Pulm Path Pt 2 Flashcards
What does idiopathic pulmonary fibrosis lead to?
IPF damages pulmonary tissue with waves of inflammatory injury leading to fibrosis
What presents as:
- basilar infiltrates -> “honeycomb lung”
- dyspnea
- crackles on auscultation
IPF
What are the contributing factors of IPF?
- environmental: industrialized societies, smoking
- genetic factors
- increasing age (>50)
What is expected on pulmonary biopsy of IPF?
- normal areas
- inflammation
- fibroblast foci
- peripheral honeycombing
What are the potential therapies of IPF?
- lung transplantation
- medication to arrest fibrosis: tyrosine kinase inhibitors, TGFb inhibitors
Why does non-specific interstitial pneumonia (NSIP) have a much better prognosis than IPF?
It is more diffuse
- NOT non-specific
- uniform infiltrates and fibrosis
- no heterogeneity
- no fibroblast foci
- no granulomata
50-60 year old patient presents with pneumonia-like consolidation
- fibroblast foci, aka Masson bodies (organizing plugs of connective tissue that look like cotton candy)
Cryptogenic Organizing Pneumonia (COP)
What can be given to control COP?
excellent prognosis (full recovery) with oral steroid**
Why is COP a diagnosis of exclusion?
it is not infection, drug or toxin-induced, or related to connective tissue disorders
What are the three autoimmune/connective tissue diseases that can manifest as Interstitial Lung Disease (ILD)?
- Rheumatoid arthitis
- Systemic sclerosis
- Lupus erythematosus
What is prognosis linked to in ILD?
treatment of the underlying condition
What does sarcoidosis manifest as?
** non-caseating (non-necrotizing)** granulomata
African American pt, less than 40 years old, with incidental abnormal radiograph or dyspnea
- elevated serum ACE levels
sarcoidosis
- likely immune-related etiology, possible genetic predisposition
- death may occur from pulmonary, cardiac, or neurologic involvement
BERNIE MAC
What are the 5 stages of sarcoidosis on the basis of chest radiographs?
Stage 0: no abnormalities Stage 1: lymphadenopathy Stage 2: lymphadenopathy + pulmonary infiltration Stage 3: pulmonary infiltration Stage 4: fibrosis
NOTE: stages do not always occur in order, 20% of patients have progressive lung disease
If you see an Asteroid body or Schaumann bodies on a histo slide, what should you always think of?
sarcoidosis
What is hypersensitivity pneumonitis?
immune reaction to inhaled antigen
Pigeon-breeder’s lung, farmer’s lung and hot tub ling are all examples of what?
hypersensitivity pneumonitis
What does the diagnosis of hypersensitivity pneumonitis require?
thorough history!!!!
When would you see an AIRWAY-centered granulomata with associated lymphocytes?
hypersensitivity pneumonitis
40-50 year old patient, smoker, presents with restrictive lung disease
- histology shows very characteristic “stuffed” alveolar spaces full of macrophages
Desquamative Interstitial Pneumonia (DSIP)
What is the treatment for DSIP?
- stop smoking!
- corticosteriods
- good prognosis: >95% survival at 5 years
Within the spectrum of DSIP, but less symptomatic and earlier presentation (30-40 years old)
- smoking-related (dose dependent)
Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD)
NOTE: often reversible with smoking cessation if caught early
What is required to diagnose RB-ILD?
radiographic abnormalities on chest CT prompt biopsy
What is seen histologically in RB-ILD?
- macrophages present to a lesser extent
- “peribronchiolar” metaplasia
- may have fibrosis in advanced cases
Young smoker, presents with stellate lung lesion
Langerhands Cell Histiocytosis (LCH)
What is seen histologically in LCH?
- eosinophils
- langerhands cells (immature dendritic cells)
- varying fibrosis and cysts
Why would a patient with LCH present with pneumothorax?
- progressive scarring leads to cysts
2. peripheral cysts may rupture!!
Can LCH be reversed with smoking cessation?
yes!
What lab values are considered a giveaway for LCH?
CD1a positive, and abundant eosinophils
Impairment of surfactant metabolism due to defect of granulocyte-macrophage color stimulating factor (GM-CSF)
- accumulation of surfactant proteins throughout alveoli and airspaces
Pulmonary alveolar proteinosis
What are the typical demographics of pulmonary alveolar proteinosis patient?
- autoimmune pt (middle-aged female most likely)
- fluid collected from bronchioalveolar lavage shows copious amount of milky fluid
How can you treat pulmonary alveolar proteinosis?
GM-CSF (subcutaneous)
What do pulmonary infarcts look like?
wedge-shaped lesions
- begin as hemorrhagic, then fibrosis sets in
What is a saddle pulmonary embolism?
an emboli that wedges in primary arterial trunk
- patient dies of acute cor pulmonale because right heart can pump against pressure buildup
- they die before infarction/necrosis sets in
What is a hallmark of antemortem (before death) clots?
Lines of Zahn
What can obstruct vessels in a pulmonary emboli?
- blood clot
- bone marrow/fat
- cancer (tumor embolus)
- septic emboli
- air
- foreign material
- parasites (dorofilaria)
When would you see a bone marrow embolism?
after trauma
What is a talc embolism (looks shiney on gross biopsy) characteristic of?
IV drug users
What can cause nodules in drug users?
**septic emboli **
What is a septic emboli?
bloodborne infective material, valve vegetations break off and manifest in other sites -> LUNGS!