Restrictive Lung Diseases (Zaman) Flashcards
What type of lung DX has normal lung volume?
Dec lung volume?
Inc lung volume?
vascular (PAH) obstructed airway (asthma, COPD) Interstitial disease (fibrosis)
What is the definition of restrictive lung disease?
disease in which the expansion of the lung is limited WITHOUT airway obstruction
What are the classification of restrictinve lung disease
intrinsic/parenchymal= ILD or resection of lung tissue
extrinsic/extra-parenchymal= disorders of pleura, chest wall, or neuromuscular disorders
Pleural Effusion is (intrinsic or extrinsic) lung disease?
extrinsic
What does the “meniscus sign” indicate on CXR?
pleural effusion
What is the lung interstitium?
the extra-vascular and extra-pleural space
What parts of the interstitium does ILD involve?
CT and alveolar wall
T or F: ISD can be infectious or caseous
False: neither but it is due to an inflammatory process
What is the consequence of the inflammation on ILD?
excess growth of CT –> distortion of lung parenchyma –> dysfunction of gas exchange
Describe the structure of normal lung interstitium.
- parenchymal cells = type I and II alveolar epithlial cells, endothelial cells
- Inflammatory cells = macrophages > lymphocytes > neutrophils
- CT elements = fibroblasts, collagen, elastin, proteoglycans
What repopulates type I alveolar epithelial cells when they are damaged?
type II epithelial cells
Describe the structure of lung interstitium in ILD
loss of type I cells, compressed pulm capilaries, increased CT –> thickened alveolar wall
Describe the pathophysiology of ILD
Injury to the alveolar epithelial layer leads to tissue damage and cell death.
–> damage to the basement membrane and the release of a variety of factors: TGF-B, IGF-1, LGF, CTGF, PDGF
–> stimulate fibroblasts to produce extracellular matrix components, particularly collagen
–>fibrous-rich regions impedes the recovery of the normal epithelial cell layer
How do pts compensate for dec long volume assc with ILD?
increased RR
decrease size of breaths = more shallow
How are the etiologies of ILD subdivided?
known and unknown and granulomatous and non-granulomatous
WHat is the likely etiology of pneumonia in a farmer?
hypersensitivity pneumonitis
Drug induced ILD is (granulomatous or non-granulomatous)
non-granulomatous
External radiation induced ILD is (granulomatous or non-granulomatous)
non-granulomatous
What are examples of drugs known to cause ILD
bleomysin, amiroderone, nitrofurantion
What inorganic dusts are granulomatous etiologies of ILD?
non-granulomatous?
granulomatous: berylliosis
non-granulomatous: asbestos, coal, silica
Sarcoidosis is (granulomatous or non-granulomatous)
granulomatous
What are the ILD of unknown etiologies that are non-granulomatous?
idiopathic interstitial pneumonias
ILD with connective tissue diseases (RA, lupus, scleroderma, dermatomyositis)
The hypoxemia of ILD is due to? A. Hypoventilation B. VA/QC mismatch C. Diffusion defect D. Right to left shunt
B. VA/QC mismatch
V = 0
pts with ILD will be (hypo or hyper capnic)
hypocapnic = inc RR to compensate for dec lung volume
ILD pts will have a normal or widened A-a gradient?
widened
ILD pts have exercised induced ____
hypoxia
sorry, didn;t know how to ask this
Will pts wth ILD have.. Reduced expiatory flow? Reduced TLC? Reduced VC? Reduced diffusing capacity?
No
Yes
Yes
Yes
What is the most common symptom of ILD?
dyspnea
What are common signs of ILD?
Clubbing of digits
Tachypnea
Crackles/”velcro” rales
Late DZ: cor-pulomanale, cyanosis
Why is cor pulomonale a consequence of late ILD?
the RV has to work harder to pump blood through the compressed capillaries (due to increased intersititium)
What are the radiological findings assc with early, middle, and late ILD?
early: ground glass
middle: reticular, nodular, reticulo-nodular
late: honeycombing
How is the diagnosis of ILD made?
H&P + imaging studies for comparison + PFTs + BAL and lung biopsy
What is BAL?
Broncho-Alveolar Lavage: stick a bronchoscopre in and flush alveoli with saline and then suction out saline to study contents of alveoli
What are the major inflammatory cells in alveoli?
macrophages (90%)
If more lymphocytes are found than macrophages in a BAL sample what DZ might you expect?
sarcoidosis
How could a pt have decrease lung volume without alteration of the structure of the interstitium?
lung resection (Pneumonectomy)
Name disorders of the pleura that cause ILD?
mesothelioma, pleural effusion, pheumothorax
Name disorders of the chest wall that cause ILD?
Kyphoscoliosis
Pathophysiology of hypoxemia in ILD is due mainly to …
V/Q mismatch.
Describe the clinical features of ILD
dyspnea, tachypnea, hypoxemia, infiltrates, restrictive changes in PFTs
Compliance is (inc or dec) in ILD
decreased
What ILD are fibrosis?
idiopathic pulmonary fibrosis
Nonspecific interstitial pneumonia
Cryptogenic organizing pneumonia
Associated with collagen vascular disease
Pneumoconiosis
Associated with therapies (drugs, radiation)
What ILD are granulomatous?
sarcoidosis
hypersensitivity pneumonia
WHat ILDs are are characterized with the infiltration and activation of eosinophils
Loeffler syndrome
Drug allergy related
Idiopathic chronic eosinophillic pneumonia
What ILDs are smoking related?
Desquamative interstitial pneumonia
Respiratory bronchiolitis
Idiopathic pulmonary fibrosis is (uni or bi lateral)
epidemiology?
bilateral
Males > Females
60+
What is pneumoconioses?
non-neoplastc lung reaction to inhation of mineral dust
