02b: Sarcoidosis, HP, et al

Question 1

Sarcoidosis pathology is characterized by (X) involving multiple organs systems, but most commonly:

Answer 1

X = granuloma

Lungs, lymph nodes, eyes, skin

Question 2

T/F: Less than 20% of cases of Sarcoidosis resolve spontaneously.

Answer 2

False - over 80% (most)

Question 3

Which diseases may appear similar to sarcoidosis and should be ruled out?

Answer 3

TB and certain malignancies

Question 4

T/F: Over 90% of patients with sarcoid will have pulm involvement.

Answer 4

True

Question 5

Stage 0 Sarcoidosis: what would you expect to find on CXR?

Answer 5

Normal (no abnormality)

Question 6

Stage I Sarcoidosis: what would you expect to find on CXR?

Answer 6

Bilateral hilar lymph adenopathy (symmetrical)

Question 7

Stage II Sarcoidosis: what would you expect to find on CXR?

Answer 7

Hilar lymph adenopathy and lung parenchymal involvement

Question 8

Stage III Sarcoidosis: what would you expect to find on CXR?

Answer 8

Lung parenchymal involvement (no more hilar lymph node enlargement)

Question 9

Stage IV Sarcoidosis: what would you expect to find on CXR?

Answer 9

Lung fibrosis

Question 10

List the differential diagnosis of all interstitial lung diseases.

Answer 10

Acronym: SHITFACEDMD

1. Sarcoid
2. Hemorrhagic
3. Idiopathic Interstitial Pneumonia (UIP, DIP, COP, RB-ILD)
4. TB
5. Failure (heart)
6. Asbestos/amyloid
7. CVD (collagen)
8. Eosinophilic lung diseases (granulomas)
9. Drugs
10. Malignancy
11. Dirt (inhaled; organic/inorganic)

Question 11

When diagnosing sarcoid, it’s mandatory to rule out (X) cause of granulomatous response. What would you order to rule this out?

Answer 11

X = infectous

Cultures/special stains for infectious agents

Question 12

Unless clinical presentation and CXR are highly indicative of sarcoid, (X) is the procedure of choice used to make diagnosis.

Answer 12

X = transbronchial biopsy

Question 13

Sarcoidosis: DLCO is (increased/decreased/unchanged) and TLC will be (increased/decreased/unchanged).

Answer 13

Decreased;

Decreased with progressive disease BUT sarcoid has obstructive component that may preserve TLC (air trapping)

Question 14

Beryllium exposure/poisoning will mimic pathogenesis of which disease?

Answer 14

Sarcoidosis

Question 15

List some occupations that run the risk of Beryllium exposure.

Answer 15

1. Aerospace
2. Ceramic manufacturing
3. Electronics
4. Lab and dental work
5. Mineral extraction

Question 16

(X)% of patients with sarcoid present with pulmonary symtpoms.

Answer 16

X = 30-50% (though pulm involvement seen in over 90%)

Question 17

Lofgren syndrome involves (X) symptoms and can be highly indicative of (Y) disease.

Answer 17

X = acute febrile illness with erythema nodosum on legs
Y = Sarcoid (ESP if CXR shows bilateral hilar adenopathy)

Question 18

Treatment of sarcoidosis typically involves (X) therapy.

Answer 18

X = immunosuppressive (steroids)

Question 19

T/F: Sarcoidosis is always treated with drugs.

Answer 19

False - unless highly symptomatic or involves critical organ (eyes, heart, brain), wait few months to monitor progression/resolution

Question 20

Sarcoidosis: assessing severity of disease involves the use of which tests?

Answer 20

1. Serum ACE
2. Ca and liver function tests
3. EKG
4. Eye exam
5. PFTs

Question 21

HP (Hypersensitivity Pneumonitis) is a(n) (acute/subacute/chronic) condition. Give the two most common examples of this disease.

Answer 21

Could be any;

Farmer’s lung and Bird fancier’s lung

Question 22

Which lung sounds would you expect to hear in acute HP?

Answer 22

Diffuse crackles (rarely any wheezing)

Question 23

Acute HP biopsy would show which pathology?

Answer 23

Poorly formed, non-caseating granulomas (interstitial) in peribronchial distribution

Question 24

Your patient has acute HP. After doing a biopsy, you know you’re likely to find very prominent (X) cells.

Answer 24

X = giant

Question 25

T/F: PFTs won't be altered in acute HP.

Answer 25

True (will be in subacute and chronic HP)

Question 26

Subacute HP biopsy would show which pathology?

Answer 26

1. Well-formed non-caseating granulomas (interstitial) 2. Bronchiolitis (maybe with pneumonia) 3. Interstitial fibrosis

Question 27

In subacute and chronic HP, which drug treatment may be required?

Answer 27

Glucocorticoids (Prednisone)

Question 28

T/F: Sarcoid presenting with Lofgrens Syndrome is more than 95% likely to undergo spontaneous remission.

Answer 28

True

Question 29

T/F: Long term oral corticosteroid therapy for sarcoid has no/minimal effect on natural history of pulmonary disease.

Answer 29

True (groups similar at 24 months)

Question 30

T/F: Both HP and Sarcoid will present with crackles in lung sounds.

Answer 30

False - only HP

Question 31

T/F: HP, but not Sarcoid, will present with clubbing of finger nails.

Answer 31

True

Question 32

Bronchoalveloar lavage (BAL) CD4/CD8 can distinguish HP from (X) disease. Which has higher ratio?

Answer 32

X = Sarcoidosis Sarcoid (over 3.5); HP less (under 1)

Question 33

Serology for HP detects:

Answer 33

Precipitins (Ab) for offending antigen (ex: pigeons)

Question 34

(X) is pulmonary disease caused by inhalation of (Y), the most abundant mineral on earth.

Answer 34

``` X = silicosis Y = (crystalline) silicon dioxide (silica) ```

Question 35

(Crystalline/amorphous) silica is the form that causes pulm disease. What is the cause of its toxicity?

Answer 35

Crystalline; Generates free radicals and injures alveolar macrophages (inflammation and fibrosis)

Question 36

Occupational exposure, especially involving processing of rock, is the major risk factor leading to (X) disease. List examples of these occupations.

Answer 36

X = silicosis; "Dusty trades": Mining, Construction, Sandblasting, Stone cutting, Tunneling, Ceramics/glass making, Furnace workers

Question 37

How soon after exposure do silicosis symptoms appear?

Answer 37

Few weeks to few years

Question 38

CXR of patient reveals diffuse interstitial markings that are nodular in nature. He denies smoking history and worked as sandblaster for 3 years. What's on the top of your differential? Will you expect to hear normal lung sounds?

Answer 38

Silicosis; No, probably crackles

Question 39

T/F: Acute Silicosis has rapid, progressive deterioration involving cor pulmonale/resp failure and death 4-5 years after symptom onset.

Answer 39

True

Question 40

T/F: Silicosis features diffuse micronodular markings on CXR with lower lobe predominance.

Answer 40

False - upper lobe

Question 41

List some diseases associated with silicosis.

Answer 41

1. Lung cancer 2. TB (mycobac infection) 3. COPD/chronic bronchitis 4. RA and sceroderma

Question 42

T/F: Biopsy usually not needed to diagnose silicosis.

Answer 42

True - get adequate history of exposure, CXR, mycobac tests (to exclude TB)

Question 43

Progressive Massive Fibrosis (PMF) results from combination/coalescence of (X) to form large opacities. CXR shows eggshell calcification pattern, characteristic of which disease?

Answer 43

X = silicotic nodules Silicosis

Question 44

Eggshell calcification of hilar lymph nodes suggestive of (X). What would you expect the PFTs to look like?

Answer 44

X = Progressive Massive Fibrosis (PMF) in chronic silicosis SUPER abnormal (decreased compliance, FEV1, FEV1/FVC ratio, and DLCO)

Question 45

Asbestosis is characterized by (slow/fast) progressive pulmonary fibrosis. Which occupations/exposures increase risk of disease?

Answer 45

Slow; Shipbuilding, Insulation, Renovation/demolition of buildings, secondary exposure (ex: soiled clothes)

Question 46

Pathogenesis of asbestosis: inhaled fibers deposit at (X) sites in lung, where they affect (Y).

Answer 46

X = resp bronchioles and alveolar duct bifurcations Y = alveolar macrophages (injured), inducing inflammation and producing free radicals

Question 47

T/F: Unlike silicosis, asbestosis patients present sooner with symptoms after exposure (within a year).

Answer 47

False! Most asbestosis patients asymptomatic for 20-30 years after initial exposure

Question 48

Earliest symptom of asbestosis is (X). This (progresses/resolves) if exposure to asbestos is removed.

Answer 48

X = dyspnea (progressive) on exertion Progresses (even if exposure removed)

Question 49

T/F: Patients with asbestosis will have normal FEV1/FVC ratio.

Answer 49

True (no obstruction

Question 50

(X) is the only known risk factor for malignant mesothelioma.

Answer 50

X = Asbestos exposure

Question 51

A "shaggy heart border" on CXR with basilar interstitial disease. Patient's husband worked in old building renovations. What's on the top of your differential?

Answer 51

Asbestosis (secondary exposure, likely via husband's clothes)

Question 52

CXR with pleural plaques. What's on the top of your differential?

Answer 52

Asbestos exposure (NOT necessarily asbestosis/disease state)

Question 53

Histopathological diagnosis of asbestosis requires presence of (X) in association with interstitial pulmonary fibrosis.

Answer 53

X = asbestos bodies (asbestos fibers uncoated/coated by iron and protein)

Question 54

T/F: Detection of asbestos bodies is enough to diagnose asbestosis.

Answer 54

False - need DEFINITE evidence for pulmonary fibrosis

Question 55

Asbestos exposure increases risk of pulmonary malignancy by (X) fold. Smoking cigarettes by (Y) fold. Together, by (Z) fold.

Answer 55

``` X = 6 Y = 11 Z = 59 ```