CMS Final: Pulm pt2

which disease has a hallmark of honeycombing on imaging?

interstitial lung disease (IPF) → also shows ground-glass appearance and reticular infiltrates

what is found on physical exam in interstitial lung dz?

velcro rales, clubbing, prominent P2

what diagnostic test is used for iPF?

6 minute walk test → screens for hypoxic resp. failure

what is the definitive treatment for IPF?

lung transplant

what is the MC cause of ILD?

idiopathic (no specific cause)

what are common s/sx of ILD?

gradual onset dyspnea (MC), dry cough, pleuritic chest pain, hemoptysis

what do PFTs look like in ILD?

decreased lung volumes
reduced TLC
reduced FRC
reduced RV

what leads to decreased gas exchange in ILD?

irreversible fibrosis/scarring

what is the hallmark of sarcoidosis?

noncaseating granulomas

what diagnosis shows bilateral hilar LAD on imaging?

sarcoidosis

what are the pulmonary symptoms of sarcoidosis?

- persistent dry cough
- fatigue, fever, malaise
- SOB
- laryngeal sarcoid → dysphagia and hoarseness

what are the cutaneous manifestations of sarcoidosis?

- lupus pernio (nares)
- erythema nodosum

What is Lofgren's syndrome?

type of acute sarcoidosis associated with triad of:
- bilateral hilar lymphadenopathy
- erythema nodosum
- polyarthritis

what is the first line of treatment in stage 2-3 sarcoidosis?

glucocorticoids

what diagnosis shows ferruginous bodies on histo?

asbestosis

what is the most common cause of asbestosis?

general community exposure (road surfaces, landfills, playgrouds, paint)

what does a CXR for asbestosis show?

linear opacites in LOWER lung

which diagnosis shows diffuse pleural thickening and calcified plaques on CT?

asbestosis

which diagnosis is caused by inhalation of crystalline silicon dioxide?

silicosis

which diagnosis shows egg shell calcifications on CXR? where is this located?

silicosis → well-defined opacities in posterior portion of UPPER lobe

which diagnosis has large, amorphous black masses with cavitation resulting from ischemic necrosis?

coal workers pneumoconiosis

a coal worker with rheumatoid arthritis presents with pulmonary symptoms. Imaging shows rheumatoid nodules with layer of black dust around a central necrotic area. what is the diagnosis?

caplan syndrome

what is virchow's triad and what disease is it associated with?

virchow's triad:
1. hypercoagulability
2. venous stasis
3. endothelial injury

associated with pulmonary embolism

what are risk factors for pulmonary embolism?

factor V leiden deficiency, protein C/S deficiency, immobilization, recent ortho surgery, cig smoking, HRT

what does pulmonary HTN lead to?

RVHF

what is the MC cause of pulm embolism?

LE DVT

a patient presents with sudden onset severe dyspnea, pleuritic chest pain, cough. EKG shows sinus tachycardia. CXR shows Hampton's hump. what is the diagnosis?

pulmonary embolism

if a pregnant patient presents with symptoms consistent with pulm embolism, what diagnostic test is appropriate?

V/Q scan

which diagnosis has ABGs that show: resp alkalosis, hypoxemia, and hypocapnia?

pulmonary embolism

a patient who recently underwent a knee replacement presents with worsening dyspnea and pleuritic chest pain. CXR shows westermark's sign. Their BP is 96/78. what is the appropriate treatment?

unstable pulm embolism → thrombolytics. HOWEVER, this patient just had surgery which is an absolute contraindication for thrombolytics, treatment should be: surgical embolectomy, PCI, ECMO

what is the treatment for stable patient with pulm embolism?

heparin

what is the treatment for stable pulm embolism if anticoags are contraindicated?

IVC filter

What does S1Q3T3 on EKG indicate?

pulm embolism

what is the gold standard for diagnosis of pulm HTN?

right heart catheterization

What is Ortner's syndrome and what is it associated with?

hoarseness → assoc. with pulm HTN

what are the groups of pulmonary HTN?

• 1: idiopathic
• 2: Left HD
• 3: Lung disease
• 4: Pulmonary emboli
• 5: Everything else

pulm artery HTN pathophys refresh

molecular/genetic changes → hypertrophy of smooth muscle, endothelial cells, and adventitia → restricted flow through pulm arteries → increased vasc. resistance → RV increases filling and SV →increased pulm artery pressure → can lead to RSHF

what is the treatment for low risk pulmonary HTN?

ERA (ambirsentan, bosentan, macitentan) + PDE5 inhibitor (sildenafil, tadalafil)

what is the treatment for high risk pulmonary HTN?

triple combo: ERA + PDE5i + prostacyclin analog (epoprostenol)

a patient presents with worsening dyspnea that begins with exertion, blue lips, dull chest pain, and dry cough. CXR shows cardiomegaly and RVH. what is the likely diagnosis?

pulm HTN (would need tte and right heart cath but that would've given it away :D)

what diagnosis is an alteration in structure and function of the RV caused by disease of the lung or pulm. vasculature?

cor pulmonale

what is the MC acute cause of cor pulmonale?

massive PE

cor pulmonale pathophys refresher

increased pul artery pressure → increased RV afterload → RV dilation and increased RV EDP

right volume overload → septal displacement and decreased LV filling and CO

what are some physical exam findings in cor pulmonale?

RSHF sx:
- JVD
- cyanosis
- hepatomegaly
- ascites
- tachypnea
- palpable left parasternal lift
- loud S2
- TR

what is the goal of cor pulmonale treatment?

decrease pulm vasoconstriction and increase RV contractility:

- O2 therapy: relieves vasoconstriction
- diuretics: decrease RV filling
- CCBs: decrease pulm pressure
- pulm vasodilators: support fxn by decreasing TPR

what is seen on CXR of cor pulmonale?

enlarged pulm artery and cardiomegaly

what is neonatal respiratory distress syndrome (RDS) also known as?

hyaline membrane dz (this is how they tested us on the pulm exam)!

what disease is caused by alveolar surfactant deficiency or inactivation?

RDS

risk factors of premature birth weight and low birth weight are associated with what?

RDS

what is RDS heavily associated with?

atelectasis

what is used to prevent RDS?

treating the pregnant mother with antenatal steroids: betamethasone and dexamethasone 1-7 days before birth

a premature newborn patient presents with nasal flaring, cyanosis, decreased breath sounds and tachypnea. CXR shows air bronchogram sign. what is the appropriate treatment?

RDS → O2 supp (nCPAP or NIPPV) and exogenous surfactant within 2 hrs

a patient is brought into the ER after a severe MVA with acute profound dyspnea. PE shows labored breathing, cyanosis, and diffuse crackles throughout the lungs. ABGs show paO2/FiO2 < 300. what do you expect to find on CXR?

ARDS → bilateral lung infiltrates "white out" that is not uniform

what are risk factors for ARDS?

infection/injury/surgery, aspiration/sepsis, drowning/OD

ARDS pathophys refresh

1. alveolar injury
2. cytokine release
3. endothelium damage
4. protein leak in interstitium
5. pneumocyte death
6. no surfactant → atelectasis

Which phase of ARDS is this:

1-10 days: protein rich fluid, blood and inflam cells in air spaces → alveolar edema → dec. gas exchange → hypoxemia

exudative

Which phase of ARDS is this:

8-21 days: absorption of intra-alveolar fluid and increased functional recovery

proliferative

Which phase of ARDS is this:

>21 days: interstitial and vascular fibrosis. increased mortality

fibrotic

if a patient appears critically ill following an inciting event with profound dyspnea, what should be on your differential?

ARDS

what is associated with improved mortality for ARDS?

prone positioning

which bronchus is FB aspiration more common?

right main bronchus

what is the mainstay treatment for FB aspiration?

bronchoscopy

what diagnosis is defined by repetitive collapse of the upper airway during sleep?

obstructive sleep apnea

what is the gold standard for diagnosis of OSA?

polysomnography (sleep study)

what do the ABGs look like in OSA?

- high bicarb (renal compensation bc chronic dz)
- high CO2
- low O2

what is the first line tx for OSA?

CPAP

what is an awake alveolar hypoventilation in an obese person not due to underlying pathology?

obesity hypoventilation syndrome (OHS)

a 73 year old male patient presents with complaints of morning headaches, sleepiness, and loud snoring. his BMI is 35. what is the likely diagnosis?

OSA

a patient presents with complaints of drowsiness and declining concentration and memory. they have a BMI of 32. what is the cause of their symptoms?

decreased nocturnal CO2 clearance d/t OHS → CPAP

what imaging study should be done on any patient with suspected lung cancer?

CT with contrast

What is Horner's syndrome?

partial ptosis
miosis
anhidrosis

indicates suspected lung cancer

how is SCLC staged?

limited and extensive

How is NSCLC staged?

TNM

what is the treatment regimen for limited SCLC?

chemo + radiation

what is the treatment regimen for extensive SCLC?

immunotherapy and chemo

Where is SCLC located in the lung?

central location

where is NSCLC located in the lung?

peripheral location

what is the MC type of NSCLC?

adenocarcinoma

what is the MC type of lung cancer in non-smokers?

adenocarcinoma

what is the treatment for stage 1 and 2 NSCLC?

surgical resection

add chemo for stage 2

what is the treatment for stage 3 NSCLC?

chemo + radiation

what is the treatment for stage 4 NSCLC?

palliative

where is SCC (NSCLC) commonly found?

central lung or main airways

a 63 year old patient presents with cough, hemoptysis, hoarse voice, and chest pain. CXR shows a pancoast tumor. what type of lung cancer does this patient have and what abnormal lab finding do you expect to see?

NSCLC → SCC!!! hypercalcemia

which type of lung cancer has histology showing sheets of highly atypical cells with focal necrosis without keratinization or gland formation?

NSCLC - large cell

Where do carcinoid tumors usually originate?

GI

what diagnosis has a triad of flushing, diarrhea, and bronchospasms?

carcinoid syndrome

which type of tumor is associated with horner's syndrome?

pancoast tumor

why do pancoast tumors cause ipsilateral shoulder/arm pain, thenar muscle atrophy, and paresthesias of 4/5th digits and medial forearm?

they involve the brachial plexus

for which type of lung tumor is multiple endocrine neoplasia type 1 (MEN-1) syndrome a risk factor?

bronchial carcinoid tumor

which type of lung tumor originates from kulchitsky cells and secretes serotonin, ACTH, somatostatin, and bradykinin?

bronchial carcinoid tumor

what screening is necessary for lung cancer?

annual low-dose CT in 50-80 year olds with 20 pack year history, current smokers, or ex-smokers who quit within 15 years

for which diagnosis is a CT without contrast the 1st line for monitoring?

solitary pulmonary nodules

which lung findings is indicated by popcorn calcifications on CXR?

hamartoma → MC benign lung lesion

what are rare, systemic clinical manifestations from underlying malignancy?

paraneoplastic syndrome: SCD, LEMS, SIADH, autonomic neuropathy

a patient with hx of SCLC presents with n/v, dizziness, dysphagia, and ataxia. what paraneoplastic syndrome do they most likely have?

subacute cerebellar degeneration (SCD)

a patient with hx of SCLC presents with proximal muscle weakness throughout the thigh and pelvis. which paraneoplastic syndrome are they most likely to have?

lamber-eaton myasthenic syndrome (LEMS) → impaired voltage gated Ca++ channels d/t autoantibodies on NMJ

a patient with SCLC presents with nausea and fatigue. labs show hyponatremia, hypoosmolality. what is the likely paraneoplastic syndrome?

SIADH

a patient with SCLC presents with dryness, altered pupils, and bladder/bowel dysfunction. they also report episodes of orthostatic hypotension. what is the dx?

autonomic neuropathy

what medication can be used to treat carcinoid symptoms?

somatostatin analogs