An overview of ILD

1. Interstitial Lung Disease

2. Interstitial Lung Disease
• Broad Category of diseases characterized by
inflammation and fibrosis
– Grouped together because of similar
presentations, CXRs, and physiologic appearance
– Over 100 separate disorders
• Only some have know etiology

3. Known cause
Exposures
Systemic
Diseases
Environmental
(Inorganic dusts, asbestos),
Drug induced,
Radiation,
Smoking,
Oxygen,
Connective tissue
disease,
Inflammatory bowel
disease,
Sarcoidosis

4. Unknown cause
Idiopathic pulmonary fibrosis,
Nonspecific interstitial pneumonitis,
Cryptogenic organizing pneumonia,
Hamman-Rich Syndrome,
Eosinophilic Granuloma

5. Pathophysiology
• The lung is injured
• Inflammation results
• If repair is not correct:
Increased interstitial tissue (especially
connective tissue) replaces normal alveoli,
capillaries and healthy interstitium
– Fibroblasts in interstitium produce collagen
– Fibrotic cystic airspaces are produced

6. Normal Lung Tissue and small bronchi

7. Increased connective tissue

8. Lung architecture side by side

9. Signs , symptoms
• Excertional dyspnea
• nonproductive cough
• May have Pulmonary Hypertension, leading to
Cor Pulmonale

10. EXAM
• Inspiratory Crackles, diminished BS
• Occasionally wheezing - if airway involved
(like sarcoidosis)
• CXR: low lung volume with bilateral nodular
opacities / ground glass
• PFT: show restrictive disease: All volumes are
reduced, FEV1/FVC =

11. Gas Exchange
• ABG: Arterial PaO2 and PaCO2 are reduced, pH
normal, greater Hypoxemia with exertion
• Diffusion impairment contributes to hypoxemia on
exercise.
• Physiologic dead space and physiologic shunt and
VQ mismatch are increased.
• There is marked reduction in diffusing capacity due
to thickening of blood gas barrier and VQ mismatch.

12. CXR
Interstitial Disease Airspace disease

15. Interlobar thickening

16. Asbestosis - environmental
• Pleural plaques, fibrosis, effusions, atelectasis
and parenchymal scarring
• Exposure usually many years before disease
Pleural plaques
(bilat areas of fibrosis ,usually calcified)

18. Silicosis - environmental
• From chronic exposure to inhaling Silica
particles (sand, quartz)
– Mining, tunneling, sandblasting,
• Increased risk of lung CA and TB (2 to 30 fold
more active TB)

19. Coal Workers Pneumoconiosis-
environmental
• Coal Minors - very similar to Silicosis
• Simple CWP can be asymptomatic with + CXR
finding and not progress
• No true therapy other than alleviating
symptoms and exposure

20. Coal Workers Pneumoconiosis

21. Hypersensitivity Pneumonitis-
environmental
• Immune reaction to inhaled antigens
• Organic:
– Bacteria or Fungus:
– Farmer’s Lung - Moldy Hay
– Humidifier Lung - Home Humidifier systems
– Bird breeder’s lung
– Mushroom Worker’s lung
• Inorganic
– Vaporized paints and plastics

22. • Can be acute or chronic
• Acute:
– Present with Sudden SOB, Chest pain,
• Chronic = months or years and can be difficult
to distinguish from other ILD
• History taking is critical (hobbies, work, etc)
• Treatment is avoidance of antigen and
steroids
Hypersensitivity Pneumonitis

23. Drug induced
• Many drugs can cause Pulmonary fibrosis
– Index of suspicion, timing of drug
• Treatment supportive, corticosteroids and of
course, stop the drug
• Treatment for CA can result in ground glass at
site and beyond
• Treatment is supportive only
Radiation

24. NEW SLide: Drugs
• Antibiotics: nitrofurantoin, sulfasalazine
• Anti-inflammatory: aspirin, methotrexate
• Cardiovascular: amiodarone, tocainide
• Chemotherapeutic: bleomycin, methotrexate
• Illicit drugs: heroin, methadone, propoxyphene, talc
as an IV contaminant

25. Known cause
Exposures
Systemic
Diseases
Environmental
(Inorganic dusts, asbestos),
Drug induced,
Radiation,
Smoking,
Oxygen,
Connective tissue
disease,
Inflammatory bowel
disease,
Sarcoidosis

26. Connective Tissue Diseases
• Scleroderma, Rheumatoid arthritis, Sjogren’s
syndrome, Polymyositis, Dermatomyositis,
Sytemic Lupus Erythematosis have all been
connected with ILD
• Poor correlation between connective tissue
symptoms and severity of lung disease
• Treatment individualized

27. Sarcoidosis
• Multisystem granulomatous inflammatory
disorder usually involving the lung
• Inflammatory cells create granulomas in
several organs, lung most common
• Most common ILD in US
• more common with younger patients
• much more common in African Americans
than white
NOTE: Sarcoidosis is in the “known” box because we know it causes ILD,
BUT…we don’t know what causes Sarcoidosis

28. Non-caseating granulomas
in Sarcoidosis

29. • Symptoms can include chest pain and
wheezing, Inflammation of iris, adenopathy
• Corticosteroids for marked symptoms
Sarcoidosis

30. Unknown cause
Idiopathic pulmonary fibrosis,
Nonspecific interstitial pneumonitis,
Cryptogenic organizing pneumonia,
Hamman-Rich Syndrome,
Eosinophilic Granuloma
NOW TO THE …..

31. Idiopathic PULMONARY Fibrosis
• Progressive fibrotic lung disease isolated to
lung
• Usually > 60 with smoke, hairdressing
chemical, farming or metal dust exposure
• Lung biopsy needed to determine
• No real therapy, steroids used, but only
effective in few

32. Cryptogenic organizing PNA
• Inflammation of the bronchioles and
surrounding lung tissue
• The pneumonia is due to inflammation of the
lung tissue, not infection
– Often presents after failed PNA tx with antibiotics
• Used to be called BOOP
– bronchiolitis obliterans organizing pneumonia

34. Generalized therapy for ILD
• Oxygen
– Check sat at rest and with exertion – mild disease
will desat during exertion, even with a normal sat
at rest
• DO 6 MINUTE WALK TEST TO ASSESS DISEASE
PROGRESSION
– O2 may increase quality of life
– AS per CDC : pneumococcal and influenza
vaccinations if treated with immunosuppressive
medications
– Lung transplantation

35. Mechanical Ventilation
• Compliance is very low, so PIP will be______
• Vt 4-8 ml/kg
• Rate 20-35
• I time .5 - .8

36. Honeycomb Cystic Changes
End stage Fibrosis in ILD

40. General Treatment of NM Disorders
• Bronchial Hygiene techniques
– Assisted cough
– Postural drainage
• Assisted Ventilation
– NPPV
– Negative pressure Ventilators
– Mechanical Ventilation
• Anticipate pneumonia, PE
• Watch VC, MIF and intubate accordingly