Restrictive Pulmonary Disease

Question 1

restrictive lung disease

Answer 1

difficulty fully expanding one’s lungs with air

Question 2

what is restrictive lung disease often a result from

Answer 2

• condition causing stiffness (reduced compliance) in the lungs
• sometimes stiffness of the chest wall, weak muscles, damaged nerves

Question 3

symptoms of restrictive lung disease

Answer 3

SOB

dry or non-productive cough

Question 4

restrictive lung disease characterized by

Answer 4

• reduced lung volume: decreased total lung compliance, FEV1 remains unchanged, FEV1 to FVC preserved
• reduced compliance

Question 5

internal causes of restrictive lung disease

Answer 5

• destruction or scarring of distal lung parenchyma due to infiltrates from inflammation, toxins, etc.
• idiopathic fibrotic disease
• CT disease
• drug-induced lung disease
• environmental exposure
• sarcoidosis

Question 6

external causes of restrictive lung disease

Answer 6

• kyphoscoliosis
• pleural conditions like effusions, pleural scarring
• obesity
• neuromuscular disease
• ascites
• altered mechanical function induced by deformations of the rib cage

Question 7

pathophysiology of restrictive lung disease

Answer 7

inflammation –> recruitment of fibroblasts –> pulmonary fibrosis –> sclerotic injury to alveolar walls –> imposing physical barrier to gas exchange –> decreases DLCO and ability of the alveoli to expand

Question 8

obesity hypoventialtion syndrome

Answer 8

• pickwickian syndrome
• obese pt with sleeping disordered breathing
• hypoventilation
• daytime hypercapnia
• daytime hypoxemia

Question 9

prognosis of restrictive lung disease

Answer 9

• varies depending on the etiology of pulmonary restriction
• pt’s diagnosed with interstitial pulmonary fibrosis have a median survival of 3-5 years after
• acute interstital pneumonia, 70% mortatlity and leads to ARDS

Question 10

physical exam of restrictive lung disease

Answer 10

• lung sounds
• cyanosis
• digital clubbing
• cor pulmonale
• decreased tactile fremitus, dullness upon percussion, decreased intensity of breath sounds

Question 11

medical management of restrictive lung disease

Answer 11

• symptom management
• corticosteroids
• anti-inflammatories

Question 12

treatment for restrictive lung disease

Answer 12

• insuring adequate oxygen, maintaining an airway and obtaining maximal function
• surgical modification of MSK deformities
• heart-lung transplants
• not reversible

Question 13

pneumoconiosis

Answer 13

• lung disease caused by breathing in certain kinds of dust particles that damage your lungs
• inflammation, coughing, fibrosis
• blood vessel and alveolar damage-interface becomes thicker and stiffer

Question 14

interstitial fibrosis

Answer 14

inflammation and fibrosis of the pulmonary interstitium

Question 15

interstitial lung disease

Answer 15

large group of disorders, most of which cause scarring of lung tissue

Question 16

types of interstitial lung disease

Answer 16

• exposure related
• autoimmune related
• idiopathic

Question 17

examples of pneumoconiosis

Answer 17

black lung disease
brown lung disease
asbestoses
silicosis

Question 18

pneumoconiosis signs and symptoms

Answer 18

• range in severity
• cough
• phlegm
• SOB
• progressive respiratory failure
• lung cancer
• TB
• heart failure

Question 19

treatment for chronic interstitial diseases

Answer 19

meds
treat complications
chest physiology
exercise

Question 20

treatment for chronic interstitial diseases

Answer 20

meds
treat complications
chest physiology
exercise

Question 21

relationship btw static lung volume and chronic interstitial disease

Answer 21

• decreased secondary to increased lung stiffness and decreased number of alveoli

Question 22

honeycomb lung

Answer 22

wide spread fibrous

dilated and thickened terminal and respiratory bronchioles

Question 23

why does a neuromuscular chest wall disorder result in restrictive lung disease

Answer 23

affects nerves that control respiration

Question 24

why does skeletal deformities of the chest wall result in restrictive lung disease

Answer 24

osteoporosis

Question 25

how does obesity result in restrictive lung disease

Answer 25

limited space for thoracic cavity to move

Question 26

how does collagen vascular disease result in restrictive lung disease

Answer 26

connective tissue disease –> fibrosis –> restrictive

Question 27

idopathic pulmonary fibrosis

Answer 27

• progressive interstitial pulmonary fibrosis and respiratory failure
• environmental factors: smoke
• genetic factors: smoking + gene variants
• age: rare under 50

Question 28

how does idopathic pulmonary fibrosis begin

Answer 28

• insidiously with gradual increasing dyspnea on exertion and dry cough
• unpredictable progression

Question 29

signs/symptoms of idiopathic pulmonary fibrosis

Answer 29

hypoxemia
cyanosis
clubbing

Question 30

prognosis of idiopathic pulmonary fibrosis

Answer 30

median survival 3 yrs after dx

Question 31

therapy for idiopathic pulmonary fibrosis

Answer 31

lung transplant

Question 32

RA and pulmonary involvement

Answer 32

30-40% have pulmonary involvement

- chronic pleuritis, pneumonitis & fibrosis, pulmonary HTN

Question 33

systemic sclerosis (scleroderma)

Answer 33

diffuse interstital fibrosis

Question 34

systemic lupus and pulmonary involvement

Answer 34

• about 50% of peope with SLE will experience lung involvement during the course of their disease
• chronic, long term pneumonitis
• pulmonary HTN

Question 35

pleuritis

Answer 35

inflammation of the pleura

Question 36

pneumonitis

Answer 36

general term that refers to inflammation of the lung tissue - lung scarring