Lecture 2 - Restrictive Lung disease Flashcards
What are restrictive diseases characterized by?
- reduced lung volumes due to :
- alternation in lung parenchyma
- diseases of the pleura, chest wall, or neuromuscular tissue
Extrapulmonary, pleura, or parenchymal respiratory diseases that restrict lung expansion result in what two things?
- Decreased lung volume
- Increased work breathing
What are alveoli?
Tiny airsacs where O2 is absorbed, these expand with every breath
Interstitial Lung Disease causes?
Alot of them are idiopathic
Medications
Organic and inorganic dusts are the principle cause!
Patient with an Interstitial Lung Disease often have what history?
- some are more common in certain age groups, male or female predominant
- PMHx = connective tissue disease, IBD, malignancy
- cardiac medications (ex. amiodarcone)
- radiation pneumonitis - usually following 4-12 weeks of treatment
- Allergic rhinitis/asthma (hx of esosinophilic pneumonia)
- occupational/environmental hx (review home and work environment and their exposure to dust, gases, and chemicals)
What can ILD ultimately cause? aka (results it has to the lunge)
- Inflammation or scarring of the lung tissues
- Result in filling of the air spaces with exudate and debris
Etiologies of ILD
- Idiopathic fibrotic disease
- connective tissue disease
- drug induced disease
- primary disease of the lung like Sarcoidosis
What is an extrinsic disorder (extra-pulmonary disease?)
- when the chest wall, pleura, and/or respiratory muscles do not function normally for effective ventilation
what happens when there is an extra-pulmonary disease?
diseases will result in:
lung restriction
impaired ventilation
respiratory failure
Five things that involve lung function
PAINT
- pleural
- alveolar
- interstitial
- neuromuscular
- thoracic cage abnormalities
Common signs and symptoms of restrictive pulmonary disorders
- progressive dyspnea
- late inspiratory crackles
- DRY cough (usually not productive
- CLUBBING
- uncommon: wheezing and chest pain
What is clubbing?
A common factor in digital clubbing is increased blood flow because of vasodilation to the distal portions of the fingers
Labs on a restrictive pulmonary disease disorder
- ANA (lupus titer that will tell if there is an autoimmune disease and if there is another autoimmune issue)
- RF (rheumatoid factor)
- DS DNA-specific for SLE (rarely find in other patients with other disorders, titers fluctuate with disease activity)
- ANCA (found in autoimmune diseases causing vasculitis)
What imaging should be done for a suspected restrictive lung disorder?
CXR - reticular pattern complex network of opacities that involve the lung diffusely
Chest CT scan - high resolution
PFT with DLCO - decrease TLC (total lung capacity)
Sarcoidosis:
- Etiology?
- What is a common finding on imaging?
- What is never an etiology?
- Who is this common in? What time in their lives?
- What two disease states do not cause sarcoidosis?
- Unknown etiology
- Non caseasting granulomas
- No bacterial, fungal, or viral etiologies
- North American blacks, northern european whites; 3-4th decades of life
- Malignancy or autoimmune diseases
Signs and Symptoms of Sarcoidosis
- 5% cases are asymptomatic (discovered on a CXR)
- fever
- anorexia
- DOE
- cough
- chest pain
- parotid gland enlargement
- derm, ocular, cardiac manifestations
- cardiomyopathy
- iritis
- hepatosplenomegaly
- arthritis
- lympahadenopathy
- skin involvement
- erythema nodosom (hard red nodules directly beneath the skin)
Work up for Sarcoidosis
- CXT
- PFT
- annual EKG
- Holter monitor
- Bx for diagnosis (lymph node, salivary gland, skin lesion) –> the transbronchial lung Bx is best yield
- CMP (looking at live/kidney function)
- UA
- ACE
Sarcoidosis:
- CXR findings?
- PFT findings?
1. HILAR NODES!!! Stage 0 = normal chest Stage 1 = bilateral hilar nodes Stage 2 = bilateral hilar nodes with infiltrates Stage 3 = Infiltrate alone Stage 4 = fibrosis alone
- -restrictive pattern
- decreased DLCO
- hypercalcemia AND ACE (bc non caseating granulomas secrete Vit D and ACE) –> serum ACE levels may correlate w/ total body granuloma load, sensitivity and specificity is limited at 60-70%, decision to treat should not be on ACE levels alone
Treatment and prognosis of sarcoidosis?
Treatment
- asympotmatic = no treatment needed
- respiratory symptoms = coricosteroids (prednisone daily for 6 months)
Prognosis
- many patients do not require therapy and can spontaneously improve
- markers for a poor prognosis are advanced CXR stage, extra pulmonary disease, pulmonary HTN
Ocular, cardiac, and neruo manifestations of sarcoidosis
Ocular - 30-60% of patient develop intraocular inflammatory signs and bilateral granulomatous uveitis
Cardiac - heart block and sudden cardiac may occur
Neuro - cranial nerve palsies
Long term monitoring of Sarcoidosis
- PFT and XCR every 6-12 months
- assess for progression or resolution
- determine if previously uninvolved organs have become affected
- annual slit lamp eye exam
- annual ECG
Idiopathic Pulmonary Fibrosis (IPF)
- This is a specific form of what?
- what does the progression of the disease look like?
- who does this disease affect?
- what is important to ask the patient before diagnosing this disease?
- progressive fibrosing interstitial pneumonia of unknown cause, limited to the lungs
- gradual onset of exertional dyspnea and/or a nonproductive cough
- men more than women, 50-70 years old
- critical to obtain the following hx:
- medical
- drug
- social
- occupational
- recreational
- environmental
* ***Assess HIV risk!!!
Physical exam findings of idiopathic pulmonary fibrosis
- fine bibasilar “velcro” crackles (stronger sound than pneumonia)
- digital clubbing
- pulmonary HTN at rest
What labs, tests, and procedures would you want to do with a patient with IPF and what would you find?
- PFT - restrictive pattern and decrease DLCO
- ABG - chronic hypoxemia
- ANA & RF
- **High resolution CT scan (specific, sensitive, and essential for the diagnosis of IPF – you will see patchy, peripheral, sub pleural, and bibasilar reticular opacities)
- transthoracic echo-pulmonary HTN (sonogram of the heart to look for HTN)
- Procedure
- bronchoscopy
- surgical lung bx best for distinguishing the usual interstitial pneumonia from others
Treatment of IPF
- *Most common disease referred for lung transplantation (second most common reason why lung transplantation is performed)
- supportive care: O2, pulm rehab, treat infections, influenza, and pneumococcal vaccine
- Nintedanib - antifibrotic drug, works by reducing lung fibrosis
- Pirfenidone - Tyrsine kinase inhibitors - slows down decrease in FCV
What is asbestosis?
Describe the incidence of the disease?
- nodular interstitial fibrosis occurring in workers exposed to asbestos fibers
- incidence varies w/ the cumulative dose of inhaled fibers (the greater the cumulative dose, the higher the incidence of abestosis)
All types of asbestosis fibers are ____ to the lungs
Fibrogenic
Occupations that may put a patient at risk for asbestosis
- insulation workers
- boil makers
- pipe fitters
- plumbers
- steam fitters
- welders
- janitors
Asbestosis
- Epidemiology?
- Important history piece and pertinent signs and symptoms?
- What would be found on a physical exam?
- What would advanced symptoms looks like
- Some examples of products that may contain asbestos
- No reliable number exists regarding the number of people presently at risk of asbestosis, but strict regulation has prohibited spraying asbestosis in building and controlling levels of fibers in the air have drastically reduced the risk.
- Dose dependent = symptoms appear only after a latent period of 20 years or longer. Patient may present with DOE and dry cough.
- Rales at the BASES, fingers clubbing (32-42% of cases)
- Cor pulmonale, JVD, hepatojugular reflux, pedal edema
- pipes, shingles, vinyl abestosis floor boards, textile products, spray products useful for thermal and fireproofing purposes
CXR findings for asbestosis
- Early stages?
- At the bases of the lungs?
- Advanced stages?
- What happens to the pleura?
- What is the hallmark???
- What is found along the 6-9th ribs along the diaphragm ?
- Combined interstitial and pleural involvement may cause a hazy “ground glass” appearance to the CXR that may blur the diaphragm and the heart boarder
- Diffuse reticulonodular infiltrates
- Honeycombing appearance
- Bilateral pleural thickening
- Pleural involvement is the hallmark of asbestos exposure where it is unusual in other interstitial advanced disease
- Pleural Plaques!!
What factors would diagnose asbestosis?
- hx of asbestosis exposure and an appropriate latency period between exposure and disease
- characteristic changes of pulmonary fibrosis on imaging studies
- absence of other fibrotic disease
- DOE
- bilateral basilar inspiratory crackles
- restrictive pattern on PFT w/impaired DLCO
- bx = confirmation of both fibrosis and accumulation of fibers or asbestos bodies
Why would you worry about a diagnosis of asbestosis?
Malignancy: huge risk for lung carcinoma
Risk for malignant mesothlioma and carcinoma of the UPT, esophagus, biliary system, and kidney
Pulmonary HTN, Core pulmonale, progressive respiratory insufficiency
What would a high resolution CT scan allow for with a patient with asbestosis?
Better definition of interstitial infiltrates and may be helpful in diagnosing asbestosis in early stages
Why is evaluation of asbestosis important?
Uncorrected hypoxemia causes pulmonary HTN and may lead to Cor pulmonale
What does treatment for asbestosis entail?
- inform patients about work related causation of the disease and report is to agency
- stop smoking
- DRUGS ARE NOT EFFECTIVE
- influenze and pneumococcal vaccine
- assess the patients O2 with rest and exercise
- prompt abx therapy for infections
- ***steroids do not alter the disease
Sarcoidosis
- Etiology?
- How do you diagnosis? Simple? Complicated?
- Extensive, prolonged inhalation of silicon dioxide causes the formation of small rounded opacitities in the lung
-rock mining
-quarying
-stone cutting
-sandblasting
-pottery - Simple = Usually asymptomatic and has no effect on the routine PFT
Complex = large conglomerate densities appear in the upper lung and are accompanied by SOB
What does the xray look like for silicosis?
- calcification of the periphery of hilar lymph nodes
- eggshell calcification is an unusual radiographic finding that strongly suggests silicosis
- What is hypersensitivity pneumonitis?
- How does someone get it?
- CXR findings?
- Treatment?
- Classifications types?
- disease of the lungs in which they become inflammed and sensitive an an allergic reaction to inhaled dust, fungus, or mold
- occupational –> exposure to inhaled organic antigens leads to an acute illness
- small nodular densities sparing the apices and bases of the lungs
- avoid offending agents, prednisone, change in occupation
- Farmers lung = source is moldy hay
Bird Fancier’s lung = bird serum and excretion is the source
**Prompt diagnosis is essential since symptoms are reversible if the offending antigen is removed from the patients environment early in the course of the illness
Physical Exam findings for hypersensitivty pneumonitis
- sudden onset of malaise, chills, fever
- cough, dyspnea, and nausea 4-5 hrs after exposure to offending antigen
- bibasilar crackers, tachypnea, tachycardia
Antigens that may be found in the home that could cause hypersensitivy pneumonitis
- bird droppings
- moldy hay
- heating and humidifier
