interstitial ling disease

Question 1

which area exactly is the interstitium of the lung ?

Answer 1

the area between the alveolar epithelium and pulmonary vascular epithelium

Question 2

what are the different groups of interstitial lung diseases?

Answer 2

ILD of known association
granulomatous ILD
Idiopathic interstitial pneumonia
miscellaneous ILD

Question 3

what are the types of exposure related ILD?

Answer 3

pneumoconiosis
drug-induced
connective tissue disease
radiation pneumonitis

Question 4

what kind of drugs can induce ILD ?

Answer 4

amiodarone
methotrexate
nitrofurantoin

Question 5

what are the differential diagnosis with ILD ?

Answer 5

infectious causes :
community acquired pneumonia + atypical pneumonia
chronic or remote mycobacterial infections
non-infectious possibilities:
lymphoproliferative disorders, cardiac pathology, acute respiratory distress syndrome

Question 6

what are the symptoms associated with ILD ?

Answer 6

dyspnea
cough 
fever ( more in an acute attack)
hemoptysis 
chest pain/pleurisy

Question 7

what would a smoking history suggest in terms of ILD ?

Answer 7

Idiopathic pulmonary fibrosis
Respiratory bronchioloitis-associated interstitial lung disease (RBILD)
Desquamating interstitial pneumonitis (DIP)
pulmonary langerhans cell histocytosis (PLCH)

Question 8

which ILD is less likely to be associated with smoking ?

Answer 8

Sarcoidosis and hypersensitivity pneumonitis

hypersensitivity pneumonitis

Question 9

what ILD is associated with bird breeders and farmers ?

Answer 9

hypersensitivity pneumonitis

Question 10

what ILD is associated with ceramic workers?

Answer 10

silicosis

Question 11

what ILD is associated with pipe fitters and shipyard workers?

Answer 11

asbestosis

Question 12

what ILD is associated with coal miners ?

Answer 12

coal workers’ pneumoconiosis

Question 13

what pathology is amiodarone associated with ?

Answer 13

pulmonary fibrosis

Question 14

what pathology is associated with nitrofurantoin?

Answer 14

alveolar haemorrhages

Question 15

what symptoms are more suggestive of collagen vascular diseases ?

Answer 15

Raynaud’s phenomenon
proximal muscle weakness
joint swelling/pain

Question 16

what does mild inspiratory squeak in physical examination suggest ?

Answer 16

suggests airway centred diseases:
constrictive bronchiolitis
hypersensitivity pneumonitis

Question 17

what is the main finding on auscultation with ILD?

Answer 17

classic velcro rales or inspiratory crackles

Question 18

what kind of Lab investigations are ordered for ILD ?

Answer 18

CBC 
liver function
calcium, liver function test and a urine analysis 
along with immunological tests:
ssDNA and complement levels in SLE 
Anti-SCL 70 for diffuse scleroderma

Question 19

what is the current gold standard for imaging in ILD ?

Answer 19

HRCT

Question 20

what are the radiological findings in idiopathic pulmonary fibrosis ?

Answer 20

1- X-ray: reticular opacities
2- HRCT : traction bronchiectasis
bilateral, basal honeycombing

bilateral, basal honeycombing

Question 21

what are the radiological findings in sarcoidosis on X-ray and HRCT ?

Answer 21

HRCT: septal bleeding
nodules next to bronchovascular bundles

X-ray: hilar lymphadenopathy

nodules

Question 22

which ILD show cystic changes with imaging ?

Answer 22

LAM

PLCH

Question 23

what is the hallmark of ILD ?

Answer 23

restrictive changes in pulmonary physiology

Question 24

what is the cause of the hypoxia inn patients with ILD?

Answer 24

ventilation perfusion mismatch

Question 25

what to do if gas exchange appears normal at rest ?

Answer 25

do exercise testing ( 6 minute walk test) to unmask the defects

Question 26

when can bronchoalveolar lavage be useful when it comes to ILD ?

Answer 26

useful in ruling out malignancies and infections

Question 27

bronchoscopic biopsies are of limited utility in the evaluation of ILD except with ....

Answer 27

sarcoidosis

Question 28

what is the treatment option for ILD?

Answer 28

``` providing oxygen to hypoxemic patients pulmonary rehabilitation pneumococcal and influenza vaccination remove offending environmental agents corticosteroids and immunosuppressants ```

Question 29

what specific indications should be taken with LAM patients ?

Answer 29

avoid pregnancy , oophorectomy, progesterone, antiestrogens give sirolimus

Question 30

what is more commonly seen after the age of 50 ?

Answer 30

idiopathic pulmonary fibrosis

Question 31

where is the affection in chronic hypersensitivity pneumonitis ?

Answer 31

upper lobe

Question 32

where is the affection in acute hypersensitivity pneumonitis ?

Answer 32

lower lobe

Question 33

when do we hear an expiratory wheeze refractory to inhaled bronchodilators ?

Answer 33

constrictive bronchiolitis

Question 34

what is the sign seen on HRCT associated with hypersensitivity pneumonitis ?

Answer 34

head cheese sign

Question 35

what is seen on HRCT of lymphangioleiomyomatosis LAM ?

Answer 35

thin walled cysts

Question 36

what is seen on HRCT off PLCH ?

Answer 36

diffuse variable sized bizarre shaped cysts

Question 37

what is PAP ?

Answer 37

pulmonary alveolar proteinosis , accumulation of surfactants within the alveoli

Question 38

what is seen on HRCT in PAP ?

Answer 38

ground glass opacities demarcated by thickened interlobular septae crazy paving ( variable alveolar filling )

Question 39

what is the appearance of BAL associated with pulmonary alveolar proteinosis ?

Answer 39

BAL of PAP appears as a milky fluid with debris and foamy macrophages

Question 40

what are the results of flow cytometric analysis of lymphocytes in sarcoidosis vs Hypersensitivity pneumonitis ?

Answer 40

hypersensitivity pneumonitis : CD4/CD8 < 1 sarcoidosis : CD4/CD8 > 2.5

Question 41

an increase in neutrophil count shows a worse prognosis in which diseases ?

Answer 41

sarcoidosis hypersensitivity pneumonitis idiopathic pulmonary fibrosis

Question 42

what method of obtaining a tissue sample is preferred when it comes to ILD ?

Answer 42

thoracoscopy guided and open lung biopsy

Question 43

when can we perform plasmapheresis ?

Answer 43

diffuse alveolar hemorrhage

Question 44

what is the treatment for PLCH ?

Answer 44

cladribine

Question 45

what is the treatment for PAP ?

Answer 45

whole lung lavage GM-CSF therapy

Question 46

definitive management for any end stage lung ?

Answer 46

lung transplant

Question 47

what type of granulomatous disease is sarcoidosis ?

Answer 47

non caseating granuloma

Question 48

what is the most common presentation associated with sarcoidosis ?

Answer 48

hilar and left paratracheal adenopathy positive despine sign

Question 49

what are the markers positive in pulmonary langerhans cell histocytosis

Answer 49

CD1a and S100

Question 50

what is the management in LAM ?

Answer 50

avoid pregnancy and give sirolimus