Introduction Classification Epidemiology Pathogenesis Clinical features Investigation Treatment

1. DR MUHAMMED ASLAM
MBBS MD PULMONARY MEDICINE
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Interstitial Lung Disease(ILD)
or
Diffuse Parenchymal Lung Disease
(DPLD)

2. OUTLINE
• Introduction
• Classification
• Epidemiology
• Pathogenesis
• Clinical features
• Investigation
• Treatment

3. INTRODUCTION
• Interstitial Lung Disease refers to a broad range of
conditions that have common clinical, physiological, and
radiological features.
• ILD is not one disease but several diseases that do not
necessarily share a common histopathological or
pathophysiological basis

4. INTRODUCTION
• By strict definition Interstitial lung disease involves
abnormalities of the interstitium – “the potential space
between the epithelium and capillary endothelial basement
membrane within the alveolus”. HOWEVER…………..

5. INTRODUCTION
• Interstitial is a misleading terminology because most of
these disorders are associated with extensive alteration of
airway and alveolar architecture in addition to changes in
interstitial compartment.
• For this reason Diffuse Parenchymal Lung Disease or
DPLD is the better term.

6. CLASSIFICATION OF DPLD
• DPLD : Two large groups
(1)Idiopathic DPLD ( no known cause )
(2) Those with identifiable cause or occurs secondary to other
diseases

7. IDIOPATHIC ILD
1.Idiopathic pulmonary fibrosis (IPF) – most common
2.Acute interstitial pneumonia (AIP)
3.Cryptogenic Organizing Pneumonia ( COP) / Bronchiolitis obliterans
organizing pneumonia (BOOP)
4.Desquamative interstitial pneumonia (DIP)
5.Lymphocytic interstitial Pneumonia ( LIP)
6.Non specific interstitial Pneumonia (NSIP)
7.Respiratory bronchiolitis associated Interstitial lung disease (RBILD)

8. THOSE WITH IDENTIFIABLE CAUSE
1.Autoimmune- rheumatoid arthritis, S.L.E, Sarcoidosis,
Scleroderma
2.Certain infections
3.Medications
4.Radiation
5.Occupational exposure :asbestos, coal, cotton, silica

9. CLASSIFICATION

10. EPIDEMIOLOGY
• Incidence ranges from 3-26/1,00,000 per year.
• Prevalence of preclinical and undiagnosed ILD is estimated
to be 10 times that of clinical recognized disease.
• IPF is the most common form representing at least 30
percent of the incident cases.

11. IPF - PATHOGENESIS VIDEO

12. NORMAL DPLD

13. RESPIRATORY SYMPTOMS
Breathlessness (most common): Initially, dyspnea on
exertion→ later at rest
 Nonproductive cough
 Pleuritic chest pain
Wheeing
 Hemoptysis

14. NON RESPIRATORY SYMPTOMS
ASSOCIATED WITH DIFFERENT DPLDS
• Arthritis
• Ocular
• Skin and muscle
• GERD
• Lower GI symptoms
• Recurrent sinusitis
• Neurological symptoms
• Epilepsy & mental retardation
• Diabetes inspidus

15. ONSET OF SYMPTOMS
1. Acute presentation (days to weeks)- eg: Acute idiopathic
interstitial pneumoni,Hypersensitive pneumonitis
2. Sub-acute presentation (weeks to months)- eg:
Sarcoidosis,Drug induced ILD ,Alveolar hemorrhage
syndromes
3. Chronic presentation (months to years) – eg: IPF

16. ILD-HISTORY
•Smoking
•Medication history- Amiodorane, Methotrexate,
•Occupational history
•Environmental exposure history

17. OCCUPATIONAL HISTORY
􀂾 Pneumoconioses – miners
􀂾 Silicosis – sand blasters & granite workers
􀂾 Asbestosis – welders, electricians, mechanics, workers with
brakes, shipyard workers
􀂾 Berylliosis – aerospace, nuclear, computer & electronic industries
􀂾 Hypersensitive pneumonitis – farm workers, poultry workers,
bird breeders
􀂾 The degree of exposure, duration, latency of exposure, and the
use of protective devices should be elicited

18. ENVIRONMENTAL EXPOSURE HISTORY
• 􀂾 Exposures to pets (especially birds)
• 􀂾 Air conditioners
• 􀂾 Humidifiers
• 􀂾 Hot tubs
• 􀂾 Evaporative cooling systems

19. ILD-SIGNS
• Crackles - Dry, velcro , end inspiratory, predominantly
bibasilar
• Inspiratory squeaks- Mid inspiratory, high pitched- Seen in
Airway centred pathologies
• Cor pulmonale features
• Clubbing – IPF

20. EXTRA PULMONARY -SIGNS
• Skin abnormalities
• Lymphadenopathy
• Hepatosplenomegaly
• Maculopapular skin rashes
• Erythema nodosum
• Subcutaneous nodules
• Proximal muscle weakness
• Arthritis

21. INVESTIGATIONS

22. CHEST X RAY
• Typically small lung volumes with
Reticular , Nodular, or
RETICULONODULAR shadow

23. HIGH-RESOLUTION COMPUTED TOPOGRAPHY
(HRCT)
• HRCT is more sensitive
• Combinations of ground
glass changes,
reticulonodular shadowing,
honeycomb cysts and
traction bronchiectasis

24. PULMONARY FUNCTION TESTING
• Objective assessment of Resp Symptoms
• Grading the severity
• Monitoring the response to therapy

25. PULMONARY FUNCTION TESTING
• Restrictive Defect
• ↓ Lung volumes (TLC, FRC,RV <80%)
• ↓ FEV1, FVC With Normal or ↑FEV1/FVC
• Reduced diffusing capacity (DLCO)

26. LABORATORY INVESTIGATIONS
• Full blood count: lymphopenia in sarcoid; eosinophilia in pulmonary
eosinophilias and drug reactions; neutrophilia in hypersensitivity
pneumonitis
• Ca2+: may be elevated in sarcoid
• Serum angiotensin-converting enzyme: non-specific indicator of
disease activity in sarcoid
• ESR and CRP: non-specifically raised
• Autoimmune screen: autoantibodies may suggest connectivetissue
disease

27. OTHER INVESTIGATIONS
• ARTERIAL BLOOD GAS ANALYSIS
• Tuberculin test – negative in 2/3 of sarcoidosis patients
• BRONCHOALVEOLAR LAVAGE- cellular profile , special
stains or studies
• Lung biopsy- TRANS BRONCHIAL BIOPSY , OPEN LUNG
BIOPSY

29. MANEGEMENT
PRINCIPAL AIMS:
(1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the
pulmonary parenchyma
(3) to palliate the manifestations of these diseases.

30. CORTICOSTERIODS
• Prednisone, 1 mg/kg
• Gradually tapered (5 mg/week) over several months to a
maintenance dose

31. IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or
immunosuppressive agents (Azathioprine) may be used in
patients who do not improve on steroid therapy or who
cannot tolerate corticosteroids

32. N-ACETYL CYSTEIN
• Antioxidant
• 600 mg PO tid added to prednisone and azathioprine, preserves
vital capacity and FVC and DLCO

33. PIRFENIDONE
• Antifibrotic
• Reduces acute exacerbations and reduction in FVC

34. OXYGEN THERAPY
• F or pa tients w i t h d o c u m e nted h y p ox i a –
S p O 2 <89% , P a O 2 <55m m H g
• Improv e s e x e r c is e tolerance

35. CONCLUSION
• DPLD is the better term , not a single disease
• IPF is the most common DPLD
• Restrictive Airway Disease
• Main treatment aim is to suppress inflammation to prevent further
destruction of the pulmonary parenchyma

36. Thank you !!