lec 6+7 restrictive lung disease

Question 1

Restrictive pulmoanry diseases:

Answer 1

-predominantly diffuse
-acute- ARDS
-Chronic involves fibrosis of pulmonary connective tissue

Question 2

pulmonary function tests compared to obstructive:

Answer 2

-FEV1 slightly decreased
-FVC significantly decreased

FEV1/FVC close to normal or sometimes increased

Question 3

3 Characteristics of restrictive disease:

Answer 3

-inspiratory dyspnea
-X ray: bilateral diffuse pulmonary infiltrates
-resp failure, pulmonary hypertension, honeycomb lung

Question 4

Pathogenisis of restrictive lung disease:

Answer 4

-injury of type 1 epithelium—-alveolitis
-Macrophage activation —- fibrogenic cytokines recruitment, inducing fibrosis.

Question 5

Later stage of ARDS:

Answer 5

-Macrophage derived fibrogenic factors
-fibroblast recruitment—- fibrosis

These can be counteracted by antiproteases,antioxidants,anti-inflammatory cytokines.

clotting cascade is also activated

Question 6

Fibrosing reaction: 1)idiopathic pulmonary fibrosis

Answer 6

-M>F, older than 60
-diagnosis of exclusion
-severe hypoxemia and cyanosis

-histological pattern of usual interstital pneumonia UIP

Question 7

Clinical features of IPF:

Answer 7

-Insidious presentation
-Non productive cough and severe dyspnea

Question 8

Examination of IPF:

Answer 8

-Cyanosis, clubbing, dry crackle inspirations
- Chest x-ray: bilateral basal nodular infiltrates then progresses to whole lung later
-Lung biopsy+* bronchoalveolar lavage* (applying fluid into trachea then collecting it)

prognosis: death in 2-4 years

Question 9

Morphology of IPF:

Answer 9

-cobblestone surface
-interstitial chronic inflammation
-Temporal heterogeneity temporal=time Heterogen= diverse
-basal, along pleura and septa
- patchy fibrosis, irregular cystic fibrosis lined by hyperplastic type 2 pneumocytes or bronchiolar lining (honeycomb lung)

Question 10

2)non specific interstitial pneumonia:

Answer 10

-Abscence of temporal heterogeneity.
-wastebasket diagnosis, of ANY pneumonia
-mature fibrosing pattern
-cellular pattern: infiltrate

Question 11

3)cryptogenic organizing pneumonia:

Answer 11

-no temporal heterogeneity, interstital inflammation
-polypoid plugs fibrosis in bronchioles and alveolar ducts and alveoli
-no destruction of lung architecture
-recovery within 6 months with steroids

Question 12

4)collagen vascular disease

Answer 12

rheumatoid arthritis
SLE
scleroderma
any interstitial pattern

can trigger systemic inflammation in lung, causing lung fibrosis

Question 13

5)pneumoconiosis/environmental diseases

Answer 13

-non neoplastic lung reaction exposure to coal , silica, asbestos dusts
- Particles 1-5 micrometers in diameter, most dangerous. because they get loaded at distal airways .
- tobacco smoke worsens it.

Question 14

Types of pneumoconiosis: Anthracosis (coal miners lung)

Answer 14

-seen in tobacco smokers
-inhaled carbon pigment
-No increase in risk of lung cancer

Question 15

1)simple coal worker pneumoconiosis

Answer 15

coal macule: consist of dust laden-macrophages

Coal nodule: larger and contains collagen

upper lobe more involved than lower lobe: centrilobular emphysema might occur

Question 16

2)complicated CWP

Answer 16

we have multiple nodules, then they coalesce, forming a large nodule (2-10cm)

Question 17

3)progressive massive fibrosis:

Answer 17

pulmonary dysfunction
pulmonary hypertension
Cor pulmonale

Question 18

B-sillicosis:

Answer 18

inhaling crystalline silica: quartz.
acute heavy exposure— ARDS
chronic after 20-40yrs exposure

Question 19

pathogenisis of sillicosis:

Answer 19

-sillica in macrophages — injury to cell membrane
- starts as small nodules, then enlarges to 1-10cm

• polarized microscopy shows weakly befringent silica particles
  -X ray shows: egg shell calcification
  -honeycomb

Question 20

Clinical picture of sillicosis:

Answer 20

-asymptomatic
-inc chance of lung cancer and TB

Question 21

C) asbestos induced lesions

Answer 21

-2 types: Serpintene and amphibole

-Lesions include:
1)asbestosis, lower lobe–honeycomb lung and cor pulmonale
2)pleural effusion and fibrosis
3)pleural plaques:most common
4)malignant mesothelioma
5)**inc risk of lung CA and larynx **

Question 22

Drug and radiation induced pulmonary diseases:

Answer 22

from cancer chemotherapy(bleomycine)

-anti arrythmic patients (amiodarone)

Question 23

1) Sarcoidosis

Answer 23

multisystem disorder
age younger than 40

etiology:
genetic predisposition (HLA-A1) and (HLA-B8).

Question 24

immune mechanisms of Sarcoidosis:

Answer 24

-Type IV hypersensitivity reaction

-T lymphocyte abnormalities:
- oligoclonal expansion of T cell subsets
- decreased CD4/CD8
- alveolar lavage: inc T cells
- Inc level of IL-8, TNF, MIP-1a locally
- polyclonal hypergammablobulinemia

Question 25

pathology of Sarcoidosis:

Answer 25

-non caseating granulomas eith giant cells containing schaumann and asteroid bodies.
-Occular involvment = SICCA syndrome
-Parotid involvment= MIKULICZ syndrome

Question 26

Clinical picture and radiograph of sarcoidosis:

diagnosis

Answer 26

fever, dyspnea

chest radiograph shows;
bilateral hilar lymphadenopathy
ground glass miliary shadows in both lungs
hypercalcemia

diagnosed by exclusion of all other granulomatous conditions

Skin test: KVEIM test

Question 27

hypersensitivity pneumonitis:

Answer 27

-type III and type IV reactions
-immunologically affects airways and interstitium
- can be farmers lung , pigeon breeder, air condition lung

Question 28

the way hyeprsensitivity pneumonitis is presented in questions:

Answer 28

-pigeon breeder /farmer lung
-asthma attack, dyspnea, fever
-morphology: poorly formed epitheliod granulomas (non caseating)