ILD

Question 1

When type of granuloma do you get in the lung in ILD?

Answer 1

LOOSE

caseating= TB
non caseating=sarcoid/ beryliosis/ silicosis

Question 2

how can you differentiate HSP with RBILD?

Answer 2

RBILD will be localised to the upper lobes.

Question 3

What are the HRCT features of HSP?

Answer 3

Non-fibrotic- centrilobular nodules in all lobes, ground glass, mosaic attenuation, air trapping.

Fibrotic- honeycombing, traction bronchiectasis, reticulation, 3 density pattern (GG+airtrapping+nodule in same lobe), SPARES base of lungs

Question 4

What are the poor prognostic factors in HSP?

Answer 4

cigarette smoking, lower baseline VC, lack of BAL lymphocytosis, persistent exposure to the inciting agent, and/or inability to identify an inciting agent

Question 5

What is the diagnostic criteria for HSP?

Answer 5

1) exposure identification, serum IgG testing against potential antigens associated with HP, and/or specific inhalation challenge
2) imaging pattern
3) BAL lymphocytosis/ histopathological findings

Question 6

What is the cell count in a normal BAL?

Answer 6

Alveolar macrophages >85%
Lo (CD4/CD8 0.9–2.5) 10–15% (higher in smokers) Neutrophils <3%
Eo <1%
Squamous epithelial <5%

Question 7

What is the BAL differential for >15% lymphocytes?

Answer 7

Sarcoidosis
NSIP
HSP
Drug-induced pneumonitis
Collagen vascular diseases
Radiation pneumonitis
COP
Lymphoproliferative disorders

> 25% granulomatous ILDs- sarcoid/HSP/beryllium

> 50% HSP

Question 8

What is the BAL differential for >1% eosinophils?

Answer 8

Eosinophilic pneumonia
Drug-induced pneumonitis
Bone marrow transplant
Asthma
bronchitis
Churg-Strauss
ABPA
Bacterial, fungal, helminthic, Pneumocystis infection
Hodgkin’s disease

> 25% eosinophilic pneumonia

Question 9

What is the BAL differential for >3% neutrophils?

Answer 9

Collagen vascular diseases
IPF
Aspiration pneumonia Infection: bacterial, fungal Bronchitis
Asbestosis
ARDS
Diffuse alveolar damage

> 50% Acute lung injury, aspiration or suppartive infections

Question 10

what differentiates Langerhans cell histiocytosis on BAL?

Answer 10

> 5% CD1a-positive cells

Question 11

what differentiates Pulmonary alveolar proteinosis on BAL?

Answer 11

PAS-positive amorphous debris

Question 12

What genes are associated with familial interstitial pneumonias (namely IPF)?

Answer 12

SFTPC (z1%), SFTPA2 (z1%), TERT (z15%), and TERC (z1%) are responsible for about 20% of all familial interstitial pneumonias

MUCB gene assoicated with IPF

Question 13

What can fibrosing COP be associated with?

Answer 13

underlying polymyositis or antisynthetase syndrome

Question 14

Which biomarkers are associated with rapidly declining LFTs?

Answer 14

SP-A, SP-D, KL-6 (Krebs von den Lungen-6), CCL18 (chemokine ligand-18), and MMP-7
These are all in IPF

Question 15

What are the associated RF for IPF?

Answer 15

Male, smokers, GORD, chronic viral infections such as EBV/Hep C, FHx of ILD

Question 16

When doing an HRCT when are the images taken?

Answer 16

end inspiration and end expiration, +/- prone

Question 17

What are the HRCT findings of UIP?

Answer 17

traction bronchiectasis, subpleural reticulation/thickening, honeycombing- basal predominence, (GGO)

Question 18

Which blood tests should be done in ILD?

Answer 18

CRP, ESR, ANA, RF, anti-CCP, anti-Jo1 etc

then if suspected can do anti-Scl70, anti-toperiomase, anti-centromere, Anti-Ro/La, ANCA

Question 19

What are the histological findings on UIP?

Answer 19

Dense fibrosis with architectural distortion (i.e., destructive scarring and/or honeycombing)
subpleural/paraseptal distribution of fibrosis
Patchy involvement of lung parenchyma by fibrosis
Fibroblast foci

Question 20

What defines progressive lung fibrosis in LFTs of ILD patients?

Answer 20

Absolute decline in FVC of >5% within 1 year of follow-up.
Absolute decline in TLCO >10% within 1 year of follow-up.

INBUILD and RELIEF are the studies that show that antifibrotics help in non IPF PPF setting too

Question 21

criteria for antifibrotics?

Answer 21

ATS:
1. Absolute decline in FVC of >5% within 1 year of follow-up. Absolute decline in cDLCO >10% within 1 year of follow-up.
2. Radiological evidence of disease progression
3. Worsening respiratory symptoms

NICE:
FVC 50-80% predicted
and treatment stops if >10% decline in 1yr

Question 22

What other malignancies is Langerhans associated with?

Answer 22

myeloid neoplasms
lymphomas
solid organ malignancies (especially lung and thyroid ca)

Question 23

When should spiro be carried out according to NICE for IPF?

Answer 23

baseline, 6 months and 12months and earlier if three is deterioration

Question 24

What are the treatments for cough in IPF?

Answer 24

treat co-morbs/underlying cause, opoids, (thalidomide- off license)

Question 25

What are Birbeck granules seen in?

Answer 25

Langerhans

Question 26

what sort of pattern of fibrosis do you get with prolonged nitrofurantoin use?

Answer 26

tends to be NSIP

Question 27

What are the absolute contraindications to pirfenidone?

Answer 27

preg/breastfeeding
smoking (increases clearance!)
eGFR <30
severe hepatic impairment (Child Pugh C)

Question 28

With which antibiotic does pirfenidone need to reduced?

Answer 28

ciprofloxacin

CYP1A2 inhibitors interact

Question 29

which drugs are implicated in organising pneumonia?

Answer 29

rituximab, amiodarone, interferons, minocycline and statins