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73 Cards in this Set

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Definition of Interstitial Lung Disease (ILD).
thickening of alveolar walls by inflammation and fibrosis
What are the most common ILDs?
idiopathic
What is the most common of the IIPs?
Usual interstitial pneumonia
Name the disease: "plugs" of fibroblasts in airways and consolidation seen on CT
bronchilolitis obliterans organized pneumonia (BOOP)
Only disease caused by inhalation of organic particles.
Hypersensitivity Pneumonitis (HP)
Abnormal immunological reaction of lung to specific organic antigens
Hypersensitivity Pneumonitis
What is the two key factors in determining whether a particle will be inhaled?
amount and size, (small enough (5 microns) to get into alveolar space)
Common organic particles seen in HP?
fungi, mold, mildew, and avian proteins
Steps in pathogenesis of HP.
antigen expoure -> accumulation of inf. cells -> lympocytic alveolitis in more chronic phases -> granuloma formation -> normal repair or fibrosis
Presentation of acute HP.
fever, chills, dyspnea, cough w/in hours of exposure
CXR of acute HP.
will show infiltrates
HRCT of acute HP.
"ground glass opacifications" typically in upper zones of lungs
Presentation of chronic HP.
persistent cough, SOB w/ exercise, sputum production (same as someone w/ IPF or fibrotic lung disease)
CT of chronic HP.
More fibrosis seen instead of ground glass opacifications
T/F: Removal of pet bird usually fixes the problem in a family with chronic HP.
False. Bird antigens can stay in house for a long time.
PFTs seen w/ chronic HP.
restrictive pattern (decreased FEV1, FVC, TLC, DLCO)
What is the gold standard for diagnosing HP?
lung biopsy
Lung biopsy of acute HP.
loose granulomas, mostly around airways -- suggestive but not diagnostic of HP
Lung biopsy of chronic HP.
looks like UIP. Honeycombing in lower zones.
Treatment for HP.
Removal of aggravating agent, along with possible steroids (if you give them steroids and they stay on the farm, they won't get better)
What is asbestos?
naturally occuring mineral w/ thermo-insulating properties
Where is asbestos commonly found?
materials that need insulation or is exposed to large amounts of heat.... paint, boilers, brakes
What industries was asbestos commonly used in?
shipbuilding, construction, automotive, railroad
What form is the overwhelming majority of asbestos in the US?
chrysotile (98%)
Why is chrysotile the more benign form of asbestos?
it can't migrate to the outer lung and to the lymphatics
What diseases does asbestos cause?
pleural disease, malignant mesothelioma, bronchiogenic cancer, and asbestosis
Definition of abestosis.
interstitial pneumonitis and fibrosis caused by exposure to asbestos fiber
What is the latency for asbestos exposure?
15-20 years
Two main componenents of the pathogenesis of asbestosis.
1) direct toxic effects which kill alveolar cells
2) release of mediators from inflammatory cells causes fibrosis and destruction
What is an asbestos body?
seen in lung biopsy - asbsestos fibers surrounded by a coating of iron and protein
Physical findings in asbestosis.
progressive dyspnea, bibasilar inspiratory crackles (Velcro), clubbing
Radiographic findings in asbestosis.
pleural plaques, round atelectasis

predominately a basilar disease
Management of asbestosis.
remove exposure, smoking cessation, early detection, supportive therapy including oxygen, vaccines, treatment of resp. infections
T/F: Corticosteroids have not been proven to be affective in the treatment of asbestosis.
True
Definition of silicosis.
chronic fibrosis disease of lungs caused by inhalation of silica dust
Where is silica dust found?
foundry work, tunneling, sandblasting, pottery making, manufacturers of glass, tiles, and brick
Three clinical types of silicosis:
1) simple/chronic - low conc. over 20 years or more
2) accelerated - moderately high conc. over 4-8 years
3) acute - massive exposure, presents similarly to ARDS
Pathogenesis of silicosis.
Deposited in distal airways -> ingested by macrophages -> macrophages activated -> inf. mediators released -> inf. and fibrosis
Clinical presentation of silicosis.
most asymptomatic, abnormal CXRs, restrictive PFTs, 10-15% develop "progressive massive fibrosis"
Typical CXR for silicosis.
micronodular opacities in the UPPER zones
How does one differentiate the diagnoses of asbestosis and silicosis?
asbestosis usually found in lower zones while silicosis is typically found in upper zones
Silicotic nodule
silicotic area surrounded by inflammation.. if you polarize you can see the sand
Associated illnesses w/ silicosis.
mycobacterial infections, especially TB
CTD occur more frequently, especially scleroderma
lung cancer
Why are silicosis patients susceptible to TB?
alveolar macrophages are filled w/ silica so they can't eat the bugs
Management of silicosis.
remove from environment, yearly screening for TB, smoking cessation, supportive therapy including oxygen and bronchodilator therapy
What causes coal worker's pneumonconiosis?
excessive inhalation of coal dust and silica
Why is the underground more hazardous for inhalation?
more concentration of particles
What is the typical presentation for coal worker's pneumoconiosis?
mostly asymptomatic w/ small nodular opacities on CXR, no lung area affected more than another
Lung biopsy of coal worker's pneumoconiosis.
normal lung w/ very thin alveolar walls
What causes the damage in ILD associated w/ CTDs?
pathogenic auto-Abs and immune complexes
Prevalence of ILD w/ lupus.
rare
Prevalence of ILD w/ scleroderma.
frequent
True/False: ILD never precedes systemic manifestations of CTDs.
False. ILD precedes in 30% of cases.
List some problems that CTDs can cause in the chest.
bronchiolitis, airway problems, pleural disease, resp. muscle dysfunction, pulmonary HTN, bleeding into alveolar space
What is the correlation btwn RF titers and likelihood of ILD.
The higher the RF titers, the higher the likelihood of ILD.
Who is ILD caused by rheumatoid more common in ?
men w/ severe disease
Infiltrates in ILD from RA are typically found in the ?
bases
Mean survival for ILD from RA.
5 years
Most common manifestatoin of ILD from RA.
pleural
What is scleroderma?
accumulation of CT in skin and visceral organs
In the diffuse cutaneous form of scleroderma, 40% have ?
antitopoisomerase 1 Ab (Scl-70)
In the limited cutaneous form of scleroderma, 60-80% have?
anticentromere Ab
Most lung abnormalities in ILD related to scleroderma are found?
in the base
What is a common symptom of scleroderma that can makes fibrosis worse?
esophageal dysmotility causing aspirations
What is the most common cause of death in scleroderma?
lung disease
If DLCO<40% what is the 5 year survival in ILD related to scleroderma?
less than 10%
The risk of ? is increased in ILD related to scleroderma.
bronchogenic cancer
Two common manifestations seen in Polymyositis/dermatomyositis?
heliotrope rash (around eyes) and Gottron's sign (reddish discoloration in knuckles)
ILD is a side effect w/ what types of drugs?
cytotoxic agents (chemotherapy), risk of ILD may be associated w/ concomitant use of radiation
ILD due to Bleomycin is ____ dependant.
dose
ILD due to cyclophosphamide/methotrexate is ? dependent.
time
Anti-arrythimia drug that can cause ILD.
amiodarone (more common if used >400 mg/day)
Treatment of amiodarone induced ILD.
stop the drug, and give prednisone.