A seminar about IPF can be presented breifly in 10 minutes.. this would give you a general idea about the disease and how to approach it.. THERE ARE SOME NOTES TO HELP GRASPING AND EXPLAINING AND EXPANDING THE TOPIC

1. Pulmonary Fibrosis
Naeel Shamia
Group – A7

2. Outlines
 Definition of IPF
 A zoom out
 Epidemiology
 Risk factors
 Patient assessment
 Diagnosing IPF
 Treatment
 Complications
 References

3. Idiopathic Pulmonary Fibrosis
 Idiopathic pulmonary fibrosis (IPF) is defined as a
specific form of chronic, progressive fibrosing interstitial
pneumonia of unknown cause. (Medscape)
 This includes varying degrees of fibrosis, bronchiectasis,
cyst formation with honeycombing, and mild interstitial
inflammation interspersed with normal lung tissue.
 IT IS A DEVASTATING AND UNRELENTING DISEASE.
Although the prognosis is variable, the mean survival is only 3 to
7 years after first diagnosis. (Step-up to medicine)

4. Interstitial lung diseases (IDL):
are a heterogeneous group of disorders that affect “lungs interstitium”
and are classified together because of similar clinical, radiographic,
physiologic, or pathologic manifestations.
Idiopathic Interstitial Pneumonias (IIP):
A subset of diffuse interstitial lung diseases of unknown etiology
characterized by expansion of the interstitial compartment with an
infiltrate of inflammatory cells.
Idiopathic Pulmonary Fibrosis (IPF)

5. {Interstitial lung diseases (IDL)}
DPLD: diffuse parenchymal
lung disease;
IIP: idiopathic interstitial
pneumonia;
LAM:
lymphangioleiomyomatosis;
PLCH: pulmonary Langerhans
cell histiocytosis/histiocytosis
X.

6. Epidemiology
 Worldwide, the incidence of idiopathic pulmonary
fibrosis is estimated to be 10.7 cases per 100,000
person-years for males and 7.4 cases per
100,000 person years for females. (Medscape)
 The average age of onset is 55 years but it occurs in all age
groups.

7. Possible inciting factors
(Risk Factors)
 Males gender.
 Older age (approximately two thirds of patients are >60 years
at diagnosis -MedStudy)
 Cigarette Smoking.
 Genetics (As it may follow an autosomal dominant pattern according
to some studies – UpToDate). Also is being contributed to some genetic
mutations.
 Occupational dust or fume exposure.

8. Symptoms:
 Dyspnea (most prominent symptom)
 Nonproductive cough
 Rapid Weight Loss
 Fatigue and Weakness
 Occasional Chest Pain
 Chest Discomfort
 Dyspnea on Exertion
Patient Assessment

9. Patient Assessment
 Digital Clubbing is common, seen in up to 60% of patients,
and more frequently in men.
 Cyanosis around the mouth or fingernails (in
advanced stages)
 Findings of cor pulmonale (eg, peripheral edema,
right ventricular heave, accentuated second heart
sound). these findings are usually indicative of
advanced disease.
 Abnormal breath sounds on auscultation
Velcro-like crackles (especially in the lower lung fields and posterior
axillary line)

10. Finger clubbing
Photo taken by- Yazeed Kashkosh
Photo taken by- Yazeed Kashkosh

11. Diagnosing Pulmonary Fibrosis
The diagnostic workup of IPF includes:
 Chest X-ray or High Resolution CT
 Pulmonary Function Tests
 BronchoAlveolar Lavage (BAL)
 Lung biopsy.

12. Chest X-ray and
HRCT show
characteristic
features of
subpleural fibrosis,
Bronchiectasis,
subpleural cysts
and ground-glass
or
honeycombed
appearance.

13. BronchoAlveolar Lavage
(BAL)
BAL results show increased macrophages,
neutrophils (PMNS = 20%), and
eosinophils (2-4%).
Because the fluid in the alveoli and the interstitial edema
has increased numbers of alveolar macrophages, And they
release cytokines and proinflammatory mediators (tumor
necrosis factor [TNF], interleukin 8, and leukotriene B4)-
some of which attract neutrophils.

14. Lung biopsy
To characterize the pathology of chest x-ray
abnormalities and to exclude cancer, infections and
vasculitis.

15. Treatment
• No known cure exists for Pulmonary Fibrosis
 OXYGEN.
 Nintedanib (a tyrosine-kinase inhibitor) and Pirfenidone (an
antifibrotic agent) to slow the progression of disease.
 PPIs to prevent acid reflux.
 High-dose Glucocorticoids With acute exacerbation
(sudden acceleration of the disease with a significant
decline in lung function).
 Lung Transplant in advanced stages.

16. Possible Complications
 Chronic Hypoxemia
 Pulmonary Hypertension
 Cor Pulmonale
 Respiratory Failure
 Polycythemia (rare)

17. References
MedStudy – Internal Medicine Core
Step-up to medicine
www.Medscape.com
www.uptodate.com
www.Pulmonaryfibrosis.org

18. Thank you..