Intrinsic Restrictive Lung Disease - Pulmonary fibrosis, Sarcoidosis, Asbestosis, Pneumonia

Question 1

IPF
-presentation

Answer 1

45+
Persistent SOB on exertion, dry cough
Bilateral inspiratory crackles
Clubbing
Restrictive spirometry

Question 2

IPF
-causes

Answer 2

Idiopathic

Drug induced
- chemo
- methotrexate
- amiodarone
- nitrofurantoin

Environmental
- asbestos, orgnaic dust
- hay, straw, grain
- bird poo

AI
-RA, SLE, Sjogrens, scleroderma, dermatomyositis, polymyositis

Question 3

IPF
-diagnosis, investigations

Answer 3

Clinical diagnosis with the support of lung function tests and imaging
LFTs - spirometry, gas transfer => restrictive intrinsic lung problem
Scans
-CXR => interstitial lung markings
Key Ix - CT => reticular honeycombing, traction bronchiectasis

Procedures done if still unsure - broncheolar lavage, lung biopsy

Question 4

IPF
-management (conservative, medical, surgical

Answer 4

MAINLY SUPPORTIVE
- Pulmonary rehabilitation
- Oxygen therapy
- Smoking cessation

Nintedanib
Pirfenidone

Lung transplantation/palliative care

Question 5

Upper lung fibrosis causes
Lower lung fibrosis causes

Answer 5

Upper - CHARTS
- Coal worker
- Histiocytosis/hypersensitivity pneumonitis
- AS
- Radiation
- TB
- Silicosis (rocks, soil)/sarcoidosis

Lower -ACID
-Asbestosis
-Connective tissue AI minus AS
-IPF
-Drugs - amiodarone, bleomycin, methotrexate

Question 6

Sarcoidosis
-epidemiology
-etiology
-pathophysiology

Answer 6

Women
Young adults

Mix of genetic and environmental

AI => non caseating granulomas on lungs and skin
Can be acute or chronic

Question 7

Sarcoidosis
-presentation

Answer 7

Loefgren syndrome - acute, mild, self limiting
Bilateral hilar lymphadenopathy
Erythema nodosum
Arthritis

SOB, persistent dry cough
Tender swollen LN

Question 8

Sarcoidosis
-diagnosis, investigations

Answer 8

Clinical diagnosis of exclusion

• Restrictive spirometry
• High Ca, ACE, CRP
• CXR - bilar hilar lymphadenopathy
• Lung biopsy - non caseating granulomas

Question 9

Sarcoidosis
-management

Answer 9

Acute episodes can be self limiting
NSAIDs/CS depending on severity
-may also use methotrexate, azathiopurine, HCQ = LFTs needed to assess impacts

If end stage lung disease - lung transplants considered

Question 10

Asbestos exposure
-presentation of 4 lung problems
-management

Answer 10

Pleural plaques - benign (20-40 years latent)

Asbestosis - severity linked to length of exposure

• lower lung fibrosis => SOB, low exerecise tolerance
• conservatively managed

Mesothelioma - limited exposure can cause disease

• SOB, chest pain, pleural effusions
• v aggressive => palliative chemo

Lung cancer

Question 11

Pneumonia
-epidemiology, spread

Answer 11

Young children/elderly
IC

Droplet inhalation
Haematogenous

Question 12

Pneumonia
-presentation, symptoms

Answer 12

Dyspnoea
Purulent/clear sputum
Cough
Fever

High RR,
Hypotension, pyrexia (systemic inflammation)
Crackles, increased VR (exudate and consolidation)
Central cyanosis/confusion (hypoxemia)

Question 13

Pneumonia
-diagnosis, investigations

Answer 13

DEFINITIVE - CXR
IDENTIFY ORGANISM
-Blood, sputum culture
-Viral PCR
-Atypical serology
-Urine AG (legionella, pneumococcal)

ABG - PO2
FBC
U&E, LFT, CRP

Question 14

CURB65
-how to use
-interpretation

Answer 14

Confusion AMTS<8
Urea >7
RR >30
BP systolic<90 diastolic<60
65

0-1 home
2 admission considered
3-5 urgent admission, maybe ITU

Question 15

Pneumonia
-management based on CURB65

-HAP

Answer 15

Empirical broad spec ABx in 4hrs

0-amox
1-2-amox+clarithromycin
3-5-coamox+clarythromycin

Coamox

Question 16

Most common causative organism for pneumonia
-presentation
-management

Answer 16

S pneumonia - amox
- acute, high fever
- pleuritic pain, cold sores

Question 17

2nd most common causative organism for pneumonia
-management

Causative organism of pneumonia post influenza
-management

Causative organism of pneumonia in alcoholics
-presentation

Answer 17

Viral - management depends on virus

S aureus - fluclox

Klebsiella pneumonia - red currant sputum

Question 18

Causes of atypical pneumonias

Answer 18

Slow onset, flulike
Dry cough, fatigue, substernal chest pain
Often no physical exam findings => CXR looks worse than patient
Extrapulmonary features

Mycoplasma pneumonia - culture, serology
- younger people
- neuro and systemic symptoms (rashes)

Chlamydophila pneumonia - culture and serology
-Mild symptoms

Legionella - urine AG
-association with aircons
-Low Na, lymphocytes, LFTs affected
-Severe pneumonia with a high mortality

CAN ALL BE MANAGED WITH ERYTHROMYCIN

Question 19

Common causes of aspiration pneumonia
- location
- bacteria involved

Answer 19

Foreign materials entering bronchial tree
- poor dentition
- dysphagia
- prolonged hospitalisation, surgery
- unconscious

S pneumonia, aureus, H influenza, P aeruginosa, sterile (pneumonitis)

Question 20

Fungal causes of pnuemonia

• epidemiology
• presentation
• causative organism, management

Answer 20

HIV, IC
- SOB
- dry cough
- fever
- v few chest signs

Aspergillus - amphotericin
PCP (HIV) - cotrimoxazole + CS

Question 21

Complications of pneumonia

Answer 21

Sepsis
ARDS
Parapneumonic effusion, empyema
Cavitations
MI