Pulmonology #2 (Restrictive Lung Diseases) Flashcards
What is typical on a PFT for restrictive lung disease?
-TLC, FVC, VC all decreased
-FEV1/FVC increased (easier to breathe out quickly)
–the lungs cannot expand fully because they are RESTRICTED from doing so
Idiopathic pulmonary fibrosis, which is ________, is MC in men > 40 and smokers. What are symptoms of this condition?
Scar tissue within the lungs due to unknown cause
-Dyspnea, dry/nonproductive cough, dry crackles at the base of the lungs
Diagnostics for idiopathic pulmonary fibrosis and what do they show?
PFT:
CXR:
Chest CT:
Biopsy:
PFT: Restrictive pattern (FVC, VC, TLC, DLCO decreased)
CXR: reticular opacities (honeycombing)
CT: reticular honeycombing, focal ground-glass opacification
Biopsy: honeycombing
Treatment for idiopathic pulmonary fibrosis
-Prednisone may help scarring
-Oxygen improves symptoms
-Smoking cessation
-Lung transplant is the only cure
What are pneumoconioses?
What is the general pathology of these conditions?
Group of lung diseases caused by inhalation of specific types of dust
Inhale something –> scarring of the lung tissue –> unable to expand completely
Coal Worker’s Lung (Black Lung Disease) is caused by inhalation of coal dust particles. Although general symptoms occur such as dyspnea, fine crackles/rales, there is a syndrome that can occur with this.
What part of the lungs does this condition affect?
Name and describe the syndrome that can occur with Coal Worker’s Lung.
Upper lobes of the lungs (coal is low, so think opposite)
Caplan Syndrome: RA + Coal Worker’s Lung
What is seen on chest X-ray for Coal Worker’s Lung?
Small nodules in the upper lung with hyperinflation of lower lobes in obstructive pattern (resembles emphysema)
A lung biopsy for coal worker’s lung shows what?
Dark/black lungs
Asbestosis affects what part of the lungs? Where do you get this condition from?
Lower lobes
Inhalation of asbestos (siding and insulation on old houses)
-Destruction, repair, renovation of old buildings. Ship building.
What is seen on CXR for asbestosis?
Pleural plaques (thickening) involving the lower lobes
-Shaggy heart sign: indistinct heart border, ground glass appearance
How about a biopsy for asbestosis? What is seen?
Linear asbestos bodies in the lung tissue (ferruginous bodies)
There are two common complications with asbestosis. What is the:
Most common:
Most specific:
Common: bronchogenic carcinoma
Specific: Malignant mesothelioma
Silicosis is due to inhalation of silicon dioxide. What careers/hobbies are at risk for this? What part of the lung does this affect?
Upper lobes
-Coal mining, quarry work with granite/slate/quartz
-Pottery makers
-Sandblasting, Masonry
What is seen on CXR for silicosis?
What is the definitive diagnostic?
-Multiple, small round opacities (miliary pattern) in the upper lobes
-Eggshell calcifications of hilar and mediastinal nodes
Lung biopsy is definitive
What careers/people are at risk for berylliosis?
Inhalation of beryllium
-Aerospace, electronics, tool manufacturing, jewelry making, fluorescent light bulbs
What is seen on CXR for berylliosis?
Lung biopsy?
CXR: may be normal, hilar LAD, increased interstitial lung markings
Biopsy: noncaseating granulomas
Treatment for berylliosis
Corticosteroids, oxygen
What is byssinosis from?
What is one unique symptom of this condition?
Cotton exposure, textile industry, hemp dust exposure
Monday Fever: symptoms get worse at the beginning of the week and improve later in week/weekend
In sarcoidosis, granulomas form throughout the body, but the lungs are the MC site for these. What is a granuloma?
Who is at risk for sarcoidosis?
Granuloma: WBC’s and macrophages that try to wall off foreign body or substance that immune system can’t get rid of
-Females, AA, Northern-Europeans
Symptoms of sarcoidosis (include all the extra pulmonary symptoms as well)
-50% asymptomatic
-Fever, weight loss, fatigue
-LAD: Hilar
-Erythema Nodosum: on shins
-Lupus Pernio (face and extremities)
-Anterior Uveitis
-Restrictive Cardiomyopathy
-Cranial nerve palsies: CNVII
In regards to sarcoidosis, what is Lofgren Syndrome?
Erythema Nodosum + Bilateral Hilar LAD + Polyarthralgias with fever
What is classic on CXR for sarcoidosis?
Bilateral hilar LAD (bat wing appearance), reticular opacities, ground glass appearance, eggshell calcifications, fibrosis
PFT for sarcoidosis is a restrictive pattern as well. What is the definitive/gold standard diagnosis for sarcoidosis?
-Tissue biopsy: noncaseating granulomas
What are the two specific lab findings in sarcoidosis?
Increased ACE levels from T-cells
Hypercalcemia (from macrophages)
Treatment for sarcoidosis
-Asymptomatic: observation
-Symptomatic: oral corticosteroids
What is hypersensitivity pneumonitis?
Name some types of this condition
-Generalized lung inflammation of alveoli and respiratory bronchioles due to organic dusts, molds, foreign proteins, and chemicals
Farmer’s/Cattle Worker’s Lung, Parrot Fever, Grain Worker’s Lung, Silo Filler Disease
What are the etiologies of the following hypersensitivity pneumonitis?
-Farmer’s/Cattle Worker’s Lung:
-Parrot Fever:
-Grain Worker’s Lung:
-Silo Filler Disease:
-Farmer’s/Cattle Worker’s Lung: moldy hay
-Parrot Fever (Psittacosis): infected birds
-Grain Worker’s: wheat infested with c weevils
-Silo Filler: nitrogen dioxide gas from plant matter
Symptoms of general hypersensitivity pneumonitis
-Rapid onset fever, chills, dyspnea, 4-8 hours after exposure
-Inspiratory crackles
-Dyspnea
-Productive cough
On chest xray, where are the diffuse, micro nodular interstitial patterns associated with hypersensitivity pneumonitis?
Lower lung fields MC
Silo Filler Disease is hypersensitivity pneumonitis from….
What are some recommendations to avoid this condition?
From nitrogen dioxide gas exposure released from plant matter stored in silos as they ferment
-Gas converted into nitric oxide in lungs when inhaled
-Also seen with combustion exposure (fires, diesel fumes)
-Do not enter the silo for 2 weeks, enter at the top of the silo, and wear N95 masks
Parrot Fever (Psittacosis) is from an infection with ______ due to infected bird exposure. This organism is inhaled in dried feces (cleaning cages, etc.)
Chlamydophila psittaci
What is the first-line treatment for Parrot Fever?
Tetracyclines (Doxycycline)!
