Resp interstitial

Question 1

Indications for corticosteroid treatment for sarcoidosis are:

Answer 1

parenchymal lung disease,
uveitis,
hypercalcaemia and
neurological or cardiac involvement

Question 2

Compliance define

Answer 2

is defined as the volume change produced by a change in the distending pressure:
ΔV/ ΔP

Question 3

Define elastic recoil

Answer 3

lung’s tendency to collapse and push the air back out

Question 4

What happens during inhalation

Answer 4

diaphragm & intercostal muscles contract to pull the ribs up and out and expand the chest cavity. = vacuum that pulls the lungs open to allow air in

Question 5

Decreased compliance and increased recoil of lung indicates

Answer 5

Restrictive
diffuse parenchymal lung diseases or DPLDs (interstitial lung diseases), the lung tissue itself is damaged. = fibrotic, rigid lung with reduced compliance and increased recoil
-doesnt allow air to enter during inhalation, thereby reducing lung volumes.

Question 6

FVC define

Answer 6

air exhaled forcefully after taking a deep breath.

Question 7

What is residual volume or RV? How is it measured?

Answer 7

air left in the lungs after exhaling as hard as possible
plethysmography

Question 8

Define total lung capacity or TLC

Answer 8

FVC + RV

Question 9

FRC define

Answer 9

volume of air that remains in the lungs after normal expiration.
FRC= RV + expiratory reserve,

Question 10

Define PFT’s for restrictive lung d’s (5)

Answer 10

1. Decrease FVC
2. Decrease RV
3. Reduction TLC, (FVC+RV)
   4.Decrease FRC
   5.Decrease in FEV1,
4. FEV1/FVC ratio= same or increased (increased elastic recoil)

Question 11

Name the Diffuse parenchymal lung diseases or DPLDs (interstitial lung diseases)

Answer 11

1. Granulomatous diseases,(sarcoidosis,hypersensitivity pneumonitis)
2. Occupational exposures, (asbestosis, silicosis, berylliosis, and coal workers’ pneumoconiosis;
3. miscellaneous diseases( RA, GPA, Goodpasture syndrome, pulmonary Langerhans cell histiocytosis,)
   4.unknown cause-idiopathic pulmonary fibrosis.

Question 12

Explain type 4 hypersensitive reaction in granuloma development

Answer 12

Type 4 hypersensitive rnx
1. antigen picked up APC, (dendritic cell or an alveolar macrophage)
2. APC then presents the antigen to a CD4+ T-helper cells, APC secrete **IL-12 **of a cause CD4+ receptor of Th cell, causing it to differentiate Th1 cell.
3. Th1 cells to start secreting IL-2, =T cells in the area proliferate,+ interferon gamma, which activates phagocytes like macrophages.
4. The activated macrophages, now called epithelioid macrophages surround the antigen, =a ball-like nodule called a granuloma, which is meant to “wall off” the antigen and prevent it from spreading.
5. when several activated macrophages fuse together. = , and have multiple nuclei, which are arranged peripherally in the shape of a horseshoe.

Question 13

Why does Sarcoidosis have raised Ca2+

Answer 13

macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)

Question 14

Sarcoidosis presentation to
- eye
-Hrt
-Joint

Answer 14

• Uveitis
• Dilatative or restrictive
• RA

Question 15

Sarcoidosis presentation to
-liver

Question 16

Sarcoidosis presentation in the liver

Answer 16

hepatomegaly, abdominal pain, cirrhosis or cholestatic liver disease with jaundice.

Question 17

Signs of neurosarcoidosis

Answer 17

Bell’s palsy

Question 18

What do find on BAL in sarcoid?

Answer 18

elevated CD4+/CD8+

Question 19

Poor prognosis Sarcoidosis (5)

Answer 19

1. insidious onset, symptoms > 6 months
2. absence of erythema nodosum
3. extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
4. CXR: stage III-IV features
5. black African or African–Caribbean ethnicity

Question 20

Stages of Sarcoidosis (0-4)

Answer 20

• stage 0 = normal
• stage 1 = bilateral hilar lymphadenopathy (BHL)
• stage 2 = BHL + interstitial infiltrates
• stage 3 = diffuse interstitial infiltrates only
• stage 4 = diffuse fibrosis

Question 21

What is the role of ACE levels in Sarcoidosis?

Answer 21

ACE levels have a sensitivity of 60% & specificity of 70%. Thus not reliable in the diagnosis of sarcoidosis although they may have a role in monitoring disease activity.

Question 22

Extrinsic allergic alveolitis/ Hypersensitivity pneumonitis
Is what type of hypersensitivity?

Answer 22

caused by immune-complex mediated tissue damage (type III hypersensitivity immune complex mediated) although delayed hypersensitivity (type IV) is also thought to play a role

Question 23

Examples Extrinsic allergic alveolitis (4)

Answer 23

1. bird fanciers’ lung: bird droppings
2. Farmers lung: spores of Saccharopolyspora rectivirgula from wet hay (formerly Micropolyspora faeni)
3. Malt workers’ lung: Aspergillus clavatus
4. Mushroom workers’ lung: thermophilic actinomycetes*

Question 24

Explain type III hypersensitivity immune complex mediated reaction in Examples Extrinsic allergic alveolitis

Answer 24

1. Antigen is picked up by dendritic cells or alveolar macrophages which take it to the nearest lymph node, where they present it to Th1 cells.
2. Th1 cells then activate B cells to produce IgG antibodies that go into the bloodstream,
3. IgG antibodies meet the antigens= immune complexes.

Question 25

Ix Extrinsic allergic alveolitis
Imaging
BAL
Serology
Blood

Answer 25

Investigation
imaging: upper/mid-zone fibrosis
bronchoalveolar lavage: lymphocytosis
serologic assays for specific IgG antibodies
blood: NO eosinophilia

Question 26

Rx Extrinsic allergic alveolitis

Answer 26

Steroids, avoid triggers

Question 27

How can you differentiate Extrapulmonary restrictive Lung diseases from other restrictive lung diseases?

Answer 27

will have decreased inspiratory volumes BUT
Because the lungs are healthy, both the diffusing capacity and A-a gradient are normal

Question 28

List the 2 categories Extrapulmonary restrictive Lung disorders

Answer 28

1. Weakness of the respiratory muscles- polio, myasthenia gravis, GBS
2. Structural abnormalities, like scoliosis, morbid obesity & ankylosing spondylitis,

Question 29

Dx idiopathic pulmonary fibrosis? PFTs,
CT
Lung biopsy

Answer 29

Dx of exclusion:
No hx of pul d’s
PFTs = restrictive pattern

CT= honeycomb pattern associated with pulmonary fibrosis

Lung biopsy showing dense fibrosis, fibroblast proliferation, and cyst formation

Question 30

Name the 4 Occupational lung disease Lung Infection

Answer 30

Asbestosis
Silicosis
Berylliosis
Coal miners lung

Question 31

Extrapulmonary restrictive Lung diseases 2 main causes

Answer 31

Weaken resp muscles : Polio, Myasina gravis, GBS
Structural defect: sclerosis, obesity, AS

Question 31

What caused Asbestosis

Answer 31

Ship building, textiles, plumbing & roofing

Question 32

Lung bases showing Bilateral reticular opacities & Plural plaques

Answer 32

Asbestosis
Roofs affect the bases

Question 33

What exposure increases risk of mesothelioma and very increased risk ofbronchogenic carcinoma

Answer 33

Asbestosis

Question 34

What causes:
Nodular opacities in both lungs & classic “eggshell” calcification of hilar lymph nodes, usually affecting the upper lobes
or
fibrotic scar with a classic onion-skin

Answer 34

Silicosis

Question 35

What are Silicosis pts more at risk of developing

Answer 35

TB

Question 36

restrictive pattern on PFTs, and a chest x-ray showing small, rounded nodular opacities typically affecting the upper lobes.

Answer 36

Coal workers lung

Question 37

causes of upper zone fibrosis:

Answer 37

C - Coal worker’s pneumoconiosis
H - Histiocytosis/ hypersensitivity pneumonitis
A - Ankylosing spondylitis
R - Radiation
T - Tuberculosis
S - Silicosis/sarcoidosis