Restrictive Lung Disease Flashcards
Total lung capacity =_____+_____.
FVC + RVC
Asbestosis:
- cause
- disease manifestation
Cause:
-inhalation of asbestos fibers; chrysotile(less toxic, 90% of cases) & amphibole
Manifestation:
- slowly progressive (20-30years), diffuse FIBROSIS
- spectrum : asbestosis, pleural dz, malignancies (non-small cell and small cell carcinoma of lungs, malignant mesothelioma)
How does one acquire asbestosis?
-direct toxic effect of the fibers on pulmonary cells and release of mediators from inflamm cells
Clinical findings of asbestosis
- asymptomatic for 20-30yrs after exposure
- dyspnea on exertion
- progress to bibasilar end expiratory crackles and clubbing
- if cough, sputum production, or wheezing are present more likely secondary to smoking**
Dx of Asbestosis consists of what tests?
- PFTs
- -reduced lung volumes (VC and TLC)
- -decreased pulmonary compliance
- -absence of airflow obstruction
- Radiographs
- -pleural abnormalities
- -shaggy heart and ground glass appearance
- -honeycombing and upper lobe involvement late stage disease
In what order does asbestosis progress in the lungs?
-begins in the lower lung zones with small parenchymal opacities with multinodular or reticular pattern
Bronchiolitis Obliterans
-how do you get this?
- chronic airway rejection in lung transplant patients d/t:
- -episodes of acute rejection
- -primary graft dysfunction
- -CMV pneumonitis
- -Noncompliance w/ immunosuppressive meds
- -lymphocyte bronchitis or bronchiolitis
-can develop farther out from transplant (5yrs after lung transplant)
Presentation of Bronchiolitis Obliterans
- indolent sx, similar to URI (cough, tired, fatigue, exertional dyspnea)
- decline in spirometry
- advanced stages we see bronchiectasis w/ obstruction and hyperinflation, often colonized w/ pseudomonas d/t their immunosuppression
Dx of Brochiolitis Obliterans
- requires transbronchial biopsies w/ Bronchiol Alveolar Lavage (BAL)
- must rule out infection (viral and bacterial cultures must be negative)
Tx of Bronchiolotis Obliterans
- changing anti-immune medications
- photopheresis
- retransplantation
- Prevention: make sure pt takes immunosuppression drugs and they are staying healthy)
Hypersensitivity Pneumonitis
- aka
- what is this?
- causes
aka: extrinsic allergic alveolitis
- this is an immunologic rxn to an inhaled agent occuring within the pulmonary parenchyma, a restrictive lung dz
Causes:
- agricultural dusts
- bioaerosols
- reactive chemical species
Who gets Hypersensitivity pneumonitis?
WHat may actually be associated with decreased risk of HP?
- Farmers lung: the farming population
- bird fanciers
-smoking is associated with a decreased risk of HP
List some etiologic agents that may cause HP
- farming, vegtable and dairy cattle workers
- ventilation and water-related contamination
- bird and poultry handling
- veterinary worka nd animal handling
- Grain and flour processing and loadign
- lumber milling, construction, wood stripping
- painting
Acute HP
- presentation
- PE findings
- Tx
- what would you see on x-ray
Presentation :
- abrupt onset 4-6hrs of:
- fever and chills
- nausea
- chest tightness & without wheezing***
PE:
-tachypnea and diffuse fine rales(crackles)
Tx:
-removal from Ag, sx subside in 12hrs to sever days, disease may recur with re-exposure
See on CXR/HRCT:
-may show micronodular, interstitial pattern
Subacute or intermittent HP
- presentation
- lab findings
- PFT findings
- Xray finding
- Tx
-gradual development of cough, dyspnea, fatigue, anorexia, and weight loss, tachypnea, diffuse rales
Lab findings:
-lymphocytosis on bronchioal alveolar lavage, mild hypoxemia
PFT:
-restriction patter or mixed restriction/obstruction pattern
Xray:
-normal or reticular opacities in middle and upper lung zones
Tx: removal from ag and glucocorticosteroids, take weeks to months to resolve.
CHronic Progressive HP
- sx
- PE findings
- Labs
- PFT
- XRAY
- Tx
sx: insidious onset of cough, dyspnea, fatigue and weight loss
PE: digital clubbing
Labs:
-lymphocytosis, neutrophilia or eosinophilia on BAL.
PFT: restrictive, obstructive often seen with it, resting and exertional hypoxemia
XRay:
-fibrotic changes, loss of lung volume, emphysema pattern changes
Tx:
- antigen avoidance
- glucocorticoids used to accelerate initial recovery.
Dx of HP
- high index of suspicion from careful review of occupational, avocational, and domestic exposures
- normal CXR doesnt rule it out, need to have HRCT
- inhalation challenge by re-exposure
- HRCT and BAL (if chronic intermittent or chronic progressive they may need these tests)
Prevention of HP
- reduction of antigenic burden (wetting compost)
- design facilities (maintain humidty, avoid having stagnant water or carpet)
- Maintenance: routinely inspect all heating, ventilation, and air conditioning equipment.
- protective devices (masks and filters)
What are the 5 acute side effects of steroids?
- insomnia
- stomach upset
- psychosis
- elevated BS
- elevated BP
What are the long term effects of steroids?
- osteoporosis
- psychosis
- weight gain
- cataracts
- immunosuppression
- adrenal axis suppression
Interstitial Lung Disease
-definition
def: diffuse parenchymal lung disease
- a group of disorders that are classified together because of clinical xray, physiologic or pathologic manifestations.
Interstitial Lung Disease
- 50% are _____.
- clinical presentation
- prognosis
- presentation on CXR
-50% are idiopathic (Idiopathic pulmonary Fibrosis)
- Clinical presentation:
- progressive exertional dyspnea and nonproductive cough
Prognosis: usually die of resp failure within 3-6 years once x-ray changes
Presentation on CXR:
-haziness progresses to nodules then linear opacities, shaggy heart border, blunted costophrenic angle
Idiopathic pulmonary fibrosis
- what is this?
- cause?
- risk factors
What is this?
-chronic relentless progressive fibrotic disorder of the lover resp. tract
-cause: idiopathic, we dont know what causes this.
Risk factors:
- smoking
- infections
- environmental pollutants
- chronic aspiration and drugs
Idiopathic Pulmonary Fibrosis
-progression of disease
alveolitis is dominated by inflamm cells early on, these cells are:
- alveolar mfs
- neutrophils
- eosinophils
- lymphocytes
- increased basophils and mast cells
