Interstitial Lung Diseases Flashcards
What population if respiratory distress syndrome common in? Why?
preterm infants (<37 weeks gestation)
due to deficiency of surfactant in immature lung
What are signs of respiratory distress syndrome? When does it present?
- tachypnea
- nasal flaring
- expiratory grunting
- intercostal, subcostal retractions
- cyanosis
presents within mins to hrs after birth- progressively worsens over 1st 48 hrs of life
What are physical exam findings you would see in a pt w/ respiratory distress syndrome?
- decreased breath sounds
- pale w/ decreased peripheral pulses
- urine output in 1st 48 hrs often low
- peripheral edema common
What is the classic finding on CXR of a baby w/ respiratory distress syndrome?
Classic reticulogranular ground glass appearance w/ air bronchograms
(+ low lung volume seen)
How do you dx respiratory distress syndrome?
Diagnosis is clinical!
must distinguish RDS from other causes of respiratory distress
How can you prevent respiratory distress syndrome?
- prevent preterm birth when possible
- Antenatal (before birth) steroids (given to all women 23-34 wks gestation at risk of preterm delivery in next 7 days)
How do you treat RDS?
CPAP
intubate
administer surfactant therapy
Non-TB mycobacteria infections are responsible for what? (what do they cause the most)
chronic lung infections (account for ~90% of pt encounters due to NTM)
Is MAC able to be transmitted from human-to-human or animal-to-human, like TB?
No
What are signs and sx of MAC?
looks like TB but less severe, often indolent
- fatigue, malaise, weakness
- cough-productive or dry
- dyspnea, chest discomfort
- fever and weight loss less common that with TB
Lung exam often nl
What are the 2 major clinical presentations of MAC? (how do their CXRs look)
- In those w/ underlying lung dz
- primarily white, middle-aged or elderly men, often alcoholics and/or smokers w/ underlying COPD
- disease resembles TB clinically and radiographically by usu. less severe - In those w/o underlying lung disease
- primarily nonsmoking women over age 50 who have interstitial pattern of CXR
What is required for clinical dx of MAC pulmonary dz?
- pulmonary symptoms, nodular or cavitary opacities on CXR, or high-resolution computed tomography scan that shows multifocal bronchiectasis w/ multiple small nodules
AND
2. Appropriate exclusion of other diagnoses
Do all pts with MAC pulmonary disease need tx?
No
anti-mycobacterial tx is prolonged, drugs can be difficult to tolerate, long-term response rates modest- may decide to monitor closely
What abx are usually given for MAC pulmonary dz? How long is it given for?
Azithromycin + Rifampin + Ethambutol
Continued until sputum cultures are consecutively negative for at least 12 months (so usually total 15-18 months)
Another name for Interstitial lung disease
pulmonary fibrosis
What are the 3 ways the lung is affected in interstitial lung dz?
- lung parenchyma is damaged (by known or idiopathic cause)
- walls of the alveoli become inflamed (bronchioles or capillaries may be involved)
- scarring (fibrosis) begin in the interstitial (tissue between the alveoli and lung becomes stiff
Interstitial lung dz etiology/associations
- Diseases (sarcoidosis, connective tissue dz, infections)
- Exposures (asbestos, coal dust, aluminum)
- Drugs (nitrofurantoin- Macrobid, amiodarone, statins, chemo meds)
- Idiopathic (idiopathic pulmonary fibrosis, sarcoidosis)
- Infections
If your pt has interstitial lung dz, what would their complaints be?
progressive dyspnea on exertion and nonproductive cough
wheezing and chest pain are uncommon!!
PE findings for a pt w/ interstitial lung dz
- crackles
- inspiratory squeaks
- cor pulmonale
- cyanosis
- digital clubbing
What are 3 things that could be seen on a CXR of a pt with interstitial lung dz?
- ground glass appearance often early finding (hazy opacity, assoc. w/ inflammation)
- reticular “netlike” (MOST COMMON), nodular, or mixed pattern of opacities (BILATERAL)
- honeycombing (small cystic spaces) indicates poor prognosis
What defect pattern do most interstitial lung disease show on pulmonary function tests?
How are TLC, FEV1, FVC, and FEV1/FVC ratio changed?
Restrictive defect
- decreased: total lung capacity
- decreased flow rates (FEV1 and FVC) but changes proportional to diminished lung volumes, so FEV1/FVC ratio is nl or increased
What is FVC?
Forced vital capacity= amount of air forcefully exhaled after max inspiration
In Interstitial lung dz, what happens to DLCO? what is it/what does it mean
REDUCED diffusing capacity of lung for carbon monoxide (DLCO)- common in interstitial lung dz
-diffusing capacity= ability of gas to cross from air to interstitial to blood
What is the gold standard for dx of Interstitial lung dz? When would it be performed?
Lung biopsy
- provide specific dx
- assess disease activity
- exclude neoplastic or infectious process
Complications of Interstitial lung dz
- pulmonary htn -> cor pulmonale -> R heart failure
- PTX
- elevated CA risk
- Progressive respiratory insufficiency
What is Pneumoconiosis?
any dz of the respiratory tract due to inhalation of dust particles (asbestosis, silicosis, coal worker’s dz, berylliosis)
Asbestos and relation to Asbestosis.
- how long of exposure?
- more in males or females?
- used extensively until when?
10-15 years of exposure
Males > females (due to work hx)
until 1960s
What is Mesothelioma and how does it differ from Asbestosis?
A form of CA almost always a/w asbestos exposure
-exposure 1-2 yrs
vs asbestosis which is 10-15 yrs
-poor prognosis
What sign/sx would a pt show if they have Asbestosis?
-no specific sign/sx
- insidious onset
- dyspnea, reduced exercise tolerance
- dry cough
- inspiratory crackles
- sometimes digital clubbing
On CXR, what finding would you see in a pt with Asbestosis?
- Opacities in lower lungs
- Thickened pleura
- Pleural plaques
What pattern do PFTs show in Asbestosis?
Restrictive pattern
- decreased TLC
- FEV1/FVC ratio nl
What do you do for a pt with Asbestosis? (management)
What is the goal?
- consult pulmonology
- smoking cessation
- eliminate exposure
- no effective therapy
Goal: eliminate progression of sx, reduce risk of other lung disorders
“Al Asbestosis”
male
60ish
construction worker
smoker- making things worse
pleural plaques on CXR
Prognosis of damage in Asbestosis
What will smoking do?
lung damage is irreversible
smoking increases risk of lung CA 59x
What is sarcoidosis? what is the cause?
Multisystem inflammatory disease
unknown etiology
What is characteristic of Sarcoidosis?
Noncaseating granulomas- predominantly in the lungs (noncaseating= no necrosis)
Who is most at risk for Sarcoidosis?
- 10x greater in African American
- peak in 20-40 yr olds
- slight female predominance
Of the pt’s who have sarcoidosis, how many resolve/how many develop chronic dz?
2/3 resolve spontaneously
1/3 develop chronic dz
What complaints are associated with sarcoidosis?
- 45% have systemic complaints (fever, anorexia, arthralgias)
- Pulmonary- dyspnea on exertion, cough, CP
- Extrapulmonary (arthritis, cranial nerve palsies, visual disturbances, erythema nodusum)
What are the different stages of Sarcoidosis seen on CXR?
Stage 0= normal
Stage 1= hilar adenopathy
Stage 2= hilar adenopathy + diffuse infiltrates
Stage 3= only diffuse parenchymal infiltrates
Stage 4= pulmonary fibrosis
What would you see on PFTs in a pt with Sarcoidosis?
- Isolated decreased DLCO
- Restrictive pattern w/ advanced dz
What is the most common pattern of lymphadenopathy in Sarcoidosis on CXR?
Bilateral symmetric hilar and R paratracheal mediastinal adenopathy
What 4 lab studies would be elevated in a pt with Sarcoidosis?
- Hypercalcemia
- increased ESR
- increased protein (excess immunoglobulins)
- increased serum ACE in 60%
Dx of Sarcoidosis requires what in most cases?
Biopsy
Sally Sarcoidosis
Female
African American
30ish
non-smoker
- hilar adenopathy
- She’s and ACE
Sarcoidosis management:
What do you do initially?
For non severe dz?
For severe?
- Consult Pulmonologist
- NSAIDs (75% require only symptomatic tx)
- Corticosteroids= mainstay of therapy for severe dz
What is Granulomatosis w/ Polyangitis (aka Wegener’s Granulomatosis)?
How is it characterized?
immune-mediated, systemic vasculitis
characterized by necrotizing granulomas of the upper and lower respiratory tracts
Besides the lungs, what other organ sx is usually involved with Granulomatosis w/ Polyangitis?
75% have renal involvement/ glomerulonephitis
Who has highest prevalence of Granulomatosis w/ Polyangitis?
40-50s
White
Men= women
What symptoms are most common in pts with Granulomatosis w/ Polyangitis?
Upper airway sx!= most common
- rhinorrhea, purulent/bloody nasal discharge
- oral/ nasal ulcers
Lower airway sx:
-dyspnea, cough, pleuritic pain, hemoptysis
What would you expect in PFTs of a pt with Granulomatosis w/ Polyangitis?
- Restrictive and obstructive patterns found
- Decreased DLCO
- Decreased lung volumes when diffuse interstitial involvement present
What would you see on CXR of a pt with Granulomatosis w/ Polyangitis?
CXR: usu. abnormal but highly variable
- opacities
- nodules which can cavitate
In Granulomatosis w/ Polyangitis, what sign is seen on CT?
what else?
“Vasculitis sign”= irregular and stellate-shaped peripheral pulmonary arteries
Also, blood vessels leading to nodules and cavities (feeding vessels)
What lab test would be positive that is more specific for Granulomatosis w/ Polyangitis?
+ ANCA (Antineutrophil cytoplasmic antibodies)
“Gary Granulomatosis w/ Polyangitis”
Male or female
40-50
Upper airway sx (chest the nose!)
Goes to AA (+ ANCA antibodies)
- Vasculitis sign
- renal involvement
What is done for management of pts with Granulomatosis w/ Polyangitis?
Consult Rheumatology
-May tx w/ immunosuppressant (methotrexate or cyclophosphamide) and glucocorticoid
What is another name for Idiopathic Pulmonary Fibrosis?
Is it common?
Unusual interstitial pneumonia
Most common of the 4 idiopathic interstitial pneumonias
What is Idiopathic Pulmonary Fibrosis characterized by?
Most at risk?
inflammation and fibrosis of lung parenchyma
~60 yrs
Slight male predominance
70% smokers
rare familial cases
How do you diagnose Idiopathic Pulmonary Fibrosis? Are there specific findings?
No specific findings
Diagnosis of exclusion
How does Idiopathic Pulmonary Fibrosis present symptomatically (most common sx/sudden or progressive)?
PE?
Insidious onset
- exertional dyspnea= most common!
- nonproductive cough
PE:
inspiratory crackles
digital clubbing (20-50%)
In Idiopathic Pulmonary Fibrosis, what would you see on CXR?
Bilateral diffuse reticular or reticulonodular infiltrates, periphery and bases
Besides CXR, what other studies could you do to help dx? What would they show
HRCT (patchy infiltrates, reticular opacities, honeycombing- poor prognosis)
PFTs- restrictive pattern
“Ivan IPF”
male
60ish
a common man
smoker
What is the most common indication for lung transplant?
Idiopathic Pulmonary Fibrosis
