Path 2nd half - W3 Flashcards
1
Q
How does idiopathic pulmonary fibrosis present?
A
- slowly progressive pulmonary fiborsis w/gradually worsening dypnea and non-productive cough
- older than 40
- males
- smokers
2
Q
What does CT of IPF show?
A
diffuse interstitial opacities
3
Q
Presents with old and young areas of fiborisis, honeycomb changes, and pathcy interstitial fibrosis
A
IPF (idiopathic pulmonary fibrosis)
4
Q
What is the likely cause of IPF?
A
- abnormal alvolear healing response to miscellaenous toxic/inurious stimuli (smoking)
- may be related to TBF-beta
- reduced telomerase activity –> more epithelial injury w/release of TGF-beta
5
Q
What is the clinical course of IPF?
A
relentlessy progressive (death within 5 years)
hypoxemia, cyanosis, digitial clubbing
cor pulmonale
heart failure (cause of death in 1/3 patients)
NO KNOWN EFFECTIVE THERAPY
6
Q
What is non-specific interstitial pneumonitis?
Histologically?
Common in?
A
- temporally uniform - cellular infiltrate of lymphocytes that progresses on to fibrosis
- BETTER PROGNOSIS
- occurs in patient’s w/autoimmune diseases
- more often in women and at younger age.
7
Q
How does NSIP present?
A
- more subacute/febrile illness
- same fibrotic distribution as IPF but no honeycomb changes
- responds to steroids - good prognosis.
8
Q
What is cryptogenic organizing pneumonia? Path? Symtpoms?
Tx?
A
- mimics community acquired pneumonia
- flu-like illness
- cough, malaise, fever, dyspnea on exertion
- PATH
- fibroblastic/granulation tissue plugs small airways and alveoli
- Treatment
- corticosteroids
9
Q
What is interstital lung disease associted with/what can it complicate?
A
- Rheumatoid arthriits
- Scleroderma
- polymyositis
- systemic lupus
- mixed connective tissue
- Sjogren’s syndrome
10
Q
What is ILD associated with from the environment?
A
-
Inhaled inorganic dusts/particles
- silicates (silicosis, asbestosis, berylliosis)
- Carbon dust (coal miners pneumoconiosis)
- Metals (tins, aluminum, hard metals)
-
Inhaled organic dusts
- hypersensitivity pneumonitis due to thermophili actinomyctes (famer’s get from hay)
- bacteria, true fungi (aspergillus), animal proteins (bird fancier’s lung)
-
Miscellaneous
- synthetic
- vinyl chlorides
- gases
11
Q
What causes silicosis? how does it present?
A
- inhalation of silica (silicon dioxide) - most abundant mineral on earth
- presents as chronic lung injury from long standing exposure (mining and sand blasting)
- progressive respiratory insuff. with multiple small fibrotic nodules in UPPER LUNG FILEDS.
- can cause massive fibrosis
12
Q
What does silicosis have increased risk for?
A
TB
13
Q
Can silicosis present acutely?
A
yes, if you have mssiave inhalation expsoure - get respiratory distress.
14
Q
What does silicosis histology show?
A
fibrocellular parenchymal nodules
15
Q
What is shown?
A
Silicosis
16
Q
What causes asbestos related lung disease? types?
A
- inhalation of natrually occurin mineral fibers (magnesium silicates)
- classically was in shipyard workers
- Types
- serpentine (less toxic)
- Amphibole (more toxic)
17
Q
How does asbestosis differ from silicosis?
A
- starts in LOWER LOBES
- may see asbestos bodies (get coated w/iron) – called ferruginous body.
18
Q
What are asbestosis patients at risk for?
A
- benign pleural disease - calified plaques, pleural effusions, visceral pleural fibrosis.
- malignant mesothelioma - delayed decades after expsoure - often always associated with asbestos.
- lung cancer risk - especially if smoker
19
Q
What is shown?
A
ferruginous body of asbestosis
20
Q
Is a biospy always done with asbesotis?
A
No, if exposure fits symptoms and chest imaging you don’t need one - see calcified pleural plaques.
21
Q
How does coal workers pneumoconiosis present?
A
-
blackening of lungs (anthracosis)
- get small nodules in lupper lung fields.
- 90% benign
- 10% have massive fibrosis - likely co-existent silica inhalation
22
Q
What causes hypersensitivity pneumonitis?
A
- immunologically mediated -mixed interstitila and alvolar
- antibody rxn to inhaled organ antigen
- many etiologies
- MUST REMOVE PATIENT FROM AGENT.
23
Q
how does hypersensitivity pneumonitis present?
A
- farmer’s lung
- pigeon breeder’s lung
- clinally
- acute attacks 4-6 hours after exposure
- fever
- dyspnea
- cough
- leukocytosis
24
Q
What is seen on CT w/ hypersensitivity pneumonitis?
A
mid/upper zone ground glass or nodular opacities
25
Treatment for hypersensitivity pneumonitis
Avoid exposure.
Course of corticosteroids w/more severe.
REVERSIBLE IF CAUGHT EARLY.
26
How is hypersensitivity pneumonitis diagnosed?
* CT
* flow cytometry
* see more CD8 than CD4 cells (lymphocytosis)
* inhalation history
27
**What is seen w/eosinophilic pneumonias?**
**Types\>**
* **pulonary infiltrates and alveolar or blood eosinophilia**
* Acute = febrile illness, **life thrreatening hypoxiab**
* **Chronic -** underlying asthma too.
* simple pulmonary eosinophilia - minimal symptoms, associated w/drugs, fungal.
Tx with corticosteroids.
28
Smoking related interstitial lung disease:
1. Idiopathic pulmonary fibrosis - SMOKERS
2. desquamative interstitial pneumonia and respiratory bronchiolitis-interstiital lung disease.
3. pulmonary langerhands cell histiocytosis.
29
What is seen in desquamative interstitial pneumonia and respiratory bronchiolitis-interstiital lung disease
* dyspnea, cough, hypoxemia, pulmonary infiltrates
* SMOKERS
* **pigmented macrphages in alveolar spaces**
* smoking cessation improves, but if they don't stop could lead to fibrosis
30
Presenation of pulmonary langerhans cell histiocytosis
* limited to smokers
* cough, dyspnea, chest pain, fever
* pneumothroax
* **diffuse pulmonary nodules** in UPPER ZONE CYSTS
* get proliferation of **langerhan's cells**
* smoking cessation improves in only 1/3 patinets
31
**Sarcoidosis**
* granulomatous disease that often invovles lung
* **cough**
* **dyspnea**
* **chest pain**
* **fatigue**
* **malaise**
* **weight loss**
* **other skin, joints, organs**
32
how old are most patinets with Sarcoidosis? Who is it more common in?
10-40 years old.
AFrican Americans - 2.4% risk.
33
What is seen on CXR with Sarcoidosis
**bilateral hilar adenopathy** - may see reticular pulmonary opacities in mid to upper zones.
progression = non caseating granulomas along lymphatics and involves alveoli.
34
What is the often diagnosis for Sarcoidosis
**biospy of lymph nodes -** see **granulomatous lymphadenitis.**
Increased CD4/CD8 ratio \> 4:1 - predicitive. Helps w/diagnosis.
may have hypercalcemia
35
What is shown
Sarcoidosis - lymph node w/non caseating granulomas
36
What is **pulmonary alveolar proteinosis (PAP)**?
* rare disorder - **diffuse lung disease w/filling of alveolar spaces by proteinaceous pink material**
* no associated inflammation or architectural issue.
* **surfactant related protein** - largely due to inability of pulmonary macrophages to metabolize it.
37
Clinical features of PAP
**progresive dyspnea**
**cough with gelatinous sputum**
fatigue
weight loss
fever
38
3 types of PAP
1. Acquired - adults - have serum autoantibody to granulocyte/monocyte CSF cuasing macrophage dysfunction.
2. Secondary - high level of DUST, heme maligancies, infection.
3. Congential - mutation in GM-CSF production.
39
Treatment for PAP
whole lung lavage - tx w/recombinant GM-CSF
40
What is **BAL** as a diagnostic tool. Use?
Use = cytology for malignant cells. Micro for clean culture.
Good for looking for fungal organisms.
41
what is seen on BAL with
PAP:
Sarcoid:
Hypersensitivity penumonitis:
cloudy milky fluid
CD4/CD8 ratio \> 3.5
CD4/CD8 decreased
42
What can cause a PE?
**bloot clot**
air
fat
amniotic fluid
tumor
43
What are the symptoms of a massive PE?
**systolic BP \< 90**
increased CVP - neck distention
Right heart failure --\> can lead to death.
44
Predisposing factors for PE:
Immobiliziation/post surgical
Pregnancy/contraceptive.
Obesity
Cardiac disease.
Cancer.
Anti phospholipid syndrome
heparin induced thrombocytopenia.
45
What can cause congenital hypercoagulability?
Factor V leidin
prothrombin gene mutation
Deficiencies of factor C or S
antithrombin III
46
What are some symptoms of PE?
Hypotension
Dyspena
Tachycardia
Pleuritic chest pain
calf/thig pain or swelling
signs of acute RV failure
47
How can you differentiate between pre and post mortem clot?
pre has lines of zahn
48
Does serum D-dimer have a high or low PPV for PE?
**high negative PV** - if norml.
normal = high PPV --- can rule out then. When you have a clot anywhere you can have d-dimer elevation.
49
What could cause chronic/recurrent small emboli?
pulmonary hypertension
cor pulmonale.
50
what do you see on path with pulonary hypertension
**medial hypertrophy** of arterioles and small arteries.
may have vasospastic component.
51
What is the mutation with inherited pulmonary hypertension
BMPR2 locus mutation - incomplete penetrance so there are environmental cofactors that trigger it.
52
What can be seen on CXR with pulmonary hypertension
normally none, otherwise increasing pulmonary vasculature
53
Clinical features of **pulmonary hypertension**
COPD, ILD underlying diseases
increased pulmonic compnent of 2nd heart sound
exertional dyspnea, lethargy, fatigue
RV hypertrophy w/RV failure/cor pulmonale.
54
what causes pulonary arteriovenous malformations
* hereditary hemorrhagic telangiectasis (osler-weber-rendu)
* presents in adulthood
55
Symptoms of pulonary arteriovenous malformations
**dyspnea**
**hypoxemia**
**hemotpysis**
**clubbing due to R to L shunt**
56
Common causes of hemoptysis - pulmonary hemorrhage
**bronchogenic carcioma**
**Bronchiectasis**
**Bronchitis**
**bacterial pneumonia**
**TB**
**PE**
**fungus ball**
**pulmonary abscess**
57
**Diffuse alveolar hemorhage (DAH) syndrome**
medical emergency
presents abrupt/rapdily evolving symtpoms: cough, hemotptysis, fever, dyspena, hypoxemia.
CT: diffuse alveolar opacities.
could casue anemia.
58
What can cause Diffuse alveolar hemorhage (DAH) syndrome
Drug/toxic injury - amidodarone.
ARDS/DAD
anticogaulation
mitral stenosis
pulmonay capillaritis
pulmonary infection/bone marrow transplant.
**cocaine use**
59
**Goodpasture syndrome diagnosis**
anit-GBM antibody - against glomerular and pulmonary alveolar basement membrane.
**linear IgG fluorescence**
60
What is seen on path with Goodpasture syndrome
**lungs - focal necrosis of alveolar walls w/alveolar hemorrhage**
**kidney: crescentic glomerulonephritis**
61
pulmonary capillaritis
**neutrophilic** inflammation of alveolar capillaries - commonest cause.
62
Wegeners granulomatosis presentaiton
manifests with sinusitis
pulmonary nodules w/alveolar hemorrhage
renal disease - rapidly progressive.
63
How is Wegener's dx?
Tx?
ANCA (c)
treat w/steroids + immunosuppression (cytoxan)
64
**SLE** diagnosis
Tx.
* Dx = ANA and anti-ds DNA.
* Tx = corticosteroids + immunsuppression.
* Sx = diffuse alveolar hemorrahge - 5% have pulmonary capillaritis
65
Who presents with idiopathic pulmonary hemosiderosis/hemorrhage
young patients/children
intermittient/recurrent
often iron dficiency anemia - macrophages w/hemosiderin
does respond to steroids
25% get autoimmune disease
66
What is the most common lung tumor?
**carcinoma**- 99%
most common visceral cancer in the world.
67
Risk factors for lung tumors (carcinoma)
Cigarette smoking (90%)
Radiation exposure.
Asbestos
Pulmonary fibrosis.
68
**non small cell carciomas:**
1. Adenocarcinoma - 40-50%
1. female dominant.
2. may not be related to smoking (small #)
2. Squamous carcioma. 20-30%
3. Large cell undifferentiated carcinoma 5-15%
4. miscellaenous
69
How is small cell carcinoma different from the others?
It's derived from neuroendocrine portion of the lung.
70
what is typical for sqamous cell on CXR
big mass, more central than peripheral.
CT - may show cavitation.
71
What drives treatment for non-small cell carcinoma (adenocarcinoma)
Histological class + ancillary molecular and biomarker testing to include EGFR, ALK, ROS and PDL-1
72
Well behaved lung maligancy =
**carcinoid tumor**
well differentiated neuroendocrine carcionoma.
73
What cancers of lung can go to salivary gland?
Mucoepidermoid
Adenoid cystic
74
Classic lung cancer presentation?
\> 40 years
long term smoker
cough
hemoptysis
**hoarsness** (tumor on laryngeal nerve or laryngeal carcionma)
dyspnea
pleural effusion/pericardial effusion
75
**central mass lesion =**
**peripheral mass lesion =**
* central
* squamous
* small cell
* peripheral
* adenocarcinoma
76
What causes pancoast tumor?
**chest wall expansion in APEX**
leads to Horner's syndrome - ptosis, constricted pupil, anhidrosis.
elevated diphgram (phrenic nerve paralysis)
77
What should you think with hoarsness in a smoker?
mass in medistinum
laryngeal cancer
78
Superior vena cava syndrome
could be caused by masses
see redness and swelling of face (especially when waking up in morning) and venous distention.
79
**Lepidic pattern of adenocarcinoma**
resembles:
fails to respond to:
resembles pneumonia.
fails to respond to pneumonia.
80
Where are lung cancers commonly metastases?
Liver.
Bone (pathological fracture may be first)
Adrenals.
Brain. (small cell carcinoma commonly) - seizure or focal neurological deficit may be first symptom.
81
What paraneoplastic syndromes can occur w/lung cancer
* tumor associated hormone compounds
* hypercalcemia due to bone metastases
* tumor production of PTH related to protein, calcitriol (Vitamin D)
82
Syndromes seen with small cell carcinoma:
* Inappropriate ADH secretion w/hyponatremia
* cushing's syndrome due to ACTH prodcution.
* Neurologic syndromes
* cerebellar ataxia.
* lambert eaton.
* Carcinoid syndrome: diarrhea, flushing, cyanosis
* see only after hepatic metastases
83
systemic effects of lung cancer;
* Heme - anemia, luekocytosis, hypercoag.
* MS: **clubbing, hypertrophic osteoarthropathy, inflammatory myopathy**
*
84
How is adenocarcionma treated (what drug)
EGFR inhibitors.
85
How do carcinoid tumors commonly present?
Treatment?
central/bronchial location w/obstruction, hemoptysis or peripheral nodule.
Usually resectable.
Typical type have good 10 year survival.
86
Most common benign tumor of the lung:
Hamartoma - disorganized tumor of the native tissue.
87
What is the most common site of metastatic neoplasia?
lUNG
88
how do metasases to lung present commonly?
**multiple, bilateral** lung nodules.
89
What could cause increased fluid production with nonifnlammatory (transudate)?
* increased hydrostatic pressure (CHF)
* nephrotic syndrome
* cirrhosis w/ascites
* peritoneal dialysis
90
Term for decreased lymphatic resorption
chylothorax
can occur due to interuptions to thoracic duct.
91
what defines exudate?
pleural fluid protein \> 2.9 gm
pleural fluid cholesterol \>45 mg
pleural fluid LDH
92
most common causes of exudative effusions
bacterial pneumonia
malignancy
viral
pulmonary embolism
93
What lab values are associated w/parapneumonic effusion/empyema
low pleural fluid glucose \<60mg
and pH \< 7.3
iMPLIES NEED FOR CHEST TUBE DRAINAGE.
94
HOW SHOULD you treat recurrent pleural effusions
pleural space obliteration w/talc pleurodesis
95
What causes 2' spontaenous pneumo
air leak from underlying lung disease:
emphysema, astham, CF, TB, pneumonia, neoplasia
96
How does a pneumothorax present?
Dsypnea w/chest pain
physical exam: diminished breath sounds, hyperresonant percussion.
Arterial blood gases - hypoexmia and hypercarbia.
97
How is pneumothorax treated?
* Tube thoracostomy w/water seal device.
* resportion occurs gradually in most patients.
persistent or recurrent
* stapling of blebs
* pleural space obliteration
98
List some primary pleural tumors
1. Solitary fibrous tumor - benign
1. **submesothelial fibrous tissue**
2. **large** - displace lung.
3. surgery cures.
2. **Malignant mesothelioma**
1. BIG BAD TUMOR OF PLEURA
2. 90% to asbestos exposure
3. latent for up to 45 yrs.
99
Symptoms and imaging of malignant mesothelioma
chest pain, dyspnea, bloody pleural effusion
**diffuse pleural thickening, effusion + calcified pleural plaques**
Histology: epithelio and or sarcomatous w/invasion of chest wall.
100
Most common congenital lung lesion
Congenital pulmonary airway malformation
101
List some neonatal lung diseases w/o congnetial malformation - **caused by dysfunctional transition from fetal life.**
* Transient tachypnea - late term infants - see in first 72 hours.
* Persistent pulmonary hypertension - term/late term
* failure to convert to postnatal circulation
* Respiratory distress syndrome - most common
* premature infants - more common in 36 weeks or less.
* hyaline membrane disease
102
What do you see with RDS (possible long term risk)
Bronchopulmonary dysplasia - have a lot of fibrosis and diffusion defect issues.
103
What 2 things are used to monitor fetal lung maturity?
1. **Lamellar body count**
1. counts small particles - low ocunts are sign of immaturity
2. want \> 50,000 .
2. **Phosphatidyl glycerol**
1. ****if negative = signficant RDS risk
2. high positive = no risk
