Pulmonary 3

Question 1

Hypersensitivity Pneumonitis - “extrinsic allergic alveolitis”:

Answer 1

Allergic response after inhalation of organic dusts, or simple chemicals in sensitized patient, leading to granulomatous inflammation of the alveolar epithelium - Delayed reaction 4-6 hours after exposure.

Clues: recurrent pneumonia, new home/school, contact with birds, water damage, swimming, sxs get better on vacation.

Etiology: inhaled organic particulates - fibers (cotton, flax) bagasse (sugar cane), hemp, coffee, animal dander, mold, hay, maple bark, saw dust, flour brewer’s yeast, mites, compost, detergent, paints/resins.

Sxs: often nonspecific, chronic cough and SOB or a history or recurrent episodes/exacerbations of acute respiratory symptoms without definite infectious triggers.

Question 2

Acute hypersensitivity pneumonitis

Answer 2

acute onset, usually within 4-6 hours after exposure. Fevers, chills, dry cough, dyspnea, chest tightness, malaise, headache

PE: ill appearance, tachypnea, crackles, often NO wheezing resolves within 12 hours to days after the antigenic exposure is eliminated.

Question 3

Subacute hypersensitivity pneumonitis

Answer 3

Gradual onset, less severe and lasts longer. Cough (productive), dyspnea, fatigue, anorexia, weight loss

PE: ill appearance, tachypnea, crackles

Question 4

Chronic hypersensitivity pneumonitis

Answer 4

insidious onset, cough, progressive dyspnea, fatigue, anorexia, weight loss, and exercise intolerance

PE: crackles, possible digital clubbing and an inspiratory squawk in some patients.

Question 5

Hypersensitivity pneumonitis workup, imaging, and complications

Answer 5

Work-up: CBC, allergy testing, PFT, BAL shows lymphocytosis
Lung biopsy

Imaging: Radiographic studies may show irreversible pulmonary fibrosis.

Acute: diffuse interstitial micronodular “ground glass” opacities
Subacute: micronodular or reticular opacities
Chronic: loss of lung volume, alveolar destruction “honey combing”
High resolution CT scan, ground glass opacities.

Complications: permanent lung damage with pulmonary fibrosis, sub pleural blebs may rupture, leading to spontaneous pneumothorax. Cor pulmonale or premature death.

Question 6

Eosinophilic Pulmonary Disorders - Definition

Answer 6

Accumulation of eosinophils in lung interstitium (alveoli possible) - considered allergic response to parasite, drug (antibiotics, phenytoin, L-tryptophan), inhaled toxins.

Question 7

Acute Eosinophilic Pneumonia

Answer 7

Does not recur, rapid eosinophilic infiltration of lung interstitium.

Sx:

Question 8

Chronic Eosinophilic pneumonia

Answer 8

may be recurrent; abnormal chronic accumulation of eosinophils in lung interstitium.

Sx: fever, weight loss, fatigue, dyspnea, dry cough, wheezing, chest discomfort

Note: clinical picture may lead to misdiagnosis of community-acquired pneumonia

Work up: CBC, increased ESR, CXR shows opacities in mid/upper lobes.

Question 9

Idiopathic Interstitial Pneumonias

Answer 9

Interstitial lung diseases with unknown etiologies, but very common in smokers. Present similarily, suspect on history. Leads to restrictive lung changes, seen on CXR

History: family history, tobacco use, drug use, home and work environment

Sx: cough, dyspnea, tachypnea, reduced chest expansion, bibasilar crackles

Dx: CXP or CT, PFTs, lung biopsy

Question 10

Idiopathic pulmonary fibrosis

Answer 10

most common, male smokers, gradual onset, poor prognosis

Question 11

Nonspecific interstitial pneumonia

Question 12

Cryptogenic organizing pneumonia

Answer 12

M=F, community-acquired pneumonia like syndrome, progressive dyspnea

Question 13

Respiratory Bronchiolitis-related ILD

Answer 13

M:F 2:1, heavy smokers; metaplastic cuboid epithelium in bronchioles/alvoli

Question 14

Desquamative interstitial pneumonia

Answer 14

> 30 yo smokers, pigmented macrophages in distal airways

Question 15

Acute interstitial pneumonia

Answer 15

healthy men and women >40; abrupt fever, cough, dyspnea. Possible resp. failure

Question 16

Drug-Induced ILD

Answer 16

many drugs/drug categories have direct toxic pulmonary effects leading to: respiratory symptoms , CXR changes, decreased respiratory function.
Examples: antibiotics, chemotherapy, anti-arrythmics, statins, illicit drugs, anticoagulants.
Diagnosis based on response to withdrawal of the suspected drug.

Question 17

Environmental causes of ILD

Answer 17

Group of diseases causing replacement of normal lung tissue by abnormal tissue. Restrictive pulmonary changes. Get clear and complete occupational/exposure history.

Sx: insidious onset of dyspnea, exercise limitation, dry cough

PE: mid to late inspiratory crackles, tachypnea; late findings: cyanosis, pulmonary hypertension leading to cor pulmonale.

Workup: CXR shows patchy, sub pleural, bibasilar interstitial infiltrates, cystic radiolucencies, “honeycombing”

Question 18

Pneumoconiosis

Answer 18

Caused by the inhalation of inorganic mineral dusts

Question 19

Asbestosis

Answer 19

inhalation of asbestos fibers. Source: mining, milling, manufacture - insulation leads to pulmonary fibrosis - dose dependent, pleural thickening.

Can lead to: bronchogenic carcinoma (10x > risk in non-smokers; 60-90x in smokers)

Malignant pleural mesothelioma - seen on CXR and staged with chest CT

Sx: insidious onset of dyspnea; may also have coughing and wheezing.

Question 20

Silicosis

Answer 20

Inhalation of silica particles - source: mining, pottery, sand-blasting, brick-making, foundries, glass makers

Occurs 5-20 years after 1st exposure, worse in smokers

Sx: dry cough, dyspnea, tachypnea, later weight loss, hemoptysis

Imaging: CXR shows >1 cm nodules in upper lobes - eggshell calcification of hillier nodes

DDX: COPD, lung cancer

Question 21

Anthracosis

Answer 21

“Black lung” >15 years exposure, worse in smokers

Sx: may be no resp. sxs, productive cough possible

More severe state leads to progressive massive fibrosis

Question 22

Berylliosis

Answer 22

From mineral beryllium dust
Source: older fluorescent light bulbs, ceramics, electronics, aerospace industry

Sx: dyspnea, cough, weight loss

Question 23

Irritant Gas Inhalation Injury

Answer 23

Inhaled gases dissolve in respiratory tract fluids, release acidic or alkaline radicals which cause inflammation in trachea, bronchi, bronchioles, alveoli and into interstitium.
May be from industrial accidents, mixing household ammonia with bleach
Directly toxic agents: cyanide, carbon monoxide

Sx: depends on extent and duration of exposure. Severe burning of eyes, nose, trachea, bronchi with cough, hemoptysis, wheezing, retching, dyspnea.

May lead to ARDS or bronchiolitis obliterans (granulation tissue accumulates in bronchioles and alveolar ducts).

Question 24

Air pollution related illness

Answer 24

Airway hypersensitivity to pollutants: oxides of nitrogen and sulfur, ozone, carbon monoxide, lead, volatile organic compounds, chlorofluro carbons, particulates.

Triggers exacerbation’s in asthmatics, COPD

Most vulnerable: elderly, kids, those with underlying lung disease

Sx: airway inflammation,
bronchoconstriction, may be permanent decrease in lung function.

Question 25

Pulmonary Vasculitides (vasculitis)

Answer 25

Inflammatory leukocytes in pulmonary and other blood vessel walls with reactive damage to mural structures, leading to bleeding hemoptysis, ischemia and necrosis

Question 26

Wegener’s granulomatosis

Answer 26

Autoimmune condition that affects lung, nose, kidneys. Pulmonary infiltrates, rhinosinusistis, alveolar hemorrhage, glomerulonephritis

Sx: cough, dyspnea, hemoptysis, pleuritic pain

Lab: hematuria, proteinuria

Question 27

Churg-Strauss Syndrome

Answer 27

Allergic ganulomatosis and angitis - allergic rhinitis, asthma, alveolar hemorrhage.

Question 28

Goodpasture’s Syndrome - Connective tissue disorder with pulomary manifestations

Answer 28

pulmonary hemorrhage with severe and progressive glomerulonephritis. Often in young men, present with severe hemptysis with secondary iron deficiency, dyspnea and rapidly progressive renal failure

Question 29

Rheumatoid Lung Disease

Answer 29

associated with RA. Autoimmune disease of joints (pain, stiffness, deformity), skin (nodules), lungs, kidneys
Usually in a patient with sero-positive RF

Pulmonary sx: pleuritic chest pain

CXR shows pleural effusion, nodules in lungs, interstitial fibrosis, vasculitis

Question 30

Lupus (SLE)

Answer 30

Autoimmune disease of blood, heart, joints, skin, lungs, liver, kidneys

Pulmonary sx: pleuritic chest pain, cough, dyspnea, recurrent URI, hemoptysis

CXR: decreased lung volume

Question 31

Pulmonary Amyloidosis

Answer 31

Rare. Amyloid protein deposition in lung (commonly in heart, spleen, intestine, kidney)

Unknown cause

3 main pulmonary types: tracheobronchial, nodular pulmonary, alveolar septal

Sx: (chronic and mild) fever, dyspnea, cough, hemoptysis

CXR shows multiple pulmonary nodular opacities, low density, irregular contours
Biopsy will confirm.

Question 32

Sarcoidosis

Answer 32

Chronic disease of unknown cause that affects multiple systems, characterized by non-caseating granulomas, (nodules with macrophages) leading to inflammation of the involved tissues (in genetically susceptible people).

Lungs are usually the first place to be affected, may lead to pulmonary fibrosis.
Incidence: found worldwide, in all races and both sexes.
Age: most common 20-40yo
Sex: F>M

Sx: vary depending on the are involved, and may be mild, moderate, severe.
First sx are usually vague: fever, weight loss, or joint pain, SOB, persistent cough.
Other sxs:
skin: erythema nodosum. Eyes: conjunctivitis, tearing, blurry vision, and photophobia, rarely blindness.
Also affects brain, nerves, heart, liver, and endocrine system.
PE: Crackles on inspiration

Question 33

Sarcoidosis Labs/DDx

Answer 33

Labs: PFTs, CBC, CMP, LFTs
Imaging: CXR, CT, bronchoscopy
Dx: biopsy of granuloma during bronchoscopy. 90% of cases, CXR shows non-caseating granuloma, hillier adenopathy. CBC reveals anemia, eosinophilia, leukopenia
PFTs in advanced disease

Course: majority of pts. (2/3) is appears and disappears. 20-30% have some permanent fibrosis. Death can occur is spreads to vital organs.

DDx: TB, Aspergillosis, histoplasmosis, RA, lymphomas, Wegener’s granulomatosis, hypersensitivity pneumonitis.

Question 34

Lung Cancer Rates

Answer 34

Males: #1 Prostate, #2 Lung, Mortality ~30%
Females: #1 Breast, #2 Lung, Mortality ~25%

Question 35

Tumor of the lung DDx

Answer 35

Seen on CXR

• Carcinoma (up to 50% in >50yo)
• Benign tumor
• Tuberculosis granulomas
• Histoplasmosis (yeast in alveoli)
• Coccidioidomycosis (nodular infiltrates)

Question 36

Types of Lung Cancer

Answer 36

Small cell (airways)

Non-small cell: Adenocarcinoma, SCC (airways), Large cell carcinoma

Question 37

Lung cancer etiology

Answer 37

• Smoking - risk is 13.3 times greater than non-smoker
• Second-hand smoke (~15%)
• Asbestos exposure - tobacco smoke + asbestos exposure = risk 80-90 times greater
• Radon exposure
• Other environmental agents
• Genetics

7-10% are asymptomatic, advanced disease - unintended weight loss, respiratory distress.

Question 38

Primary tumor

Answer 38

Central tumors are usually SCC.
Sx: cough, dyspnea, wheezing, hemoptysis, atelectasis

Peripheral tumors are usually adenocarcinomas or LCC.
Sx: cough, dyspnea, and symptoms of pleural effusion (pleuritic chest pain)

As tumor spreads and grows, downstream there may be problems presenting. Apex tumors can block superior vena cava. Paralysis of laryngeal or phrenic nerve changing voice or diaphragm function. Pressure on sympathetic plexus can cause Horner’s syndrome. Dysphagia resulting from esophageal compression. Pericardial effusion (pancoast tumor)

SCC - associated with hypercalcemia from parathyroid-like hormone production.

Adenocarcinomas - clubbing and Trousseau syndrome (hypercoagulability and venous thrombosis)

Question 39

Metastasis of lung cancer

Answer 39

From lung to (primary): liver, brain, bone

To lung from (secondary): Breast, stomach, pancreas, colon, thyroid, prostate, kidney, cervix, etc

Question 40

Lung Cancer: PE, Dx, Prognosis

Answer 40

PE: may find local wheezing over tumor
decreased breath sounds
dullness to percussion with large tumor
enlargement of axillary and supraclavicular nodes hepatomegaly.

Dx: CXR shows opaque mass
PET scan +IV imaging agent
CT scan of the lungs, sometimes with transthoracic needle aspiration biopsy.
Cytology of sputum or pleural fluid
chem panel: high serum calcium
Bronchoscopy - guided biopsy for intralumenal cancer (SCC)

Prognosis: Based on type, staging and grading. Overall prognosis is poor, often because of advanced stage at dx.

1-5 Staging: tumor size, spread, node involvement (seen on CT)
Grading: Amount of cell differentiation (seen on BX), use gleason score.

Question 41

ARDS - Acute Respiratory Distress Syndrome

Answer 41

Sudden life threatening respiratory failure from inflamed alveoli, causing them to fill with fluid and collapse, gas exchange ceases, and the body becomes hypoxic.

Etiology: variety of acute processes that directly or indirectly damage lung tissue. Conditions that predispose: Sepsis, pneumonia, inhalant injuries, drug overdose, pancreatitis, trauma, emboli, shock, burns, head injury, uremia.

Signs & Sxs: usually develops within 72 hours of initial injury/illness, then acute onset of urgent distress: dyspnea, tachypnea, pulmonary hypertension.

PE: labored breathing and tachypnea, cyanosis and moist skin, scattered crackles, tachycardia, agitation then lethargy

Labs: arterial blood gases
Imaging: CXR shows abnormal bilateral diffuse infiltrates

Dx: presence of fluid in the alveolar spaces of both lungs.

Tx: mechanical ventilation

Complications: Shock, multiple organ failure

Prognosis: high mortality rate. Survival is possible if treated correctly.

Question 42

Atelectasis

Answer 42

Collapse of alveoli (airless area) leading to reduced or absent gas exchange. Due to blockage or compression of airway.

Signs & Sxs: Variable, cough, chest pain, dyspnea, cyanosis

PE: low bp, absent or decreased breath sounds, high temp, tachycardia,

Question 43

Pulmonary Embolism

Answer 43

Obstruction of pulmonary artery leading to loss of blood supply to lung parenchyma, infarction. Usually from thrombus formation from elsewhere in body.

Signs & Sxs: Sudden onset SOB, dyspnea, pleuritic chest pain, hemoptysis (pink frothy), anxiety, cough.

PE: Normal chest exam is possible. Tachypnea, tachycardia, low BP, low fever, crackles, decreased breath sounds, splitting S2 heart sounds, S4 gallop, jugular venous distention.

Lab: d-dimer assay: elevated - degradation product of fibrin.
CBC, arterial blood gases, cardiac enzymes, BNP

Procedures: ECG, pulmonary angiography “gold standard”

Imaging: CXR

Well’s Criteria for Embolism:

DDx: Acute MI, pneumothorax, pleurisy from pneumonia, pericardial tamponade.

Question 44

Pulmonary Hypertension

Answer 44

Elevated pulmonary arterial pressure and secondary right ventricular overload and failure. Pulmonary vessels constrict, hypertrophy, and become fibrotic. Usually from increased vascular resistance and/or increased pulmonary venous pressure.

Question 45

Cyanosis

Answer 45

Blue discoloration of skin and mucus membranes, usually due to at least 5g of reduced Hb in arterial blood.

Central: due to hypoxemia caused by acute or chronic cardio/pulm. dz, COPD.
PE: skin and mucus membranes blue, chronic-may see clubbing of finger tips/nails

Peripheral: due to stagnant

Question 46

Sleep Apnea

Answer 46

Periodic cessation of breathing during sleep for 10 seconds or more, sometimes >300x/night
M:F 3:1, most common >30yo
Ethnicity- greater risk in AA, hispanics, Pacific islanders.

Blockage usually from upper airways, nose mouth, throat. Birth defects, injuries to face, obesity, alcohol, sedatives, spine deformities, facial changes.

Signs & sxs: loud snoring and excessive daytime somnolence - restless tossing and turning during sleep. Might have sense of choking.

Dx: clinically or with sleep study.