Chronic ILDs (interstitial lung disease)

Question 1

what are some ILD examples?

Answer 1

Granulomatous
Inhalational
Idiopathic pneumonias
Connective tissue
Drug induced
Other

Question 2

ILD:
Granulomatous =
Inhalational =
Idiopathic pneumonias =
Connective tissue =
Drug induced =
Other =

Answer 2

Granulomatous = Sarcoidosis

Inhalational = Hypersensitivity pneumonitis, pneumoconiosis (asbestos/silicosis)

Idiopathic pneumonias = Pulmonary fibrosis (MC ILD) + Non pul fibrosis

Connective tissue = scleroderma, RA

Drug induced = Biomycin, amiodarone

Other = good pastures, vasculitis

Question 3

Idiopathic Pulmonary Fibrosis
is it MC?
Seen in which Px?
Cause?

Answer 3

Commonest ILD
Seen in older men 60+, who SMOKE
idiopathic (unknown cause)

Question 4

Idiopathic Pulmonary Fibrosis
RF?

Answer 4

Smoking
occupational (eg. dust)
Drugs (methotrexate)
Viruses (EBV, CMV)

Question 5

Idiopathic Pulmonary Fibrosis
pathology?

Answer 5

Pul fibrosis (progressive scarring) –> type 1 resp failure

Question 6

Idiopathic Pulmonary Fibrosis
Sx?

Answer 6

exertion dyspnoea
dry unproductive cough

Question 7

Idiopathic Pulmonary Fibrosis
Dx?

Answer 7

Spirometry = Restriction; FEV1:FVC >0.7 BUT LOW FVC (<0.8 normal)

GS = High res CT (chest) = GROUND GLASS LUNGS + traction bronchiectasis

Question 8

Tx for Idiopathic Pulmonary Fibrosis

Answer 8

Smoking cessation + vaccines
Pirfenidone, nintendinir
Surgery (lung transplant)

Question 9

Pneumoconiosis
How is it acquired?
2 types?

Answer 9

Occupationally acquired ILD
Silicosis
Asbestosis

Question 10

Pneumoconiosis

How is silicosis caused?

Answer 10

Inhalation of silicon dioxide, egg shall calcification at hilar lymph nodes

Question 11

Pneumoconiosis

How is asbestosis caused?

Answer 11

Inhalation of asbestos, affects pleura, MESOTHELIOMA = T1 RESP FAILURE

Question 12

Sarcoidosis
what is it?
most common in what type of population?

Answer 12

Idiopathic granulomatous disease
women, 20-40, afrocarribean

Question 13

Sarcoidosis
Sx?

Answer 13

Fever, fatigue
Resp = dry cough, dyspnoea
Other = eye lesions (uveitis), lupus pemio (blue-red nodules on nose/cheeks)

Question 14

Sarcoidosis
Dx?

Answer 14

Chest x ray (staging 1-4)
shows BILATERAL HILAR ADENOPATHY + pulmonary infiltrates

Diagnostic = Biopsy showing non caveating granuloma

Also high serum Ca2+ and ACE (seen in granulomas)

Question 15

Sarcoidosis
Tx?

Answer 15

Early stages = self resolving but Symptomatic Px = corticosteroids
(Sx 2+3, any stage 4)

Question 16

What is Lofgren’s syndrome?
Triad of?

Answer 16

Subset of sarcoidosis
Triad of:
Bilateral hilar lymph node infiltration
Erythema Nuclosum
Acute polyarthritis (MC = ankles)

Question 17

Hypersensitivity Pneumonitis
What is it?

Answer 17

T3 hypersensitivity; immune (Ab-Ag) complex deposition at lung tissues + causes immune hyper response

Question 18

Hypersensitivity Pneumonitis
RF?
Types?
Tx?

Answer 18

Occupation (eg. farming), bird keeping

Farmers lung (MC) - mouldy hay
Pigeon fancier’s lung (a non protein bird dropping)
Malt workers lung
Cheeseworkers lung
Humidifier fever

Tx = remove allergy

Question 19

Goodpastures
What is it?
Pathology?

Answer 19

T2 autoimmune hypersensitivity response

AutoAb, Anti GBM attack LUNGS + KIDNEYS causing lung fibrosis and glomerulonephritis

Question 20

Goodpastures
Dx?
Tx?

Answer 20

Lung + kidney biopsy = damage and Ig deposition
Serology = anti GBM +ve

Tx = Supportive, corticosteroids, plasma exchange to get rid of Anti-GBM

Question 21

Wegener’s granulomatosis
What is it?
What is it associated with?

Answer 21

Granulomatosis with polyangitis
Granulomatous vasculitis affecting small + medium vessels
Typically causes ENT, lung, kidney Sx (ELK)

C-ANCA associated vasculitis

Question 22

Wegener’s granulomatosis
Sx? ELK

Answer 22

ENT = Saddle shaped nose, ear infection

Lungs = diffuse alveolar haemorrhage therefore haemophysis

Kidney = GlomerulonephrItis therefore haematuria

Question 23

Wegener’s granulomatosis
Dx?
Tx?

Answer 23

cANCA +VE
(MPA + EGPA = mostly pANCA +ve)

Tx = corticosteroids, immunosuppression (eg. nituximab)