Restrictive Pulmonary Disease

Question 1

define sarcoidosis

Answer 1

a multi-system granulomatous chronic disorder that induces widespread inflammatory changes and complications

Question 2

causes of sarcoidosis

Answer 2

unknown trigger causes type IV hypersensitivity reaction that induces granuloma formation BUT NO NECROSIS

Question 3

risk factors of sarcoidosis

Answer 3

african and caribbean descent
female sex
inheritance of HLA-DRB1 and DQB1 alleles
age 20-40

Question 4

acute sarcoidosis features

Answer 4

fever
polyarthralgia 
erythema nodosum 
bilateral hilar lymphadenopathy
bilateral diffuse ankle swelling

Question 5

chronic sarcoidosis features

Answer 5

dry cough, dyspnoea, reduced exercise tolerance

fatigue, weight loss, arthralgia and low-grade fever

hepatomegaly and splenomegaly

erythema nodosum, lupus pernio

uveitis, conjunctivitis and optic neuritis

Question 6

investigations of sarcoidosis

Answer 6

tissue biopsy - non-caseating granulomas
CXR/chest CT
bloods

Question 7

common appearance of sarcoidosis staging on CXR (0-4)

Answer 7

stage 0 = no abnormalities

stage 1 = bilateral hilar lymphadenopathy (BHL)

stage 2 = BHL with peripheral infiltrates

stage 3 = peripheral infiltrates alone

stage 4 = pulmonary fibrosis

Question 8

common bloods and results in sarcoidosis

Answer 8

FBC, LFTs, U+E, ESR, serum Ca and ACE

FBC - anaemia or ⬆️ WCC
LFTs - deranged 
ESR - often ⬆️
Ca - hypercalcemia 
ACE - raised in acute

Question 9

management if stage 1 sarcoidosis

Answer 9

none, usually self-limiting

Question 10

management if stage 2-3 sarcoidosis

Answer 10

NSAIDs or topical steroids

oral glucocorticoids (prednisolone)
- 40mg 4-6wks and decline dose over 1 year

Question 11

management of severe sarcoidosis

Answer 11

immunosuppressants (e.g. TNF monoclonal antibodies or rituximab)

Question 12

define pulmonary fibrosis

Answer 12

a group of disease that result in interstitial lung damage that progresses towards fibrosis and causes loss of lung elasticity

Question 13

causes of pulmonary fibrosis

Answer 13

lung tissue damage
irritants 
diffuse parenchymal lung disease 
connective tissue disease
medications 
hypersensitivity

Question 14

define idiopathic pulmonary fibrosis

Answer 14

a progressive fibrotic lung disease of unknown cause that usually progresses slowly

Question 15

clinical features of idiopathic pulmonary fibrosis

Answer 15

dry cough
progressive dyspnoea
fatigue
arthralgia

Question 16

signs of IPF

Answer 16

cyanosis
clubbing
fine-end inspiratory crackles

Question 17

investigations of IPF

Answer 17

bloods - CRP, ANA and rheumatoid factor
CXR
CT chest
spirometry - restrictive deficit

Question 18

common appearances of IPF on CXR and CT

Answer 18

CXR:
- bilateral lower zone reticulo-nodular shadowing

CT:

• reticulonodular shadowing
• honeycomb lung

Question 19

conservative management of IPF

Answer 19

smoking cessation
pulmonary rehab/therapy
regular exercise
weight control 
annual flu vaccine

Question 20

medical management of IPF

Answer 20

long-term O2 therapy
anti-fibrotic drugs (e.g. pirfenidine)
PPI

Question 21

when is lung transplant considered in IPF

Answer 21

if medical therapy fails