Restrictive Pulm B&B

Question 1

how can scoliosis and morbid obesity cause restrictive lung disease?

Answer 1

poor breathing mechanics —> can’t get air in —> under ventilation of lungs

normal A-a gradient

neuromuscular (ALS, polio, myasthenia gravis) or structural (scoliosis, morbid obesity)

Question 2

how does interstitial lung disease appear histologically?

Answer 2

bilateral, diffuse pattern

small, irregular opacities (reticulonodular)

“honeycomb” lung appearance

Question 3

who can DLCO be used to differentiate the cause of restrictive lung disease?

Answer 3

DLCO = diffusing capacity in lung of carbon monoxide

restrictive disease with normal DLCO - extra-pulmonary (ex- obesity)

restrictive disease with low DLCO - interstitial lung disease

Question 4

how would anemia affect the measured DLCO?

Answer 4

DLCO = diffusing capacity in lung of carbon monoxide

with anemia, measured DLCO would be lower, but would be corrected when adjusted for Hb levels

Question 5

what kind of lung disease is idiopathic pulmonary fibrosis, and in which patients would you most likely see this?

Answer 5

restrictive interstitial lung disease (most common type is idiopathic interstitial pneumonia)

typically adults over 40, slow onset dyspnea

Question 6

what kind of lung disease are pneumoconiosis?

Answer 6

pneumoconiosis: occupational restrictive interstitial lung disease

ex - coal miner’s lung, silicosis, asbestosis

Question 7

what kind of lung disease is coal miner’s lung and how does it appear on CXR?

Answer 7

type of pneumoconiosis, occupational restrictive interstitial lung diseases

CXR shows small/round modular opacities mostly in upper lobes (think of black coal as smoke rising)

Question 8

what type of occupational workers are most affected by silicosis, and what kind of lung disease is this?

Answer 8

type of pneumoconiosis, occupational restrictive interstitial lung diseases

inhalation of silica in quarts, granite, or sandstone - foundry (metal production) workers, mine workers, workers using sandblasting (abrasive blasting)

Question 9

what kind of lung disease is silicosis, and how does it develop (describe pathophysiology)?

Answer 9

type of pneumoconiosis, occupational restrictive interstitial lung diseases

macrophages react to silica, inflammation activates fibroblasts to produce collagen

affects upper lobes, shows eggshell calcifications of lymph nodes

high prevalence of TB due to impaired macrophage killing, as well as bronchogenic carcinoma

Question 10

what kind of lung disease is silicosis, and what other diseases (2) does this put patients at high risk for?

Answer 10

type of pneumoconiosis, occupational restrictive interstitial lung diseases

macrophages react to silica

high prevalence of 1. TB (impaired macrophage killing) and 2. bronchogenic carcinoma

Question 11

what portion of the lungs does silicosis primarily affect, and how does this appear on CXR?

Answer 11

type of pneumoconiosis, occupational restrictive interstitial lung diseases

affects upper lobes, appears as eggshell calcifications of lymph nodes

Question 12

what kind of lung disease is asbestosis, and which portion of the lungs does this primarily affect?

Answer 12

type of pneumoconiosis, occupational restrictive interstitial lung diseases

classically affects lower lobes (think of asbestos walls in basements)

Question 13

what 3 clinical problems classically present with asbestos exposure (shipbuilding, roofing, plumbing)?

Answer 13

1. interstitial (restrictive) lung disease (asbestosis)
2. pleural plaques
3. lung cancer (bronchogenic carcinoma most common, mesothelioma rare)

Question 14

what kind of lung disease is asbestosis and how does it appear on CXR?

Answer 14

type of pneumoconiosis, occupational restrictive interstitial lung diseases

CXR: calcified pleural plaques and ferruginous bodies (asbestos fibers surrounded by coating of iron/protein)

Question 15

which 2 types of cancer are classically associated with asbestosis?

Answer 15

asbestosis: type of pneumoconiosis, occupational restrictive interstitial lung diseases

1. bronchogenic carcinoma (most common)
2. mesothelioma: rare but asbestos is only know risk factor, occurs decades later with slow onset symptoms and poor prognosis - pleural thickening/effusion and dyspnea/cough/chest pain

Question 16

what kind of lung disease are the following all associated with:
- scleroderma
- rheumatoid arthritis
- Goodpasture’s
- Wegener’s
- Sarcoidosis

Answer 16

all systemic diseases with interstitial (restrictive) lung disease features

Question 17

what kind of lung disease is toxic use of all the following drugs associated with:
- bleomycin (chemo - intercalating)
- busulfan (chemo - alkylating)
- amiodarone (anti-arrhythmic)
- methotrexate (immune suppress - folate antagonist)

Answer 17

interstitial restrictive lung disease

Question 18

what is hypersensitivity pneumonitis caused by, and what kind of lung disease is this?

Answer 18

interstitial restrictive lung disease, caused by hypersensitivity reaction (mixed type III/IV) to environmental antigens (agricultural dusts, microorganisms, chemicals)

classic case is farmer’s lung (moldy hay/grain exposure), also common in bird/poultry handlers (dried dust of bird waste)

Question 19

what kind of hypersensitivity reaction causes hypersensitivity pneumonitis, and what kind of lung disease is this?

Answer 19

interstitial restrictive lung disease

mixed type III (immune complex) and IV (delayed) hypersensitivity

classic case is farmer’s lung, also common in bird/poultry handlers

Question 20

how does hypersensitivity pneumonitis present, and what kind of lung disease is this?

Answer 20

interstitial restrictive lung disease

classically farmer or bird handler with cough/dyspnea/chest tightness and diffuse crackles