Respiratory Pathology - 3 (ILDs) Flashcards
Interstitial Lung Diseases are what type of Lung Diseases?
Restrictive
What characterizes Restrictive (Interstitial) Lung Diseases?
DECREASED VOLUME
Levels of FEV1/FVC, FVC, TLC for Restrictive (Interstitial) Lung Diseases?
FEV1/FVC = Normal FVC = Decreased TLC = Decreased
Idiopathic Pulmonary Fibrosis involves lung tissue being damaged in what fashion?
WAVES of inflammatory injury –> Fibrosis
With Idiopathic Pulmonary Fibrosis, what does the pathology show?
UIP = Usual Interstitial Pneumonia
What is Usual Interstitial Pneumonia and what is it seen with?
- Seen with Idiopathic Pulmonary Fibrosis
1. Normal areas
2. Inflammation areas
3. Fibroblast foci
4. Peripheral honeycombing fibrosis
What is Usual Interstitial Pneumonia and what is it seen with?
- Seen with Idiopathic Pulmonary Fibrosis
1. Normal areas
2. Inflammation areas
3. Fibroblast foci
4. Peripheral honeycombing fibrosis
What are some contributing factors for Idiopathic Pulmonary Fibrosis?
Smoking
Increasing age
Genetics
What are the symptoms, auscultation and X-ray findings with Idiopathic Pulmonary Fibrosis?
- Dyspnea
- Velcro-like crackles
- Basilar infiltrates
Prognosis and possible treatments for Idiopathic Pulmonary Fibrosis?
- Poor prognosis, death within 3-5 years
- Possible treatments:
1. Lung transplant
2. Arrest Fibrosis - Tyrosine Kinase Inhibitors
- TGF-beta Inhibitors
What autoimmune disease can manifest as Idiopathic Pulmonary Fibrosis?
Rheumatoid Arthritis
Non-specific Interstitial Pneumonia (NSIP) is also idiopathic and has a better prognosis than UIP. What is the histology?
UNIFORM pattern of inflammation and fibrosis
A UNIFORM pattern of inflammation and fibrosis is seen with?
NSIP (non-specific interstitial pneumonia)
What autoimmune disease can manifest as Non-Specific Interstitial Pneumonia (NSIP)?
Systemic Sclerosis
– UNIFORM inflammation and fibrosis
When does Cryptogenic Organizing Pneumonia (COP) occur?
Superimposed on prior infection/inflammatory process
Cryptogenic Organizing Pneumonia (COP) histology findings?
Fibroblast foci = plugs of loose connective tissue
masson bodies
Masson bodies = plugs of loose connective tissue (fibroblast foci) are seen with which ILD?
Cryptogenic Organizing Pneumonia (COP)
Cryptogenic Organizing Pneumonia is a diagnosis of ______ and patients will fully recover with?
Diagnosis of Exclusion!
- Patients fully recover with oral steroids
What autoimmune disease can manifest as Cryptogenic Organizing Pneumonia (COP)?
SLE
What are 2 Granulomatous ILDs?
Sarcoidosis
Hypersensitivity Pneumonitis
Sarcoidosis
Systemic disease with NON-CASEATING Granulomata in various organs
Non-caseating granulomata in various organs, usually involves lungs/hilar lymph nodes
Sarcoidosis
Describe the patient that usually presents with Sarcoidosis
Younger Black person with elevated ACE levels and/or dyspnea
Sarcoidosis involves granulomatous inflammation. What cells/inclusions should you look for?
Giant cells
- Asteroid body or Schaumann bodies inclusions
What are the stages of Sarcoidosis and do they occur in order?
NO they do not occur in order 1 = lymphadenopathy 2 = lymphadenopathy and pulmonary infiltrate 3 = pulmonary infiltrate 4 = fibrosis
Sarcoidosis patients will usually die to what involvement?
Pulmonary
Cardiac
Neurologic
What defines Hypersensitivity Pneumonitis?
Ill-defined airway centered Granulomata
Ill-defined airway centered Granulomata
Hypersensitivity Pneumonitis
What does a diagnosis of Hypersensitivity Pneumonitis require?
HISTORY
Hypersensitivity Pneumonitis is an immune reaction due to?
Inhaled antigen
Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled protein from bird poop
Pigeon Breeder’s lung
Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled actinomycetic spore in hay
Farmer’s lung
Hypersensitivity Pneumonitis that involves an immune reaction to an inhaled MAC?
Hot Tub lung
What are 3 smoking related ILDs?
Desquamative Interstitial Pneumonia
Respiratory Bronchiolitis - ILD
Langerhans Cell Histiocytosis
What are 3 smoking related ILDs?
Desquamative Interstitial Pneumonia (DSIP)
Respiratory Bronchiolitis - ILD (RB-ILD)
Langerhans Cell Histiocytosis (LCH)
Desquamative Interstitial Pneumonia (DSIP) involves smokers at what age and what is the treatment?
40-50s
Stop smoking and corticosteroids – good prognosis
Desquamative Interstitial Pneumonia (DSIP) histology findings?
Stuffed alveolar spaces full of macrophages
Stuffed alveolar spaces full of macrophages is seen with?
DSIP (desquamative interstitial pneumonia)
– smokers in 40-50s
Respiratory Bronchiolitis - ILD involve smokers at what age?
30-40s and less symptoms than DSIP
What will be seen on histology with RB-ILD?
- Less macrophages
- Peribronchiolar Metaplasia
(abnormally located ciliated cells) - Fibrosis in later stages
What will be seen on histology with Respiratory Bronchiolitis - ILD?
- Less macrophages
- Peribronchiolar Metaplasia
(abnormally located ciliated cells) - Fibrosis in later stages
If RB-ILD is caught early and with smoking cessation it can be ____
Reversible
What prompts a biopsy for diagnosis with RB-ILD?
Radiographic abnormalities
Who does Langerhans Cell Histiocytosis affect and what lesions are present?
Young smokers with stellate lesions on lungs
Describe how Langerhans Cell Histiocytosis (LCH) may present?
- Progressive scarring
- Cysts
- Cysts rupture
= Pneumothorax
Describe how Langerhans Cell Histiocytosis (LCH) may present?
- Progressive scarring
- Cysts
- Cysts rupture
= Pneumothorax
What cells/things are present on histology with Langerhans Cell Histiocytosis?
- Eosinophils
- Langerhans cells (immature dendritic cells)
- Fibrosis and cysts
What markers are (+) with Langerhans Cell Histiocytosis?
(+) s-100 and CD1a
Langerhans Cell Histiocytosis will reverse with?
Smoking cessation
(+) s-100 and CD1a
Langerhans Cell Histiocytosis
Pulmonary Alveolar Proteinosis (PAP)
Impairment of surfactant metabolism that causes it to accumulate throughout alveoli and airspaces
Impairment of surfactant metabolism that causes it to accumulate throughout alveoli and airspaces
Pulmonary Alveolar Proteinosis (PAP)
Pulmonary Alveolar Proteinosis is due to a defect in?
GM-CSF
An autoimmune defect in GM-CSF will cause?
Pulmonary Alveolar Proteinosis (PAP)
– buildup of surfactant
What is treatment for Pulmonary Alveolar Proteinosis?
Subcutaneous GM-CSF