1. Respiratory MCNs - Interstitial lung diseases
Dr Nik Hirani
Senior Lecturer and Honorary Consultant, Royal Infirmary Edinburgh

2. Interstitial Lung Diseases
Interstitial pneumonias
Hypersensitivity
Pneumonitis
Sarcoidosis
Eosinophilic syndromes
Known causes
Connective tissue diseases
Idiopathic
Known causes
Birds, Farmers lung, drugs etc
Idiopathic
Known causes
Drugs, parasites, vasculitis, ABPA
Idiopathic eosinophilic pneumonia
DIP
RB-ILD
AIP OP
NSIP
LIP
UIP
= IPF
= COP
Other rare disease
Langerhans cell histiocytosis
Lymphangioleiomyomatosis
Alveolar proteinosis
= Hamman-
Rich syndrome

3. Interstitial Lung Diseases
GET ME OUT OF HERE!
Interstitial pneumonias
Hypersensitivity
Pneumonitis
Sarcoidosis
Eosinophilic syndromes
Known causes
Connective tissue diseases
Idiopathic
Known causes
Birds, Farmers lung, drugs etc
Idiopathic
Known causes
Drugs, parasites, vasculitis, ABPA
Idiopathic eosinophilic pneumonia
DIP
RB-ILD
AIP OP
NSIP
LIP
UIP
= IPF
= COP
Other rare disease
Langerhans cell histiocytosis
Lymphangioleiomyomatosis
Alveolar proteinosis
= Hamman-
Rich syndrome

4. 1000 patients with ILD in the Edinburgh clinic 2004-2009
IPF
Sarcoidosis
Rheumatological
NSIP HP
COP
Miscellaneous

5. Idiopathic pulmonary fibrosis
new cases per year in UK
Median age at presentation 68 yr, rare under 40 yrs
M:F 2:1
60% smokers
5000 deaths per year
Gribben et al Thorax 2006

6. Clinical Presentation of IPF
Breathlessness over few weeks/months
Bibasal fine ‘velcro’ crackles
Diffuse CXR abnormality, often basal, initially subtle
Restrictive lung defect
(finger clubbing)
Diffuse lines and nodules on CXR
= FIBROSING ALEOLITIS SYNDROME

7. HRCT is cornerstone of investigation
….but only 60% of HRCT’s are ‘diagnostic’ in the right clinical context

8. Lung biopsy enhances diagnostic process
….but only 30% of patients with ‘non-diagnostic’ clinical and radiological features have a biopsy

9. Variability in the clinical course of IPF
Diagnosis = prognosis
LUNG FUNCTION / SYMPTOMS
TIME
Sarcoidosis
NSIP, CTD-ILD
IPF
From Flaherty et al, Thorax, 2003

10. How to predict poor prognosis in IPF
The more ‘classic’ the clinical features, the worse the prognosis
Smoking males
Age?
Hospital admission with ‘exacerbation’
Serial lung function – 10% change in FVC or 20% change in TLCO

11. Treatment of IPF Best supportive care Transplant for the minority
Recruit to clinical trials

12. Standards of care in ILD

13. 1. Diagnosis of ILD Requires a multidisciplinary approach
Consensus between chest physician, radiologist and pathologist (+rheumatology, occupational health)

14. 2. Treatment including supportive care
Tailored therapy requires diagnostic precision!
Smoking cessation
Pulmonary rehabilitation
Treat co-existing chest disease
Anti-reflux therapy
Oxygen therapy
Withdrawal of potentially toxic futile therapy
Early referral for lung transplant
Recruitment to clinical trials

15. 3. Palliation Equality of access to palliative care services
Condition-specific palliative care protocols

16. 4. Organisation of services
Modified from current care models for pulmonary hypertension, CF and lung cancer
‘Regional centres’ comprising of teams that can address diagnostic and treatment standards.
Each centre must:
1)establish a consistent care pathway for ILD patients
2) hold a regular regional MDM
3) enable recruitment to multi-centre clinical trials
4) establish and audit programme of outcome measures of ILD patients
5) deliver approved new therapies to selected patients.

17. Summary ILDs are an important burden of disease
IPF is a lethal disease requiring expert care
Diagnosis and management requires a multidisciplinary approach
MCNs in ILD are in an early stage of development but will be pivotal in delivering care