Restrictive Lung Disease

Question 1

Other words for restrictive lung disease

Answer 1

Interstitial, diffuse lung disease

Question 2

What is the lung interstitium

Answer 2

The connective tissue space around airways and vessels and between basement membranes of alveolar walls. In normal they’re in contact

Question 3

Signs of restrictive lung disease

Answer 3

Reduced lung compliance, low FEV1 and FVC but normal ratio, reduced gas transfer (Tco and Kco), ventilation perfusion imbalance

Question 4

How is diffuse lung disease found

Answer 4

Abnormal CT or CXR, dyspnoea, respiratory failure, heart failure

Question 5

Diffuse alveolar damage associations

Answer 5

Major trauma, chemical injury, circulatory shock, drugs, infection, radiation, autoimmune

Question 6

How many ribs can you see in a normal CXR

Answer 6

Posterior 10 ribs (less with restrictive, more with obstructive)

Question 7

Exudative stage of DADS

Answer 7

Edema and inflammation and dense eosinophilic hyaline membranes

Question 8

Proliferative stage of DADs

Answer 8

Interstitial inflammation and fibrosis

Question 9

Histological features of DADs

Answer 9

Protein rich oedema, fibrin, hyaline membranes, denuded basement membranes, epithelium and fibroblast proliferation, scarring

Question 10

Sarcoidosis

Answer 10

A multisystem granulomatous disorder of unknown aetiology

Question 11

Histopathology of sarcoidosis

Answer 11

Epithelial and giant cell granulomas, associated fibrosis, little lymphoid infiltrate

Question 12

Who does sarcoidosis affect

Answer 12

Young adults, more females than men, more African Americans in USA, in temperate climates

Question 13

Organs involved in sarcoidosis

Answer 13

Lymph nodes, lung, spleen, liver, skin eyes skeletal muscle, bone marrow, salivary glands

Question 14

How does sarcoidosis present in young adults

Answer 14

Painful joints, swollen fat under skin, bilateral lymphadenopathy

Question 15

Antigens in hypersensitivity pneumonitis

Answer 15

Thermophilic actinomycetes, bird/animal proteins eg faeces, fungi, chemicals

Question 16

Acute Hypersensitivity pneumonitis presentation

Answer 16

Fever, dry cough, myalgia, chills, crackles, tachypnoea, wheeze, precipitating antibody

Question 17

Chronic hypersensitivity pneumonitis presentation

Answer 17

Insidious, malaise, SOB, cough, crackles and some wheeze

Question 18

Histopathology of hypersensitivity pneumonitis

Answer 18

Type 3 and 4 immune complex mediated hypersensitivity reactions, soft Centri-acinar epitheloid granulomas, Upper zone

Question 19

Causes of usual interstitial pneumonitis

Answer 19

Connective tissue diseases, drug reaction, post infection, industrial exposure. But most are idiopathic

Question 20

Histopathology of UIP

Answer 20

Patchy interstitial chronic inflammation, type 2 pnuemocyte hyperplasia (surfactant), smooth muscle and vascular proliferation. Evidence of both old and new injury

Question 21

UIP in clinic

Answer 21

Idiopathic Pulmonary fibrosis

Question 22

IPF signs

Answer 22

Elderly and m>f, dyspnoea, cough, basal crackles, cyanosis, clubbing. Most dead within 5 years. CXR shows basal posterior fibrosis and honeycombing, reduced gas transfer

Question 23

Physiological definition of restriction

Answer 23

Forced vital capacity <80% of normal

Question 24

Parts of the body that can cause restrictive disease

Answer 24

Lungs, pleura, nerve, muscle, bone

Question 25

Pleural causes of restriction

Answer 25

Pleural effusion, pnuemothorax, pleural thickening

Question 26

Skeletal causes of restriction

Answer 26

``` Kyphoscoliosis Ankylosing spondylitis Thoracoplasty Rib fractures Amyotrophic lateral sclerosis ```

Question 27

Sub diaphragmatic causes of restriction

Answer 27

Obesity | Pregnancy

Question 28

What is the interstitium

Answer 28

Space between alveolar epithelium and capillary endothelium, usually thin

Question 29

What are interstitial lung diseases

Answer 29

Diseases causing thickening of the interstitium and which can result in pulmonary fibrosis (sarcoidosis, idiopathic pulmonary fibrosis, hypersensitivity pneumonitis)

Question 30

What is sarcoidosis

Answer 30

A multisystem granulomatous disease of unknown cause. Non caseating granuloma

Question 31

Symptoms of sarcoidosis

Answer 31

Erythema nodosum, swollen eye (anterior uveitis), skin lesions, cough

Question 32

Investigations for sarcoidosis

Answer 32

History and exam and CXR Then pulmonary function tests, bloods, urinalysis, ECG, TB skin test, eye exam Then bronchoscopy including biopsies and EBUS

Question 33

CXR signs of sarcoidosis

Answer 33

Nodal adenopathy, snow storm look,

Question 34

Surgical biopsies for sarcoidosis

Answer 34

Mediastinoscopy and VATS

Question 35

How should mild sarcoidosis be treated (no vital organ involvement, normal lung function,few symptoms)

Answer 35

No treatment

Question 36

How should sarcoidosis with erythema nodosum or arthralgia (joint pain) be treated

Answer 36

NSAIDS

Question 37

How should sarcoidosis with skin lesions/anterior uveitis/ cough be treated

Answer 37

Topical steroids

Question 38

How should sarcoidosis with cardiac/neurological /eye disease not responding / Hypercalcaemia be treated

Answer 38

Systemic steroids

Question 39

Respiratory complications of sarcoidosis

Answer 39

Progressive respiratory failure Bronchiectasis Aspergilloma, haemoptysis, pnuemothorax

Question 40

Typical presentation of idiopathic pulmonary fibrosis

Answer 40

Chronic breathlessness and cough, 60-70 years old, failed infection/left ventricular failure treatment / clubbed fingers and crackles

Question 41

Idiopathic Pulmonary fibrosis CXR

Answer 41

Fluffy base

Question 42

Median survival for IPF

Answer 42

3 years

Question 43

Treatment for IPF

Answer 43

Lung transplant. Possibly oral anti fibrotics eg pirfenidone, nintedanib, palliative care

Question 44

Causes of hypersensitivity pneumonitis

Answer 44

Hay, birds etc