1. Interstitial lung disease
Obeidah masoudi. MD,FCCP.
Pulmonary , critical care & sleep medicine
American board –Brown university

2. 52 yr/ F patient otherwise healthy, presents with complains of Dry cough 6 months Increasing DOE , cardiac work up clear. Exam: RR 26 Sat 92% HR 114 Bibasilar Inspiratory Crackles Clubbing

3. ATS –ERS classification of IIPs (idiopathic interstitial pneumonia)
This is a clinical , radiological , pathological classification.

4. Hallmark of ILD DOE , cough , chest pain. History : Examination :-
Tachypnea .
Cyanosis , clubbing.
Bibasilar Inspiratory crackles.
Pul. HTN and cor pulmonale.
IMAGING : - Interstitial pattern.
PFT:- Restrictive pattern.
DLCO :- Reduced.

6. UIP or IPF Prevelance 0.8- 64/100000 Incidence 0.4 – 27/100000
Increase with age
Median survival approximately 3 years, depending on stage at presentation.
B/L Reticular bibasilar and subpleural opacities. minimal ground-glass and variable honeycomb change.

7. Characteristic HRCT pattern of UIP
Subpleural Basal Honeycombing
Traction bronchiectasis
“Propeller blade” cranio-caudal distribution
Absence of atypical features:
lobules of decreased attenuation in spared lung consolidation
GGO
nodules

8. Honeycombing
HRCT showing subpleural broncheolectasis

9. UIP/IPF HISTOLOGIC PATTERN
CHARACTERISTICS
Temporal inhomogeneity
Dense fibrosis
Fibroblastic foci
Patchy lung involvement
Subpleural and basal

10. Radiological Features of IPF
UIP pattern (all four features)
Possible UIP pattern (all three features)
Inconsistent with UIP pattern (any one of seven features)
Subpleural basal predominance
Upper or mid lung predominance
Peribronchovascular predominance
Reticular abnormality
Extensive ground glass abnormality (extent > reticular abnormality)
Profuse micronodules (bilateral, predominantly upper lobes)
Honeycombing with or without traction bronchiectasis
Discrete cysts (multiple bilateral, away from areas of honeycombing)
Diffuse mosaic attenuation/air trapping (bilateral in three or more lobes)
Absence of features listed as inconsistent with UIP pattern
Absence of features listed as inconsistent with UIP pattern
Consolidation in broncho- pulmonary segment(s)/lobe(s)
ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;183:

13. Usual interstitial pneumonia
Usual interstitial pneumonia. HRCT abnormalities predominate in the posterior, subpleural regions of the lower lobes and comprise honeycombing and traction bronchiectasis within the abnormal lung.

14. UIP PATTERN
Coarse reticular opacities, subpleural honeycombing and traction bronchiectasis

15. IPF Treatment Guideline
“The majority of patients would want the intervention, but a significant minority would not.”
Strong YES Weak YES
“The majority of patients would not want the intervention, but a significant minority would.”
Weak NO Strong NO
Lung transplant Oxygen
Antacids
Nintedanib Pirfenidone
Sildenafil NAC
IPAH drugs
(for IPF-PH)
Warfarin Ambrisentan NAC+Aza+Pred Imatinib Prednisone
Raghu G et al. Am J Respir Crit Care Med 2015

16. Treatment A)Nintedanib; TKI(VEGFR, FGFR,PDGFR) 1) FVC (125ml)
2)exacerbation

17. B) pirfenidone : antifibrotic agent , down regulate production of growth factor & procollagen I & II.
1)FVC
2)exercise tolerance
3)progression – free survival
4)fewer deaths.

18. Efficacy of nintedanib and pirfenidone over 1 year
INPULSIS
n=423
ASCEND
n=638
n=278
n=277
-50
-50
FVC change (ml/year)
-100
-100
-150
-150
49.2%
-200
-200
41.5%
-250
-250
-300
-300
Nintedanib
Placebo
Pirfenidone
Placebo
Richeldi L et al. NEJM King TE Jr et al. NEJM 2014

19. Pirfenidone and Mortality
Data from Capacity and ASCEND
Nathan SD et al. Lancet Respir Med 2017

20. Time to first acute exacerbation (investigator-reported)
pooled data from TOMORROW and INPULSIS
Nintedanib 150 mg bid Placebo
HR 0.53
(95% CI: 0.34, 0.83)
p=0.0047
Richeldi L et al. Respir Med 2016

21. Treatable or not treatable?

22. Thank you.