02b: Sarcoidosis, HP, et al Flashcards
Sarcoidosis pathology is characterized by (X) involving multiple organs systems, but most commonly:
X = granuloma
Lungs, lymph nodes, eyes, skin
T/F: Less than 20% of cases of Sarcoidosis resolve spontaneously.
False - over 80% (most)
Which diseases may appear similar to sarcoidosis and should be ruled out?
TB and certain malignancies
T/F: Over 90% of patients with sarcoid will have pulm involvement.
True
Stage 0 Sarcoidosis: what would you expect to find on CXR?
Normal (no abnormality)
Stage I Sarcoidosis: what would you expect to find on CXR?
Bilateral hilar lymph adenopathy (symmetrical)
Stage II Sarcoidosis: what would you expect to find on CXR?
Hilar lymph adenopathy and lung parenchymal involvement
Stage III Sarcoidosis: what would you expect to find on CXR?
Lung parenchymal involvement (no more hilar lymph node enlargement)
Stage IV Sarcoidosis: what would you expect to find on CXR?
Lung fibrosis
List the differential diagnosis of all interstitial lung diseases.
Acronym: SHITFACEDMD
- Sarcoid
- Hemorrhagic
- Idiopathic Interstitial Pneumonia (UIP, DIP, COP, RB-ILD)
- TB
- Failure (heart)
- Asbestos/amyloid
- CVD (collagen)
- Eosinophilic lung diseases (granulomas)
- Drugs
- Malignancy
- Dirt (inhaled; organic/inorganic)
When diagnosing sarcoid, it’s mandatory to rule out (X) cause of granulomatous response. What would you order to rule this out?
X = infectous
Cultures/special stains for infectious agents
Unless clinical presentation and CXR are highly indicative of sarcoid, (X) is the procedure of choice used to make diagnosis.
X = transbronchial biopsy
Sarcoidosis: DLCO is (increased/decreased/unchanged) and TLC will be (increased/decreased/unchanged).
Decreased;
Decreased with progressive disease BUT sarcoid has obstructive component that may preserve TLC (air trapping)
Beryllium exposure/poisoning will mimic pathogenesis of which disease?
Sarcoidosis
List some occupations that run the risk of Beryllium exposure.
- Aerospace
- Ceramic manufacturing
- Electronics
- Lab and dental work
- Mineral extraction
(X)% of patients with sarcoid present with pulmonary symtpoms.
X = 30-50% (though pulm involvement seen in over 90%)
Lofgren syndrome involves (X) symptoms and can be highly indicative of (Y) disease.
X = acute febrile illness with erythema nodosum on legs Y = Sarcoid (ESP if CXR shows bilateral hilar adenopathy)
Treatment of sarcoidosis typically involves (X) therapy.
X = immunosuppressive (steroids)
T/F: Sarcoidosis is always treated with drugs.
False - unless highly symptomatic or involves critical organ (eyes, heart, brain), wait few months to monitor progression/resolution
Sarcoidosis: assessing severity of disease involves the use of which tests?
- Serum ACE
- Ca and liver function tests
- EKG
- Eye exam
- PFTs
HP (Hypersensitivity Pneumonitis) is a(n) (acute/subacute/chronic) condition. Give the two most common examples of this disease.
Could be any;
Farmer’s lung and Bird fancier’s lung
Which lung sounds would you expect to hear in acute HP?
Diffuse crackles (rarely any wheezing)
Acute HP biopsy would show which pathology?
Poorly formed, non-caseating granulomas (interstitial) in peribronchial distribution
Your patient has acute HP. After doing a biopsy, you know you’re likely to find very prominent (X) cells.
X = giant
T/F: PFTs won’t be altered in acute HP.
True (will be in subacute and chronic HP)
Subacute HP biopsy would show which pathology?
- Well-formed non-caseating granulomas (interstitial)
- Bronchiolitis (maybe with pneumonia)
- Interstitial fibrosis
In subacute and chronic HP, which drug treatment may be required?
Glucocorticoids (Prednisone)
T/F: Sarcoid presenting with Lofgrens Syndrome is more than 95% likely to undergo spontaneous remission.
True
T/F: Long term oral corticosteroid therapy for sarcoid has no/minimal effect on natural history of pulmonary disease.
True (groups similar at 24 months)
T/F: Both HP and Sarcoid will present with crackles in lung sounds.
False - only HP
T/F: HP, but not Sarcoid, will present with clubbing of finger nails.
True
Bronchoalveloar lavage (BAL) CD4/CD8 can distinguish HP from (X) disease. Which has higher ratio?
X = Sarcoidosis
Sarcoid (over 3.5); HP less (under 1)
Serology for HP detects:
Precipitins (Ab) for offending antigen (ex: pigeons)
(X) is pulmonary disease caused by inhalation of (Y), the most abundant mineral on earth.
X = silicosis Y = (crystalline) silicon dioxide (silica)
(Crystalline/amorphous) silica is the form that causes pulm disease. What is the cause of its toxicity?
Crystalline;
Generates free radicals and injures alveolar macrophages (inflammation and fibrosis)
Occupational exposure, especially involving processing of rock, is the major risk factor leading to (X) disease. List examples of these occupations.
X = silicosis;
“Dusty trades”:
Mining, Construction, Sandblasting, Stone cutting, Tunneling, Ceramics/glass making, Furnace workers
How soon after exposure do silicosis symptoms appear?
Few weeks to few years
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?
Silicosis;
No, probably crackles
T/F: Acute Silicosis has rapid, progressive deterioration involving cor pulmonale/resp failure and death 4-5 years after symptom onset.
True
T/F: Silicosis features diffuse micronodular markings on CXR with lower lobe predominance.
False - upper lobe
List some diseases associated with silicosis.
- Lung cancer
- TB (mycobac infection)
- COPD/chronic bronchitis
- RA and sceroderma
T/F: Biopsy usually not needed to diagnose silicosis.
True - get adequate history of exposure, CXR, mycobac tests (to exclude TB)
Progressive Massive Fibrosis (PMF) results from combination/coalescence of (X) to form large opacities. CXR shows eggshell calcification pattern, characteristic of which disease?
X = silicotic nodules
Silicosis
Eggshell calcification of hilar lymph nodes suggestive of (X). What would you expect the PFTs to look like?
X = Progressive Massive Fibrosis (PMF) in chronic silicosis
SUPER abnormal (decreased compliance, FEV1, FEV1/FVC ratio, and DLCO)
Asbestosis is characterized by (slow/fast) progressive pulmonary fibrosis. Which occupations/exposures increase risk of disease?
Slow;
Shipbuilding, Insulation, Renovation/demolition of buildings, secondary exposure (ex: soiled clothes)
Pathogenesis of asbestosis: inhaled fibers deposit at (X) sites in lung, where they affect (Y).
X = resp bronchioles and alveolar duct bifurcations
Y = alveolar macrophages (injured), inducing inflammation and producing free radicals
T/F: Unlike silicosis, asbestosis patients present sooner with symptoms after exposure (within a year).
False! Most asbestosis patients asymptomatic for 20-30 years after initial exposure
Earliest symptom of asbestosis is (X). This (progresses/resolves) if exposure to asbestos is removed.
X = dyspnea (progressive) on exertion
Progresses (even if exposure removed)
T/F: Patients with asbestosis will have normal FEV1/FVC ratio.
True (no obstruction
(X) is the only known risk factor for malignant mesothelioma.
X = Asbestos exposure
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?
Asbestosis (secondary exposure, likely via husband’s clothes)
CXR with pleural plaques. What’s on the top of your differential?
Asbestos exposure (NOT necessarily asbestosis/disease state)
Histopathological diagnosis of asbestosis requires presence of (X) in association with interstitial pulmonary fibrosis.
X = asbestos bodies (asbestos fibers uncoated/coated by iron and protein)
T/F: Detection of asbestos bodies is enough to diagnose asbestosis.
False - need DEFINITE evidence for pulmonary fibrosis
Asbestos exposure increases risk of pulmonary malignancy by (X) fold. Smoking cigarettes by (Y) fold. Together, by (Z) fold.
X = 6 Y = 11 Z = 59
