calcifications (calcified hilar lymph nodes)
who is at risk for asbestosis?
insulation workers, shipyard workers (navy), paper mill workers, oil or chemical refinery workers
what are the disease manifestations of asbestosis?
pleura: fibrosis/fibrous plaques, effusions, mesothelioma; lung: interstitial fibrosis, carcinoma; extrapulmonary neoplasms like in the liver
what are the histologic features associated with asbestosis?
pleural plaque formation; candlewax drippings on pleura; histologically showing hyalinized collagen
when you get a pulmonary infarct due to pulmonary embolus, what does it look like?
wedge-shaped lesions
what is a talc embolus?
seen in people who inject drugs intravenously
