(16.1) Pulmonary Pathology II (Singh) Flashcards
Idiopathic pulmonary fibrosis
How does it damage pulmonary tissue?
IPF damages pulmonary tissue with waves of inflammatory injury leading to fibrosis
What would a CXR look like for a idiopathic pulmonary fibrosis pt?
Basilar infiltrates
“Honeycomb lung”
What do the lungs sound like on auscultation for idiopathic pulmonary fibrosis?
Crackles on exam
“Velcro-like”
What are the contributing factors to idiopathic pulmonary fibrosis?
Enviornmental factors (SMOKING)
Genetic factors
Increasing age
What is unique about the histology of idiopathic pulmonary fibrosis?
Very different patterns due to the “wave like” nature of the disease
Some patches are normal, some have inflammation, others have fibroblast foci and some have peripheral honeycombing
What do pathologists call idiopathic pulmonary fibrosis when found on pulmonary biopsy?
Usual Interstitial Pneumonia (UIP)
What do these images represent?
Honeycomb fibrosis
What is the prognosis for patients with idiopathic pulmonary fibrosis?
NOT GOOD
Most patients die from respiratory disease 3-5 years after diagnosis
Only truly effective treatment = lung transplant
What are some experimental medications being used to treat idiopathic pulmonary fibrosis?
These meds are used to arrest fibrosis
- Tyrosine kinase inhibitors*
- TGF-Beta inhibitors*
What is non-specific interstitial pneumonia (NSIP)?
VERY SIMILAR TO UIP
Idiopathic
Has UNIQUE HISTOLOGY = uniform infiltrates and fibrosis
Has better prognosis than UIP
What is this lung disorder?
Non-specific interstitial pneumonia (NSIP)
What is a cute way to remember what cryptogenic organizing pneumonia (COP) looks like histologically?
Looks like cotton candy… awwww
The “cotton candy” is fibroblast foci (Masson bodies)
Cryptogenic organizing pneumonia (COP)
Prognosis?
Very good!
Patient tend to have full recovery with oral steroids
How do you diagnose cryptogenic organizing pneumonia (COP)?
Diagnosis of exclusion
-Not an infection, drug- or toxin-induced, or related to connective tissue disorders
What is this?
Granulomatous inflammation
What is sarcoidosis?
Systemic disease manifesting non-caseating (non-necrotizing) granulomata
What is the clinical presentation of sarcoidosis?
Incidental abnormal radiograph
or
Dyspnea
What are some of the hallmark granuloma inclusions of sarcoidosis?
Granuloma inclusions:
Asteroid body (A)
Schaumann bodies (B-D)
What is the demographic of sarcoidosis?
<40 years of age
African americans
Commonly involve LUNGS
Elevated ACE levels
Sarcoidosis
Do the stages occur in order?
NO!
What is this?
Hypersensitivity Pneumonitis
What is hypersensitivity pneumonitis?
Immune reaction to inhaled antigen
What are three examples of specific types of hypersensitivity pneumonitis?
Pigeon-breeder’s lung — protein from bird feces
Farmer’s lung — Actinomycetic spores in hay
Hot tub lung — Reaction to mycobacterium avium complex (MAC)
What is one of the most imporant aspects of your clinical interaction w/ your patient that will allow you to diagnose hypersensitivity pneumonitis?
HISTORY
What is the pathology?
Desquamative Interstitial Pneumonia (DSIP)
*Notice that every alveolar space has macrophages inside. Also, a MISNOMER, these are NOT squamous cells lol.
What is the demographic of desquamative interstitial pneumonia (DSIP)?
Smokers in 40s/50s
Restrictive lung disease presentation
What is the prognosis for desquamative interstitial pneumonia (DSIP)?
Good prognosis only IF THE PATIENT CAN STOP SMOKING
What is the pathology?
Respiratory Bronchiolitis-Interstitial Lung Disease
(RB-ILD)
What is the demographic for respiratory bonchiolitis-interstitial lung disease?
Smokers
30s-40s
What is the pathology?
Langerhans cell histiocytosis (LCH)