Interstitial Lung Disease

Question 1

interstitial lung disease

Answer 1

Question 2

alphabet soup

Answer 2

• UIP = Usual interstitial pneumonia
• NSIP = Non-specific interstitial pneumonia
• LIP = Lymphocytic interstitial pneumonia
• DIP = Desquamative interstitial pneumonia
• COP = Cryptogenic organising pneumonia
• RBILB = Respiratory bronchiolitis - ILD
• AIP = Acute interstitial pneumonia

Question 3

ILD history

Answer 3

• Worsening breathlessness
• Cough
• Ankle swelling (Cor pulmonale)

Question 4

examination

Answer 4

Autoimmune screen
CXR
Full lung function tests
6 minute walk test
HRCT
Bronchoscopy
Surgical lung biopsy

Question 5

lung funcation test

Answer 5

• Baseline measurements
• Exercise tests (6MWT)
• Progress

Question 6

patterns of physiology

Answer 6

“classical” physiological response:
- restrictive pattern +/-abnormal gas exchange
Some obstructive “bronchiolocentric” diseases:
- sarcoidosis
- hypersensitivity pneumonitis
- histiocytosis X
- LAM/tuberous sclerosis
- neurofibromatosis

Question 7

when to use high resonance CT

Answer 7

• UIP pattern
• Sarcoidosis *
• LAM
• Histiocytosis X
• Hypersensitivity pneumonitis *
• Lymphangitis
• Alveolar proteinosis

Question 8

supportive treatmenr

Answer 8

• Oxygen
– Long term oxygen therapy
– As needed therapy
– Ambulatory oxygen therapy
• Warfarin
• Symptom control

Question 9

steoids and azothioprine

Answer 9

Benefits
• Disease dependant
• Occasional reversal
• Sometimes slows down
• Usually ineffective in IPF
(N-Acetyl Cysteine)

• Risks
• Infections
• Liver failure
• Bone marrow failure
• Osteoporosis
• Muscle wasting
• High blood pressure
• Diabetes
(Regular monitoring)

Question 10

transplantation

Answer 10

• Benefits
• <60 years old
• <2 years to live
• Progressive disease

• Risks
• Not an easy ride
– Waiting lists
• Some patients die
• Lifelong treatment with
immunosuppressives

Question 11

hypersensitivity pneumonitis

Answer 11

aka extrinsic allergic alveolitis

• Organic antigens or low
molecular weight
compounds
• Repeated inhalation
• Lymphocyte mediated
response

• Birds
– Avian droppings, feathers,
serum

• Mouldy Hay
– Faeni rectivirgula

• Hot tubs
– Cladosporium spp

Question 12

HSP treatment

Answer 12

• Antigen avoidance
• Corticosteroids
• Immunosuppressive therapy

Question 13

how to diagnose sarcoides?

Answer 13

1. CLINICALLY
2. EYES
3. RADIOLOGY
4. HAEMATOLOGY & BIOCHEMISTRY
5. MANTOUX
6. HISTOLOGY

Question 14

CXR sarcoides

Answer 14

• Stage 0
– Normal

• Stage 1
– Bilateral Hilar LNs

• Stage 2
– BHL + infiltrates

• Stage 3
– Infiltrates – BHL

• Stage 4
– Fibrosis

Question 15

sarcoides

Answer 15

compact non caseating granulomas

Question 16

histology

Answer 16

Question 17

indicators of disease activity

Answer 17

1. CLINICAL FEATURES
2. LUNG FUNCTION TESTS
3. RADIOLOGY
4. Ca2+ & LFTs
5. Serum ACE

Question 18

do steroids help

Answer 18

Question 19

who needs treatmen

Answer 19

• ATS GUIDELINES 1971:
• -Stage II & III + Symptoms
• -Pulmonary Fibrosis
• -Hypercalcaemia/Hypercalciuria
• -Uveitis
• -CNS/Cardiac
• -Skin/Muscle/Nodes/Spleen

Question 20

treatment

Answer 20

1. CHLOROQUINE
2. METHOTREXATE