3. Introduction
• Idiopathic pulmonary fibrosis (IPF) is a chronic
progressive fibrotic disorder of the lower respiratory
tract
⎼ Primarliy occurring over age 50-60
⎼ Limited to lung
⎼ Associated with histopathologic and/or radiologic pattern
usual of interstitial pneumonia (UIP)
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4. Introduction
IPF is an interstitial lung disease (ILD)
ILDs represent a large number of conditions that
involve the parenchyma of the lung, the alveoli,
the alveolar epithelium ,and the space between
structures, as well as the perivascular and
lymphatic tissue.
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5. CLASSIFICATION
ILD can be broadly classified on major histopathological
finding into
1) Those associated with predominant inflammation and fibrosis
2) Those associated with a predominantly granulomatous reaction
Each can be subdivided into
1) Known cause
2) Unknown cause
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7. IPF
most common ILD of unknown cause
Prevalence increases with age and is estimated at 50–
200:100,000.
IPF is commonly diagnosed in the fifth or sixth decade in
life
affects men more than women
frequently associated with a history of smoking or other
environmental exposures.
IPF is a variably progressive disease that carries a poor
prognosis with an estimated 50% 3- to 5-year survival
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8. Etiology
idiopathic
Certain risk factors have been identified
• Cigarette smoking
• Environmental pollutants
• Viral infections
• GERD
• Genetic predisposition
• Drugs
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9. Pathophysiology
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Microinjuries to alveolar epithelial
cell
Release TGF-Beta, TNF ⍶, IL1,MCP-1
Fibroblast Activation
Myfibroblast
collagen
Loss of capillary area
Fibrosis
Poor gas exchange
hypoxemia
10. CLINICAL FEATURES
Gradual onset of dyspnea
Exertional dyspnea
Non-productive cough
Weight loss
Low grade fever
Fatigue
Arthralgiase
Myalgias
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Symptms
11. Sign
Bibasilar inspiratory crackles
Digital clubbing (25-50%)
Cyanosis
Features of pulm HTN
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18. Con…
PFT
• Reduced VC, functional residual capacity And FVC
• Reduced DLCO
• Good for prognostication
6-Minute walk testing
Bronchoalveolar lavage BAL
Transthoracic Echocardiography
ANA, RF
CRP ,ESR may be elevated(60-94%)
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19. Con….
LUNG BIOBSY
• Abnormal proliferation of mesenchymal cell
• Varing degrees of fibrosis
• Overproduction and disorganized deposition of collagen
and extracellular matrix
• Distoration of pulmonary architecture and subplueral
cystic airspace called honeycomb cyst
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20. DIAGNOSTIC CONSIDERATION
Idiopathic disease by history and inspiratory crackles on
physical examination
Restrictive physiology on PFT
Charcteristic radiographic evidance of progressive
fibrosis over several years
Diffuse patchy fibrosis with plueral based honycombing
on HRCT
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21. DDX
Other idiopathic interstitial pneumonia
Systemic sclerosis/ Scleroderma
Rhumatic disease
Drug and irradiation-induced UIP
Abestosis
Chronic hypersensitivity pneumonitis
pulmonary langerhans cell histocytosis
Combined pulmonary fibrosis and emphysema
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22. Treatment
Can be devided into
• Non pharmacoloic
• Pharmacologic
• surgical
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24. treatmetnt
Pharmacologic
No medication has been found to cure IPF but two
antifibrotic medications apear to slow disease progression
and reduce the frequency of exacerbation
Nintedanib
• Blocker for multiple tyrosine kinases
• Dose 150mg twice daily
• LFT should be assessed
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25. Treatment
Pirfenidone
• TGF-b inhibitor
• Dose, 40 mg/kg, 2403mg/day/3 divided dose
• Or
• Days 1-7: 267 mg PO TID (801 mg/day)
• Days 8-14: 534 mg PO TID (1602 mg/day)
• Days 15 and thereafter (maintainance): 801 mg PO TID;
not to exceed 2403 mg /day
• Monitor LFTs
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26. Treatment
SURGICAL TREATMENT (LUNG TRANSPLANTATION)
DLCO<40%
Decline in FVC >10% during six month of follow up
Decline in DLCO >15% during 6-MWT
O2 Desaturation to <88% on 6-MWT
PHTN
Hospitalization because of respiratory decline,pneumothorax,or acute
exacerbation
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28. Prognosis
Prognosis is poor
5yr survival rate 20-40%
Poor prognostic factors
>10 decline in FVC over 6 month
DLCO < 35%
A decline in DLCO >15% over 1year
Desaturation below the threshold of 88% during 6-MWT
BAL fluid neutrophilia
Male sex
Age >65
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