Restrictive lung disease + neoplasms

Question 1

What is the overarching umbrella of restrictive disease in lungs that is exposure, autoimmune, and idiopathic related w/ insidious onset of progressive dyspnea + nonproductive cough, tachypnea, small lung volumes, bibasilar dry rales, digital clubbing, RHF w/ advanced disease?

Answer 1

interstitial lung disease

Question 2

What does clubbing in the hands indicate?

Answer 2

years of hypoxia

Question 3

What would you expect in a restrictive lung disease’s PFT?

Answer 3

REDUCED lung volumes, low DLCO, high FEV1/FVC ratio

Question 4

What would you see on a CXR of interstitial lung disease?

Answer 4

low lung volumes + patchy distribution of ground glass, reticular, nodular, reticulonodular, or cystic opacities

Question 5

What’s the standard for diagnosis of diffuse lung disease?

Answer 5

surgical biopsy w/ 2-3 biopsies from multiple sites, usually in patients <60
and can consider bronchoalveolar lavage in cases of infection or malignancy

Question 6

What should you consider diagnosis in patients >65 w/:
- Idiopathic disease by history and who have inspiratory crackles
- PFT = restrictive
- Characteristic radiographic evidence of progressive fibrosis over several years
- AND diffuse patchy fibrosis w/ pleural-based honeycombing on high resolution CT scan

with symptoms of: DOE, cough, fatigue (gradual onset)?

Answer 6

idiopathic pulmonary fibrosis

Question 7

In who is idiopathic pulmonary fibrosis common?

Answer 7

men>50, smoking

Question 8

T/F: idiopathic pulm fibrosis patients need a lung biopsy to confirm diagnosis

Answer 8

F

Question 9

What would these diagnostic studies indicate?
CT = predominantly basilar, peripheral (bronchiectasis = upper) pattern of dilated bronchiectasis, reticulation, and early honeycombing

PFT: restrictive pattern w/ increased FEV1/FVC + decreased lung volumes

Answer 9

idiopathic pulmonary fibrosis

Question 10

How do you manage idiopathic pulmonary fibrosis?

Answer 10

pulmonologist

Nintedanib + pirfenidone (reduce lung function decline but do not improve survival, $$)
Oxygen, pulmonary rehab, monitoring pulm HTN

Only definitive treatment = lung transplant

Question 11

What causes sarcoidosis?

Answer 11

systemic granulomatous inflammation forming in multiple organs (we don’t know why) – lungs, lymph nodes, skin, eyes, nerves, liver, kidney, heart

Question 12

What could a patient with sarcoidosis come in complaining about?

Answer 12

malaise, night sweats, fever, dyspnea, cough, CP

Question 13

What is stage 1 of sarcoidosis?

Answer 13

bilateral hilar adenopathy alone

Question 14

What is stage 2 of sarcoidosis?

Answer 14

hilar adenopathy AND parenchymal involvement (diffuse reticular infiltrates, focal infiltrates, bronchiole changes, nodules, pleural effusion)

Question 15

What is stage 3 of sarcoidosis?

Answer 15

parenchymal involement alone

Question 16

What is stage 4 of sarcoidosis?

Answer 16

advanced fibrotic changes in upper lobes

Question 17

In who is sarcoidosis prominent?

Answer 17

30s or 40s onset, african americans

Question 18

What could this PE indicate:
- atypical
-crackles uncommon but skin involved w/ erythema nodosum, lupus pernio, iritis, peripheral neuropathy, arthritis, cardiomyopathy
- parotid gland enlargement, hepatosplenomegaly, lymphadenopathy

Answer 18

sarcoidosis

Question 19

How can you confirm diagnosis of sarcoidosis?

Answer 19

clinical findings + consistent XR (bilateral hilar + right paratracheal lymphadenopathy) and biopsy evidence of noncaseating granuloma from safest organ

BAL fluid = increase in lymphocytes + high CD4/CD8 ratio - monitoring and ophthalmologic evaluation

Question 20

What could this indicate:
Lymphopenia, elevated ESR, hypercalcemia, hypercalciuria, elevated LFTs, elevated ACE
PFTS = restrictive changes w/ decreased lung volumes + diffusing capacity (may show airflow obstruction)
ECG = conduction disturbances & dysrhythmias

Answer 20

sarcoidosis

Question 21

What are these indications for:

Disabling constitutional symptoms
Hypercalcemia
Iritis
Uveitis
Arthritis
CNS involvement
Cardiac involvement
Granulomatous hepatitis
Cutaneous lesions other than erythema nodosum
Progressive pulmonary lesions

Answer 21

sarcoidosis = oral steroids - prednisone w/ taper

Question 22

If someone who has sarcoidosis is intolerant of steroids what do you give instead?

Answer 22

methotrexate, azathioprine, infliximab

Question 23

What does long-term follow up look like for sarcoidosis?

Answer 23

yearly physical exam and PFTs, chemistry panel, ophthalmologic evaluation, CXR, ECG

Question 24

What should you think if a patient presents asymptomatic w/ slightly affected pulmonary function or progressive, massive fibrosis w/ conglomeration + contraction in upper lungs and is characteristic of Caplan syndrome and could be from the inhalation of inorganic dusts, smoking does not?

Answer 24

coal worker’s pneumoconiosis

Question 25

What would you expect on CXR and PFT for coal worker's pneumoconiosis?

Answer 25

CXR: formation of coal macules as diffuse small opacities in upper lungs w/ hyperinflation of lower lobes in an obstructive pattern (resembling emphysema) PFT: obstructive pattern

Question 26

What is treatment for coal worker's pneumonocosis, silicosis, asbestosis?

Answer 26

supportive

Question 27

What is usually caused by inhalation of silica particles that could be asymptomatic (simple) or large conglomerate densities in upper lung w/ dyspnea, restrictive AND obstructive pulmonary dysfunction and should be suspected in construction, mining, sand blasting, or quarry work?

Answer 27

silicosis

Question 28

What does this CXR indicate: scattered, small rounded opacities (silicotic nodules) + calcification of periphery of hilar lymph nodes “egg shells”

Answer 28

silicosis

Question 29

With silicosis patients, what should you consider with testing?

Answer 29

all patients should have a tuberculin skin test + current chest XR

Question 30

What can be caused by exposure to asbestos fibers over many years that can worsen with smoking and cause progressive dyspnea, inspiratory crackles, clubbing and cyanosis, and is common in shipyard and construction workers, pipe fitters, insulators of older buildings, ~15 years post exposure?

Answer 30

asbestosis

Question 31

What do these diagnostics indicate: CXR: nodular + linear streaking at lung bases, opacities of various shapes + sizes, advanced = honeycomb changes, pleural calcifications Shaggy heart sign = indistinct heart border, “ground glass” appearance CT scan is best imaging study to detect fibrosis + pleural plaques PFTs = restrictive dysfunction + reduced diffusing capacity (increased FEV1/FVC + decreased lung volumes)?

Answer 31

asbestosis

Question 32

In who is hypersensitivity pneumonitis most common?

Answer 32

30s-50s

Question 33

What is this indicative of: Acute - fever, chills, malaise, 4-8 hours post prolonged exposure, inspiratory crackles Subacute - gradual development of dyspnea, productive cough, usually NO fever/chills Chronic - progressive worsening and is inflammation from organic antigens?

Answer 33

hypersensitivity pneumonitis

Question 34

How do you treat hypersensitivity pneumonitis?

Answer 34

Can be reversal in case of prompt diagnosis + remove offending agent Oral steroids

Question 35

What does this characterize: Profound dyspnea, cyanosis, tachypnea, intercostal retractions, crackles that can be from various diseased states and is #1 caused from sepsis?

Answer 35

ARDS

Question 36

ARDS levels

Answer 36

Mild: paO2/FlO2 ratio = 200-300 Moderate: paO2/FlO2 ratio = 100-200 Severe: paO2/FlO2 ratio = <100

Question 37

What is required for an ARDS diagnosis?

Answer 37

~ 7 days of trigger (but could be up to 2 hours after trigger) Bilateral pulmonary opacities (within alveoli) NON cardiogenic Impaired gas exchange 3 inclusion, 1 exclusion criteria

Question 38

What would you see on CXR for ARDS?

Answer 38

CXR: diffuse patchy infiltrates that proceed rapidly and become confluent Air bronchograms, normal heart size

Question 39

How do you treat ARDS?

Answer 39

Mechanical ventilation w/ PEEP (lowest levels + O2 required) Prone positioning Central venous catheter Fluid management Severe - ECMO

Question 40

What could this indicate: Anorexia, weight loss, or asthenia, new cough, hemoptysis, pain in chest or bone Neurological = brain mets Lymphadenopathy >1 cm Pneumonia, pleural effusion, change in voice (laryngeal nerve), SVC syndrome, Horner syndrome (ipsilateral ptosis miosis, and anhidrosis), brain metastases (headache, nausea, vomiting, seizures, dizziness, or AMS)?

Answer 40

lung cancer, more common NSCLC

Question 41

How can small cell present a little bit differently in lung cancer (more aggressive, but less common)?

Answer 41

SIADH, hypercalcemia, digital clubbing, anemia, peripheral neuropathy

Question 42

What are the risk factors for lung cancer?

Answer 42

~70, unusual <40, smoking = major risk factor cigar, pipe smoking, secondhand smoke, air pollution, alcohol, low fiber diet, inheritance

Question 43

What are the types of NSCLC (MCC)?

Answer 43

Adenocarcinomas - most common, from any epithelial cell within or distal to terminal bronchioles Squamous cell - from bronchial epithelium + intraluminal masses Large cell - aggressive + rapid doubling, central or peripheral masses (staging: T,N,M)

Question 44

Adenocarcinomas are ________ located

Answer 44

peripherally

Question 45

Squamous cell CCCP

Answer 45

centrally located cavitary lesions hypercalcemia Pancoast syndrome

Question 46

SCLC is most associated w/

Answer 46

paraneoplastic syndromes, central, small dark blue cells w/ Rosette formation

Question 47

Staging: T,N,M or limited/extensive Regional or distant metastasis on presentation MYC gene mutation

Answer 47

SCLC

Question 48

What do these diagnostics indicate PE: wheeze/stridor, dyspnea Hct <40%m, <35%w abnormal findings on CXR or CT?

Answer 48

lung cancer

Question 49

What other Dx can indicate lung cancer?

Answer 49

sputum cytology (specific but insensitive) CT-guided biopsy of peripheral nodules (risk of pneumothorax) Thoracentesis (malignant pleural effusions) Fine needle aspiration

Question 50

What is recommended screening for lung cancer?

Answer 50

Recommended screening with low-dose chest CT in high-risk individuals - 50-80 years who have no symptoms of lung cancer + 20 PPY smoking history who currently smoke OR have quit within 15 years

Question 51

What is staging for bone cancer?

Answer 51

Bone scan for STAGING – TNM staging system T - size + location of primary tumor N - presence + location of nodal metastases M - presence or absence of distant metastases

Question 52

What are the stages of cancer?

Answer 52

Stage I = cancer confined to lung tissue w/ no involved lymph nodes Stage II = confined to lung tissue w/ involved surrounding lymph nodes or within chest cavity Stage III = confined to lung tissue or involving adjacent structures + lymph nodes in mediastinum Stage IV = spread to opposite lung or any organ and disseminated

Question 53

What is the treatment of NSCLC

Answer 53

Surgical resection No surgery in risky situations Chemotherapy Radiotherapy

Question 54

What is the treatment for SCLC

Answer 54

Highly aggressive → chemotherapy (cisplatin + etoposide) Palliative care - Yag laser, radiation, brain resection, thoracentesis, pain control

Question 55

What separates a benign pulmonary nodule from a malignant one?

Answer 55

Smooth, defined edge = benign Ill defined margins, speculated, lobular = malignancy

Question 56

Pulmonary nodules are ___ in <30

Answer 56

rare, increasing w/ age, smokers

Question 57

What are benign pulmonary nodules?

Answer 57

infectious granulomas

Question 58

What is the diagnosis steps for pulmonary nodules?

Answer 58

CXR --> chest CT for any suspicious nodule --> assess risk if concerning, transthoracic needle aspiration/bronchoscopy, PET scan, sputum cytology

Question 59

How would you treat a pulmonary nodule?

Answer 59

Biopsy vs. excision Video-assisted surgery (VATS) resection of all solitary pulmonary nodules w/ intermediate probability of malignancy

Question 60

What are some concerning signs of a mediastinal mass?

Answer 60

Insidious onset of retrosternal CP, dysphagia, dyspnea

Question 61

What are middle mediastinal masses?

Answer 61

lymphadenopathy, pulmonary artery enlargement, aneurysm of aorta, developmental cyst, dilated azygos vein

Question 62

What are anterior mediastinal masses?

Answer 62

thymoma, teratoma, thyroid lesions, lymphoma, mesenchymal tumors (lipoma, fibroma)

Question 63

What are posterior mediastinal masses?

Answer 63

hiatal hernia, neurogenic tumor, meningocele, esophageal tumor, thoracic spine disease, extramedullary hematopoiesis

Question 64

What are diagnostic tests for mediastinal masses?

Answer 64

CT scanning Barium swallow if esophageal disease is suspected Doppler sonography or venography of brachiocephalic veins + SVC + angiography MRI **tissue diagnosis is necessary if a neoplastic disorder is suspected**

Question 65

Adenocarcinoma is one of the only lung cancers that have no association with ____

Answer 65

smoking -- may be more correlated with genetics or family history