Restrictive and Inflammatory Lung Disease Flashcards
Restrictive physiology is defined by ____
TLC
FRC (TGV)
FEV1 and FVC
reduced lung volumes
TLC < 80% predicted
FRC < 80% predicted
FEV1 and FVC symm reduced
Restrictive physiology may be caused by either (2)
1) incr in lung elastic recoil (stiffness)
2) chest wall or pleural disease
For PV curve of lung, ___ does not affect the slope of this curve
Chest wall changes
Relationship between compliance and elastance
Inverse
Compliance (dV/dP)
Elastance (dP/dv)
Compare obstructive vs. restrictive in terms of compliance
Obstructive = incr compliance (incr volume at given pressure) = hard to exhale
Restrictive = decr compliance (decr volume at given pressure) = hard to inhale
What is work of breathing?
Combined elastic + resistive work
Ptot = Pel + Pr
Pr = resistive work against airflow
Pel = work against elastic recoil
When would you have to work harder to expand your lungs (2)
1) incr lung elastic recoil
2) decr chest wall compliance
3 mechanisms that decr lung compliance (restrictive disease)
1) incr thickness of lung interstitium= inflammation or scarring may expand interstitium
2) incr lung water
3) incr alveolar surface tension
Mechanism of incr thickness of lung interstitium in restrictive disease
example of disease
chronic interstitial lung disease
1) incr deposition of elastic/connective tissue
2) fibroblasts produce XS collagen/elastin in alveoli
3) incr interstitial matrix (incr elastic recoil and stiffens lung)
Mechanism of incr lung water
example of disease
Congestive heart failure, cardiogenic or noncardiogenic pulm edema (incr lung water)
1) fluid leaves capillary and fills interstitium (incr elasticity)
2) fluid fills alveoli, disrupt surfactant, incr surface tension
3) collapses alveoli, decr compliance
Define respiratory distress syndrome in pediatric vs. adult
pediatric = immature lungs –> inadequate surfactant production
adults = ARDS = injury of type 2 alveolar cells –> noncardiogenic pulm edema that dilutes surfactant
how does restrictive lung disease affect TLC, FRC and RV
how does it affect PV curve
how does it affect FEV1/FVC ratio
Decr all
Shift down PV curve
normal or incr FEV1/FVC
how do you measure static compliance in mechanically ventilated patients?
measure pressure to inflate lung to certain volume
How does restrictive disease affect gas exchange
Decr DLCO/gas exchange
1) decr alveolar surface area, decr lung volumes
2) incr diffusion distance, exacerbated with exercise
3) V/Q mismatch
Describe traction bronchiectasisi and effect on Flow rate vs. volume curve
Supranormal airflow for given lung volume due to traction applied from adjacent parenchyma to keep airways dilated
How does pulmonary edema incr elastic recoil?
1) interstitial pulm edema = incr mass and stiffens interstitium
2) alveolar pulmonary edema - deplete surfactant, incr mass and stiffness of interstitium
3) surfactant depletion - incr alveolar surface tension - alveolar collapse (atelectasis)
4) alveolar filling process (pneumonia, alveolar hemorrhage)
What should you be aware of when determining DLCO with VA?
DLCO corrected for VA may miss interstitial lung disease because many interstitial diseases decr both VA and DLCO
Causes of increased lung water that decreases lung compliance
1) ARDS
2) pneumonia
3) cardiogenic pulm edema
Diseases that affect chest wall compliance
1) Burns- thick eschar
2) obesity- incr soft tissue mass, decr compliance
3) kyphoscoliosis- deformity of spine
4) ankylosing spondylitis - ossification of liagments in spine
5) respiratory muscle weakness- paralysis of muscles
6) pleural fibrosis/thickening- restrict expansion of lung
7) pleural effusion = fluid in pleural cavity limit lung expansion
For mild reduction in lung volumes, what does DLCO and VA look like?
DLCO decreases but DLCO is corrected for alveolar volume (VA)
How does FRC change in people with muscular weakness?
FRC is normal (low TLC and incr RV)
how can you distinguish between chest wall and muscular disease from restrictive lung diseases (fibrosis/edema)?
chest wall and muscular disease = slope of P-V is normal and DLCO/VA is normal and decr lung volume (TLC) so shifted down but same shape
restrictive disease = slope is decreased and DLCO/VA does not correct
compare gas exchange in chest wall diseases vs. restrictive lung diseases
chest wall diseases = no gas exchange problem (DLCO decr but DLCO/VA is normal)
restrictive lung disease = loss of alveolar surface area, (both DLCO and DLCO/VA is decreased)
What are examples of acute restrictive proceses?
1) pulm edema
2) ARDS
3) Pneumonia
4) Pleural effusion
What are examples of chronic restrictive proceses?
1) ILD
2) Pleural fibrosis/plaques
3) Pleural Effusion
Describe mixed obstructive and restrictive disease
Types of patients who have mixed obstructive and restrictive
Decreased lung volumes AND dcr FEV1/FVC < 0.70
•coal miner who smokes
construction worker with interstitial lung disease from asbestos or silica exposure who also has a1 antitrypsin deficiency
•obese person with alpha 1 anti-trypsin deficiency
obese person with asthma
- Combined pulmonary fibrosis and emphysema
- Fibrotic hypersensitivity pneumonitis
- Sarcoidosis
how does mixed disease appear on PFT’s?
1) decr TLC or FRC (restrictive)
2) decr FEV1/FVC (obstructive)
3) decr DLCO
Define interstitial lung disease (diffuse parenchymal lung disease)
classified by etiology and pattern
combined inflammation + scarring
classified by etiology and pattern
Causes of ILD can be grouped into 5 groups
1) Autoimmune
2) Exposure to inorganic dusts (asbestos/silica)
3) Exposure to organic molecules –> hypersensitivity pneumonitis
4) Drug effects
5) idiopathic
Breakdown of Interstitial lung diseases
1) ILD due to known cause
2) idiopathic interstitial pneumonia
a) IPF
b) not IPF
- DIP, AIP, NSIP, COP, LIP
3) granulomatous (sarcoidosis)
Most common symptoms of ILD
Rarely, this disease is ____
1) insidious onset of dyspnea on exertion
2) nonproductive cough
rarely, sudden onset unless acute hypersensitivity pneumonitis, acute interstitial pneumonitis, acute eosinophilic pneumonia
How do PFTs look for ILD
restrictive pattern (normal or incr FEV1/FVC, decr TLC and TGV) with decr DLCO
Different causes of ILD
For prognosis of ILD, what disease is most significantly causing decr survival?
UIP
Physical exam in ILD
1) Auscultation = end inspiratory crackles
2) Digital clubbing with advanced
3) Joint and Skin exam (Gottron’s papules, RA, mechanic’s hands)
Treatment optiosn for ILD
1) Remove offending exposure
2) immunosuppressive (corticosteroids and steroid sparing drugs)
3) O2
4) transplantation
More specifically, what is treatment for IPF?
Nintedanib, pirfenidone
Idiopathic Pulmonary Fibrosis
1) Define
2) Etiology
3) Age of patients
4) Associated with?
5) Symptoms
6) Physical exam
1) scarring lung disease with pattern of UIP
2) idiopathic
3) 60 + y/o
4) tobacco use
5) Cough, DOE, fatigue
6) basilar predominant “velcro-crackles”, digital clubbing
Idiopathic Pulmonary Fibrosis
Imaging findings
Pathology findings
Treatment
Imaging:
1) PAUCITY OF GROUND GLASS INFILTRATE
2) peripheral/basilar predominant reticulation
3) traction bronchiectasisi
4) honeycombing
Pathology
1) spatially and temporally heterogeneous fibrosis with fibroblast foci
2) juxtaposition of fiibrotic and normal lung
Treatment
Pirfenidone and nintedanib
NSIP
1) similar presentation to ___
2) cause
3) patient population
4) prognosis
5) treatment
1) ILD with cough and DOE
2) idiopathic, collagen vascular disease (RA, sjogren’s, scleroderma, myositis), fibrotic HP, drug
3) younger female
4) better tha UIP/IPF and collagen vascular is better than idiopathic
5) corticosteroids and steroid sparing
NSIP
Radiographic
Pathology
Radiographic
1) basilar predominant reticular abnormality
2) volume loss
3) traction bronchiectasis
4) frequent ground glass opacity
5) rare honeycombing
Pathology
1) temporally HOMOGENOUS fibrosis with varying degrees of inflamm and fibrosis
Smoking-related ILD’s
1) respiratory bronchiolitis- ILD
2) Desquamative interstitial pneumonia (DIP)
3) Pulmonary Langerhans cell histiocytosis
Pulmonary Langerhans Cell histiocytosis
1) patient population
2) radiology,
3) PFTs
4) other manifestations
5) treatment
1) young smokers
2) cysts and nodules; upper lobe predominant; pneumothorax
3) mixed PFTs (airway + nodule);
4) 15% extrapulm diagnosis = bone lesions, pituitary involvement
5) smoking cessation
Organizing pneumonia = BOOP
1) severity of presentation
2) cause
3) type of pneumonia
4) radiology
5) pathology
6) treatment
1) subacute to chronic
2) idiopathic (COP) or secondary to CVD, drugs, HP, aspiration
3) noninfectious pneumonia
4) ground glass and migratory consolidation
5) organizing pneumonia; plugs of granulation tissue and fibrosis distal to bronchioles
6) steroids 6-12 mo
Acute eosinophilic pneumonia
1) mimics ___
2) diagnosis how?
3) treatment
4) type of pneumonia
1) ARDS
2) diagnosed by BAL showing eosinophils
3) steroids
4) noninfectious = eosinophil in alveoli and interstitium
Chronic eosinophilic pneumonia
1) type of onset
2) assoc with ___
3) radiology
4) diagnosed how?
5) treatment
1) subacute (eosinophils in blood stream unlike acute)
2) asthma
3) peripheral eosinophilia “photographic negative of pulm edema”
4) BAL
5) steroids
Lymphangioleiomyomatosis (LAM)
1) type of lung disease
2) patient population
3) mutaiton
4) mechanism
5) common co-morbidities
6) PFTs
7) treatment
1) cystic
2) young women 20-30
3) mutation in tuberous sclerosis gene 1 or 2
4) peribronchovascular prolif of smooth muscle
5) pneumothorax, chylothorax renal tumors (angiomyolipomas)
6) obstructive pattern
7) mTOR inhibitors (sirolimus)
Sarcoidosis
1) describe
2) path
3) commonly affects
4) common co-morbidities
5) PFTs
6) symptoms
7) patient population
8) radiology
9) treatment
1) systemic granulomatous disorder unknown etiology
2) noncaseating granulomas
3) entire body
4) mediastinal and bilateral hilar lymphadenopathy, abnormal CXR, fibrosis, airway involve
5) normal in stage 1
airflow limitation and decr FEV1/FVC ratio with bronchodilator response
with infiltrative disease, restrictive pattern with decr DLCO
can have mixed
6) cough, chest pain, wheezing, fever, chills, fatigue, wheezing
7) before age 40 in northern Euro descent
8) Lambda sign, interstitial infiltrates and nodules
9) no treatment necessary but can use steroids/cytotoxic
how is diagnosis of sarcoidosis made?
clinical finding and granulomas
Radiographic stages of Sarcoid
Stage 1: Bilateral hilar LAD without infiltration
Stage 2: Bilateral hilar LAD with infiltration
Stage 3: Infiltration without LAD
Stage 4: Fibrotic disease
Lofgren’s syndrome characterized by (3 things)
How do you diagnosis?
PFTs?
Inflammatory arthritis
Erythema nodosum
Bilateral hilar lymphadenopathy
___
Diagnosis by finding well-formed granulomas via bronchoscopy, mediastinoscopy, surgical lung biopsy
____
PFT normal in stage 1
