Restrictive Pulmonary Diseases Flashcards
This is the inflammation and fibrosis of the pulmonary connective tissue over time.
Chronic interstitial (restrictive) dieases
What are the 4 classic Sx for restrictive diseases?
Dyspnea
Tachypnea
End-inspiratory crackles
Cyanosis
The diffusing capacity for which molecule is decreased in restrictive conditions?
CO
Why do we give 2 shits about the diffusion of CO? What does it tell us?
The Diffusing Capacity of the Lungs for CO (DLCO) is a measurement of the ability of the lungs to transfer gas across the alveoli. in restrictive diseases, it’s ↓
What are the 3 things on CXR for restrictive diseases?
b/l infiltrates of small nodules, irregular lines, or ground-glass shadows
What is the end result of restrictive diseases? (think <3)
Pulmonary HTN –> RHF
cor pulmonale
This is the fribrosis of the lung interstitum from cyclical lung injury of epithelial activation from an unkown agent,
Idiopathic Pulmonary Fibrosis (IPF)
Which mediator is responsible for the the cyclical healing and fibrosis in IPF?
TGF-beta
What is the morphology of the pleural surface in IPF?
Cobble stone appearance from scarring
What is the morphology of the alveolar walls in IPF?
sooooper doooooper THICK
What is the morphology of the interstitum in IPF?
patchy interstitial fibrosis –> honeycomb fibrosis
What are the main Sx for IPF?
Gradual increasing dyspnea, dry cough
True or False: in nonspecific interstitial PNA, there is diffuse patchy interstitial fibrosis with fibroblastic foci and honeycombing, which makes it hard to distinguish from IPF.
FALSE. THere is no fibroblastic foci or honeycombing.
What causes nonspecific interstitial PNA?
nobody knows…
This is the disease of unknown origin where there is cough, dyspnea, subpleural/peribronchial patchy areas of consolidation with polypoid plugs of loose organizing tissue.
Cryptogenic Organizing PNA
What is the Tx for IPF?
Transplant
Thse are non-neoplastic lung reactions to chronic inhalation of mineral dusts that are fibrogenic.
Pneumoconioses
Which cells eats the small particles and triggers fibrosis in Pneumoconioses?
Macrophages
True or False: the smaller the particle, the more rapid the toxic levels achieved, and the faster the acute lung injury.
True
Which substance causes coal workers pneumoconiosis (CWP)?
Carbon
This is a disease of CWP where the inhaled carbon piugment is engulfed by macrophages which then accumulate in the lungs connective tissue along with the lymphatics.
Athracosis
Does a mild or severe exposure of carbon cause anthracocis in CWP?
Mild
This is the type of CWP where there are coal macules (carbon-laden macrophages) and somewhat larger coal nodules in the upper lobes and middle lung.
Simple CWP
This is the form of CWP where there is multiple intensely blackened scars of dense collagne and pigment that is often necrotic.
Complicated CWP
What is the high-yield disease association with CWP?
Rheumatoid Arthritis
causes Caplan syndrome
What % of CWP pts get progressive massive fibroisis, leading to increasing pulmonary dysfxn, pulmonary HTN, and cor pulmonale?
< 10%
What is the susbtance inhaled to cause silicosis pneumoconiosis?
Silica dust
Who is at risk for silicosis?
Miners and sandblasters
What is the morpholgy and location for silicosis in the lung?
fibrotic nodules in the upper lobes
Inhalation of silica causes it to interact with epithelial cell and macrophages, leading to the release of what?
mediators (like TNF)
These are thin sheets of calcification in the lymph nodes in silicosis
Eggshell calcification
The nodules in silicosis are hylaninized collagen surrounded by a capsule of what?
Of more codensed collagen
collagen on collagen… a collagen sandwhich.
What are the classic Sx to silicosis?
shortness of breath, slow to kill, progressive even if pt is no longer exposed.
What is the pt susceptible to in silicosis?
TB
UPPER LUNG = SILICOSIS AND TB.
I can’t wait for this to be a test question. It will. OMG it will.
Additional Pathoma
This is the penumoconiosis in miners/aerospace workers that has noncaseating granulomas, hilar lymph nodes, and organs involved.
Berylliosis
Additional Pathoma
Berylliosis increases the risk for what?
Lung CA
This is the pneumoconiosis of construction workers/plumers/shipyard workers where there is fibrosis of the lungs and pleura.
Asbestosis
What is the most common form of asbestos, serpentine or amphibole?
Serpentine
Where does serpentine asbestos impact?
URT
Why is amphibole asbestos more rare but more pathogenic?
Dur to its aerodynamic properties (like a spear), causes mesothelioma
Both forms of asbestos are fibrogenic and increase the risk of CA by doing what?
Generates free radicles
There is a big correlation between lung carcinoma and what habit of asbestos workers?
Smoking
Thse are golden brown fusiform or beaded rods with a translucent center with Fe.
Asbestos body
What is the mrophology of the lung in asbestosis?
Diffuse interstitial fibrosis with multiple asbestos bodies.
So there is fibrosis of what area in asbestosis, lung or pleura?
TRICK QUESTION.
Both.
So there is cancer development in which area from asbestosis, lung or pleura?
TRICK QUESTION AGAIN.
Both.
What is the common Sx for asbestosis?
Progressive dyspnea with productive cough 10-20 years after exposure.
What shows up on CXR for asbestosis?
irregular linear densitis (esp lower lobes), possible pleural effusion
Which cancer is pretty unique to asbestosis, carcinoma or mesothelioma?
Mesothelioma
Which cancer is more COMMON in asbestosis, carcinoma or mesothelioma?
Carcinoma
What are the 2 common drugs to cause IPF?
Bleomycin and Amiodarone
What therapy can induce IPF from direct damage?
Radiation
Methotrexate and Nitrofurantoin can cause what problems in the lung?
hypersensitivity pneumonitis
This is the form of radiation pneumonitis where there is fever, dyspnea, pleural effusion and radiologic infiltrates 1-6mo after fractionated irradiation.
Acute radiation pneumonitis
Chronic radiation pneumonitis is the consequence of repair of the injured endothelial/epithelial cells and severe atypia of which lung cells?
Type II cells and fibroblasts
This is a systemic disease of unknown cause characterized by noncaseating granulomas in many tissues and organs.
Sarcoidosis
What 2 things do u see on CXR for sarcoidosis?
bilateral hilar lymphadenopathy or lung involvement
Who is at risk for sarcoidosis?
Black women from the south east
What type of immune response causes sarcoidosis?
CD4+ Th activation to unknown Ag
What is the morphology of the noncaseating granulomas for sarcoidosis?
aggregte of lightly clustered EPITHELIOID cells with Shaumann bodies and stellate inclusions (ASTEROID BODIES) in giant cells.
What are the Sx to eye involvement in sarcoidosis?
Uveitis
What are the Sx to lymph node involvement in sarcoidosis?
Enlarged, calcified, hilar and mediastinal nodes involved
What are the Sx to bone marrow involvement in sarcoidosis?
small circumscribed areas of bone resporption within the marrow cavity and diffuse reticulated pattern throughout the cavity.
Dry eyes and mouth mimic which syndrome in sarcoidosis?
Sjoren syndrome
What are the Sx to skin involvement in sarcoidosis?
subQ nodules
Erythmateous plaques
Flat scaly slightly reddened lesions
What are the Sx of onset in sarcoidosis?
Respiratory abnormalitits (SOB, cough, chest pain, hemoptysis)
What 2 things are increased on blood exams for sarcoidosis?
- ACE
2. Ca++ (hypercalcemia from 1a-hydroxylase activity of granulomas)
Most patients recover w/o Tx for sarcoidosis, but if they need help, what is the class of drugs u can give?
Steroids
This is a spectrum of immunologically mediated, interstitial lung disorders caused by intense, prolonged exposure to inhaled organic Ag’s.
Hypersensitivity penumonitis
What type of hypersensitivity Rxn is Hypersensitivity penumonitis?
Type IV
T-cell mediated
What 4 things show up on the lungs in Hypersensitivity penumonitis?
- Noncaseating granulomas
- Interstitial pneumonitis
- Interstitial Fibrosis/honeycombing/obliterative bronchiolitis
- Intra-alveolar infiltrate
What are the Sx to Hypersensitivity penumonitis?
Fever, cough, dyspnea after exposure, gets better after removing exposure.
You can get interstitial fibrosis after what time in Hypersensitivity penumonitis?
Chronic exposure to allergen
Which WBC is elevated in Hypersensitivity penumonitis?
Eosinophils
it’s a hypersensitivity rxn
This is the infiltration of eosinophils in the lungs from high levels of alveolar levels of attractants, like IL-5.
Pulmonary eosinophilia
What causes acute eosinophilic PNA with respiratory failure?
Unknown
Simple pulmonary eosinophilia (Leoffler syndrome) shows what in the lungs?
Transient pulmonary lesions
Infections with microfilarie can cause what type of pulmonary eosinophilia?
Tropical eosinophilia
Other infections, hypersensitivity pneumonitis, or drug allergies can trigger which type of pulmonary eosinophilia?
secondary eosinophilia
This is the form of pulmonary eosinophilia where there are focal areas of cellular consolidation (lymphocytes and eosinophils) of the lung substance distributed chiefly in the periphery of the lung fields.
Idiopathic chronic eosinophilic PNA
Desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-associated interstitial lung disease (RBAILD) are associated with what?
Smoking
DIP has large collections of which cells tpye in airspaces>
macrophages
What are in the macrophages in DIP?
dark brown (Fe) pigment
called smokers macrophages
What happens to the alveolar septa in DIP?
It thickens from inflammatory rxns
What are the Sx to DIP?
suddent onset dyspnea and dry cough, clubbing
What is the 100% effective Tx for DIP?
STOP SMOKING U IDIOT
steroids help tooooo
RBAILD has the presence of pigmented intraluminal macrophages where in the lung?
Respiratory bronchioles
What are the manifestations of RBAILD?
severe pulmonary Sx, abnrml pulm fxn, imaging abnormalitis, centriolobular emphysema
How do u improve the clinical course of RBAILD?
STOP. SMOKING. STOP IT.
STOP.
This is where there is bilateral patchy asymmateric pulmonary opacification and accumulation of acellular surfactant in intraalveolar and bronchiolar spaces.
Pulmonary Alveolar Proteinosis (PAP)
Are 90% of PAP cases acquired or genetic?
Acquired (autoimmune disorder)
What are the Sx to congential PAP?
immediate-onset neonatal respiratory distress, fatal within 3-6mo w/o a transplant
What is the morphology of the alveoli in PAP?
Homogenous, granular precipitate, large consolidation of the lungs.
What test is + for the alveolar precipitate in PAP?
PAS+
PAS for PAP
What is coughed up in adults with PAP?
large CHUNKS of gelatinous material
Is sarcoidosis related to smoking?
No
What are the 0-4 stages to classify sarcoidosis on CXR?
Stage 0- normal CXR Stage 1- hilar adenopathy Stage 2- hilar adenopathy w/ pulmonary infiltrates Stage 3- infiltrates w/o adenopathy Stage 4- fibrotic lung disease
Which elecrolyte is elevated in sarcoidosis?
Ca++
What is the first line therapy for sarcoidosis?
Steroids
What enzyme is elevated in sarcoidosis?
ACE
Which pts are at risk for lymphangioleiomyomatosis?
Women of childbearing age
What are the Sx to lymphangioleiomyomatosis?
Recurrent pneumothoraces, chylous pleural effusions, diffuse infiltrates with hypoxemia, and airflow obstruction
What are scattererd throughout the lungs in lymphangioleiomyomatosis?
Well-defined cysts
Which hormone worsens the disease in lymphangioleiomyomatosis?
Estrogen
What is the Tx for chronic hypersensitivity pneumonitis?
Corticosteroids
WHAT ORGANISM IS PATIENTS WITH SILICOSIS AT RISK FOR?
MYCOBACTERIUM TB
OMG LEARN THIS.
What are the 4 causes of ARDS?
Sepsis
PNA
Pancreatitis
Trauma
