ILD -Interstitial lung disease

1. INTERSTITIAL LUNG DISEASE
Dr Praveen Raman Mishra PG 3rd year
Dr Akash Bharti PG 2nd year

2. INTRODUCTION
• Interstitial Lung Disease refers to a broad range of conditions that
have common clinical, physiological, and radiological features.
• 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”.

3. • 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

4. 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.

7. Diagnostic Approach

8. Respiratory Symptoms
• Breathlessness (most common): Initially, dyspnea on exertion→ later
at rest
• Nonproductive cough

9. Physical Examination
• Typical ‘velcro’ crepts - IPF, crepts frequently absent in sarcoidosis.
• Clubbing- IPF, DIP, IBD.
• Skin involvement- Sarcoidosis, CTD, Vasculitis, Tuberous sclerosis.
• Arthritis- CTD, sarcoidosis.
• Eye changes (uveitis, conjunctivitis)- , Sarcoidosis, CTD.
• Muscle weakness- Polymyositis, dermatomyositis.
• Neuropathy- Sarcoidosis, CTD.
• Lymphadenopathy- Sarcoidosis, CTD.

10. Gastrointestinal symptoms
• Esophageal motility problems- systemic sclerosis and polymyositis
• Chronic, intermittent aspiration can lead to progressive fibrotic lung
disease.
• Bloating and diarrhea- inflammatory bowel disease

11. Musculoskeletal Symptoms
• Connective tissue disease- arthralgias, morning stiffness, joint swelling
and erythema.
• Swollen fingers (“sausage digits”) may be observed in systemic
sclerosis and polymyositis.
• Raynaud’s phenomenon- scleroderma, mixed CTD, SLE.

12. Ophthalmologic Symptoms
• Dry eyes- Sjögren syndrome
• h/o uveitis may have- SLE or sarcoidosis

13. Dermatologic symptoms

15. Systemic lupus erythematosus
• malar rash,
• photosensitivity skin reaction,
• hair loss

16. • H/o wheezing- hypersensitivity pneumonitis, eosinophilic pneumonia
or sarcoidosis.
• H/o pleuritic chest pain- serositis in a patient with CTD, or
pneumothorax from LAM, LCH.
• Hemoptysis- diffuse alveolar hemorrhage

17. AGE
• IPF most commonly occurs
in patients aged >60 years
• <50 years rare
• 20-40 years
1) Sarcoidosis
2)CTD associated ILD
3)LAM
4)PLCH
GENDER
Female-premenopausal
1.LAM
2.Tuberous scelerosis
Men-
1.RA
2.IPF and occupational
related ILDs

18. Time Course of Disease Onset
Acute: [days to week]
• 1.AIP
• 2.eosinophilic pneumonia
• 3.hypersensitivity pneumonitis
Subacute : [weeks to month]
1.sarcoidosis
2.drug induced ILD
3.Alveolar hemorrhage syndrome
4.COP
Chronic: months to years
• 1.sarcoidosis
• 2.PLCH
• 3.CTD

19. PAST MEDICAL HISTORY
• Prior diagnosis of connective tissue disease
• Case of HIV disease- lymphocytic interstitial pneumonia (LIP) are
common.
• h/o acute or chronic kidney disease might suggest underlying
vasculitis, pulmonary– renal syndromes, or CTD.
• h/o liver disease could suggest sarcoidosis, primary biliary cirrhosis.
• h/oAsthma and allergic rhinitis - GPA

20. MEDICATION
HISTORY
• Nitrofurantoin
• Amiodarone
• Bleomycin
• Methotrexate
• Azathioprine
• Rituximab
FAMILY
HISTORY
• Percentage of familial pulmonary
firbrosis varies 5 to 20 %
1.non specific interstitial pneumonia
2.desquamative interstitial pneumonia
3.unusual interstitial pneumonia

21. SMOKING
HISTORY
1.IPF
2.PLCH
3.resoiratory broncholitis
4.DIP
OCCUPATIONAL
HISTORY
• Inorganic Exposure
• Organic Exposure

23. Chest Imaging
• Abnormal chest radiograph is often the first indication of underlying
ILD

24. Pattern of ILD

28. HRCT
• More sensitive than chest radiograph
• Radiographic Characteristics of the UIP Pattern “Definite UIP”
• Peripheral, subpleural distribution
• Basilar predominance
• Reticular markings and traction bronchiectasis
• Honeycombing
• Absence of inconsistent features

29. Pulmonary Function Test
• Most forms of ILD demonstrates a restrictive ventilatory defect due to
decreased compliance and increased recoil of the lung parenchyma.
• Presence of obstruction suggests either concomitant obstructive lung
disease, or the presence of an airway-centered lung ILD such as LCH,
LAM or sarcoidosis.

30. BRONCHOSCOPY
• Useful in the diagnosis of DPLD.
• Inspection of the upper and lower airways, bronchoalveolar lavage
(BAL), and the performance of transbronchial lung biopsy.
• BAL:
1)Cell count and differential,
2) Cytology
3) Viral assays
4) Microbiologic cultures

31. • Blood lavage specimens- diffuse alveolar hemorrhage
• milky white BAL fluid- pulmonary alveolar proteinosis
• BAL eosinophilia (>25%)- acute eosinophilic pneumonia
• BAL lymphocytosis - granulomatous ILD, suggestive of
hypersensitivity pneumonitis, drug reaction, or cellular NSIP

32. • Positive lymphocyte proliferation assay in chronic beryllium disease.
• Asbestos bodies in asbestosis.
• CD1a positive cells on flow cytometry may lead to a diagnosis of
LCH.
• In the immunocompromised host, BAL fluid is highly sensitive for the
diagnosis of bacterial, viral, fungal, and mycobacterial diseases.

33. SURGICAL LUNG BIOPSY
• Despite a high yield in certain forms of lung disease, the utility of
transbronchial biopsy for most of the IIP (such as IPF, NSIP, and LIP)
is low and surgical biopsy is often required for accurate diagnosis.
• The usual technique is video-assisted thoracoscopic surgery (VATS)
that has a low morbidity and mortality in selected populations.

34. Idiopathic Interstitial Pneumonia

35. UIP or IPF
• MC of all chronic ILD
• Typical c/f presentation
• Median survival approximately 3 years,
depending on stage at presentation.
• B/L Reticular bibasilar and subpleural opacities.
minimal ground-glass and variable honeycomb
change.
• Type I pneumocytes are lost, and there is
proliferation of alveolar type II cells. "Fibroblast
foci" of actively proliferating fibroblasts and
myofibroblasts.

38. Patchy,sometimes migratory,subpleural consolidative opacities often with
associated ground-glass opacities in COP/BOOP.

42. Smoking –related ILD
• 1) Desquamative Interstitial Pneumonia(DIP)
• 2) Respiratory Bronchiolitis–assciated Interstitial Lung Disease(RB-ILD)
• 3)Langerhan’s cell Histiocytosis(PLCH)
• Examples:
• A)young male+smoker+nodules& cysts in UL+ spontaneous
pneumothorax= PLCH
• B)Basal GGO with cysts in a smoker= DIP
• C)Bronchial wall thickening+centrilobular nodules in a smoker= RB-ILD

43. Occupational ILD

44. Coal worker pneumoconiosis
Rounded opacities between 1 and 5 mm (upper and
middle zones)
small irregular and linear opacities Progressive
massive fibrosis almost always starts in an upper
zone Calcification is not a feature
Cavitation of PMF can occur
Caplan's syndrome is the name given to the
combination of rheumatoid disease and several
round nodules (usually 1 to 5 cm in diameter) in the
lungs of a coal miner.

45. Silicosis
Active molucule:free silica or crytalline(quartz).
Clues to diagnosis : Micronodular pattern and crazy paving
pattern on HRCT
2 forms:a)Acute silicosis: within 2 yrs
b)Chronic silicosis:10-30yrs
Simple silicosis : Upper lobes Small multiple nodules Egg
shell calcification
Complicated : >1 cm nodules
BAL yields a PAS-positive milky white fluid
A/w- increased incidence of TUBERCULOSIS

46. Asbestosis
Clues to diagnosis X Ray: reticular interstitial
pattern pleural plaques ( lower lung field ,
cardiac border and diaphragm ) Irrregular linear
opacities first noted in lower lung fields.
HRCT : Distinct subpleural curvilinear opacities
5-10 mm length parallel to pleural surface
BAL: Asbestos bodies
Most common asbestos-related cancer-
Adenocarcinoma of Lung.
Mesotheliomas –Tumors that arise from
mesothelial cells that line the pleural cavities

47. ILD in vasculitis
Suspect if Mononeuritis mutiplex Renal involvement Skin
lesions haemoptysis
MC seen is Wegeners Granulomatosis
X ray : consolidation, typically resolving within a matter of days,
multiple abcesses
HRCT : ground-glass partial alveolar filling. Hb : anaemia ( iron
defeciency )
BAL :- frank blood-staining in sequential lavage (acute
presentation) and numerous macrophages containing iron,
identified by Perl's stain
DLCO :- may be increased in acute conditions but is chronically
low

49. ILD ASSOCIATED WITH CONNECTIVE TISSUE DISEAE
• 1)Rheumatoid Arthritis – UIP Pattern
• 2)scleroderma –Diffuse
• 3)Dermatomyositis/polymyositis
• 4)Sjogran’s disease
• 5) SLE

51. Granulomatous ILD
• 1) Sarcoidosis :
• A multisystemic disoder of unknown cause having Non caseating granuloma in
lungs and other systems.
• F>M(20-40yrs)
• DIAGNOSIS-
• 1) Chest X-ray- B/L- Hilar adenopathy(Bat-wing type)
• 2)ACE level
• 3) sr Ca2+
• 4)Transbronchial Biopsy to see Non-caseating granuloma(IOC)
• Treatment:a) self resolving over 2-3 yrs b) Corticosteroids (prednisone)
c) Methotraxate
• 2) Hypersensitivity Pneumonia

54. Rare ILD

58. Pulmonary alveolar proteinosis

59. Pulmonary alveolar microlithiasis

60. Treatment objectives in ILD
• 1. Provide symptom-relief
• 2. Slow down disease progression
• 3. Prevent complications
• 4. Improve quality of life
• 5. Prolong survival
• 6. Prevent treatment-complications
• 7. End-of-Life care and palliative treatment

61. Treatment
• REMOVAL FROM EXPOSURES
• IMMUNOSUPPRESSIVE THERAPY
• ANTIFIBROTIC DRUGS
• TREATMENT OF COMORBIDITIES
• PALLIATIVE CARE
• LUNG TRANSPLANTATION

62. Removal From Exposures
• Drug reaction is suspected- should be discontinued
• Mold growth, removal of birds from the home, extensive cleaning of
upholstery, window coverings, and ventilation systems.
• Occupational exposures- avoided

63. Immunosuppressive Therapy
• Some forms of ILD, including COP, CTD– associated ILD, and
sarcoidosis, shows favorable response to steroids and other
immunosuppressive agents.
• When a more prolonged course of therapy is anticipated, azathioprine
or cyclophosphamide, permit low dose of steroids.
• If no clinical improvement is seen after 3 to 6 months of therapy,
discontinuation of immunosuppressive therapy should be strongly
considered.

64. Antifibrotic Drugs
• Useful in progressive fibrotic lung diseases.
• 1) Pirfenidone, a small-molecule drug which have antifibrotic
properties.
• stabilize lung function.
• 1) Nintedanib- anti PDGF

65. Lung Transplant
• Most patients with ILD referred for lung transplantation have IPF-
advanced stage.
• a severely impaired DLCO (< 39%) as well as advanced fibrosis on
HRCT predict poor survival and are considered to be triggers for
active listing.

66. Thank you