Interstitial Lung Disease

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Interstitial Lung Disease
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Interstitial Lung Disease

• Diseases of lung interstitium
• Not a single disease over 250 different
  diseases with similar clinical and/or
  radiological features
• Also known as Diffuse Parenchymal Lung Disease
  or Diffuse Lung disease because of Involvement of
  air spaces , vessels, airways, ?pleura - i.e.
  diffuse involvement
• Idiopathic pulmonary fibrosis (IPF) or Idiopathic
  Interstitial Pneumonias (IIPs) are sometimes used
  synonymously with ILD

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(No Transcript)
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Characteristics

• Common clinical, radiological, physiological and
  histo pathologic features
• Hetero genous conditions
• - Inflammatory
• - Granulomatous
• - Infections
• - Depositions
• - Oedema

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Causes and Pathogenesis

• Exact etiology, not know Idiopathic
• Secondary to other known causes
• Inflammation, damage and fibrosis
• Repair mechanisms (for inflammation) - aberrant
• Diffuse involvement
• Progressive

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AETIOLOGICAL CLASSIFICATION

1. Occupational and environmental exposures
2. Connective tissue diseases
3. Drugs and Poisons
4. Infections (residual scars)
5. Miscellaneous systemic dis
6. IDIOPATHIC (or PRIMARY)

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Primary ILDs - IIPs

• UIP Usual Interstitial Pneumonia or IPF
• AIP Acute Interstitial Pneumonia
• DIP Desquamative Interstitial Pneumonia
• NSIP Non-specific Interstitial Pneumonia
• LIP Lipoid Interstitial Pneumonia
• OP Organizing pneumonias

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Secondary I.L.Ds

• Acquired /Systemic Inhertied
• CTDs Familial
• Granulomatoses Tuberous sclerosis
• Pneumoconiosis Neurofibromatosis
• Infections Metabolic
  storage disease
• R.B-I.L.D H-P syndrome
• Hypersenstivity Miscellaneous
• Pneumonias Idio. pulm. haemosiderosis
• Iatrogenic Veno-occlusive disease
• Drugs, Radiations L.A.M.
• Toxic gases,fumes Histiocytosis

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Connective Tissue Disorders

• PSS Skin and digital changes
• Rh arth Polyarticular
  arthritis
• SLE Multisystem disease
• PM-DM Skin and muscle
• Sjogren Dryness in
  eyes, mouth, glands
• Ank Spond Seroneg. Spondylo-arthritis
• Mixed CTD SLE, SSC and PM-DM
• Relapsing PC Polychondritis (nose, ear)
• Behcet Dis. Aphthous ulcers genital

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Iatrogenic ILD

• Drugs
• Cytotoxic Bleomycin, mitoC, busulfan, BCNU
• Noncytotoxic Aspirin
• Antibiotics NFT, sulfasalazine
• Anti arrythmics Amiodasone, BB
• Anti-inflammatory NSAIDs, gold
• Anticonvulsants Dilantin, CM
• Vasodilators Hydralazine
• Narcotics Opiates, heroin
• Miscellaneous Penicillamine,
  vitamins
• Therapeutic radiation
• Oxygen toxicity

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Drugs and Chemicals

• Chemotherapeutic agents
• Busulphan, Metho, Bleo
• 2. Antibiotics
• Misc. drugs Dilantin, Gold,
• Procainamide, Aminodarone
• Fumes of Zn, Cu, Mn, Cd, Fe,
• Mg, Ni, Se, brass
• Aerosols Fats
• Vapours Resins, TDI, Hg
• Paraquat

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SYSTEMIC DISEASES

• Sarcoidosis
• Vasculitides
• Wegeners GM
• Churg Strauss Syn
• Good Pastures syn
• Idiopathic haemosiderosis
• Histiocytosis X
• Lymphangitis carcinomatosis
• Ch. Pulm oedema / uraemia
• Alveolar proteinois

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Honey Comb Lung

• Irreversible end-stage disease
• Cystic space formation
• Dense fibrosis
• Metaplastic cuboidal epithelium
• Mucus formation
• Interstitial smooth m. proliferation
• Pulmonary vascular change
• Malignant change (potential)

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DIAGNOSIS

• Clinical features
• Laboratory investigations Hematological,
  biochemical, immunological etc.
• Radiology CXR, HRCT
• Pulmonary function tests
• Bronchoscopy BAL, lung biopsy
• Surgical lung biopsy

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Diagnosis Issues

• Diffuse vs. Local
• Primary vs. Secondary
• Cause of secondary ILD
• Acute vs Chronic
• Disease activity and progression
• Responsive vs Nonresponsive
• (to tmt)
• 7. Presence of complications

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DIFF. DIAGNOSIS

• Causes of breathlessness
• . Left heart failure
• . Pulm. Thromboembolism
• . Miscellaneous-anemia
• Respiratory Diseases
• . COPD
• . Bronchiectasis
• . Ch. Pneumonias- eosinophilic

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SYMPTOMS

• Progressive breathlessness
• Cough (non productive)
• Onset Insidious / acute
• Symptoms / history of underlying disease/exposure

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PHYSICAL EXAMINATION

• Tachypnoea
• Reduced chest expansion / intercostal retraction
• Exercise induced cyanosis
• Clubbing
• Breath sounds well heard
• Bibasilar end-inspiratory day (Velcro) crackles
• Pulm hypert / cor pulmonale
• Signs of CTD or other causes responsible for ILD

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INVESTIGATIONS

• Haematological leucocytosis, Polycythaemia /
  anaemia
• Biochem (renal, Liver)
• Raised ESR, hypergammaglob.
• Immunological tests LE cell, Rhematoid factor,
  Antinuclear Ab., Immune complexes
• Cardiac function
• Others ACE, LDH, antibodies (etc.)

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RADIOLOGICAL STUDIES

• What is most helpful?
• Diagnosis
• Disease extent
• Progression
• Radiological findings distribution and pattern
• Correlation
• Cost-effectiveness

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CLASSICAL RADIOGRAPH

• Basilar / diffuse shadows
• reticular/nodular/RN
• Mixed patterns ?
• Honey combing
• Decreased volume
• Pulm hypertension

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ATYPICAL PATTERNS

• Upper zone predominance Granulomatous dis.,
  Pneumoconioses
• Increased volume LAM, Sarcoidosis
• Pleural involvement CTD, sarcoidosis,
  Asbestosis, Drugs
• Miliary pattern TBL, Granulomatoses

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• 5. Hilar / mediastinal LN
• Sarcoidosis
• Lymphoma / Lymphangitis
• Pneumothorax
• Histocytosis X / LAM
• Kerley B lines
• Normal CXR

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HIGH RESOLUTION CT

• Better resolution / more accuracy
• Earlier detection
• Better assessment of extent and distribution
• Occult adenopathy
• Selection of biopsy site

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PULM. FUNCTION TEST

• Restrictive pattern
• Reduced TLC, VC
• FRC and RV
• Flow rates Reduced due to decreased VC
• . Obstructive/mixed pattern
• smoking/other causes
• Compliance Low
• DLCO Reduced
• Blood gases

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BRONCHO ALVEOLAR LAVAGE

1. Nonspecific
2. Narrow down diff. diagnosis
3. Defines stage of disease
4. Assessment of progression
5. Assessment of treatment response

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ACTIVITY ASSESSMENT

• Symptomatology
• Chest radiography
• Pulm. Function tests
• BAL ? TBLB
• Scanning Ga-67,
• TC99m DTPA

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TREATMENT
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Objectives of Treatment of ILDs

1. Symptomatic relief
2. Slow down disease progression
3. Prevent/ Treat complications
4. Prolong survival
5. Improve Quality of Life
6. Prevent drug-induced problems
7. End of Life Care

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Treatment Principles

• I. Secondary ILDs
• Treatment of ILD of a known primary cause
  essentially comprises the treatment of the
  primary disorder.
• Symptomatic
• Anti-inflammatory
• Supportive
• II. Primary ILD (Idiopathic Interstitial
  Pneumonias) and Pulmonary Fibrosis

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Treatment of IIP/IPF

• Of all IIPs, Idiopathic Pulmonary Fibrosis (IPF)
  i.e. U.I.P. is the most common form.
• It is associated with an extremely poor prognosis
  for survival in most patients.
• Life expectancy after diagnosis varies, but is on
  average less than 5 years.

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Current Drug Treatment

• 1. Corticosteroids
• 2. Immunosuppressive drugs Azathioprine,
  Cyclophosphamide, Cyclosporine
• 3. Antifibrotic drugs Pirfenidone,
  Colchicine, D-penicillamine, Pentoxyfylline
• 4. Anti-oxidants N. Acetyl-cysteine

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Supportive Treatment

• Underlying cause Identification and management
• Oxygen therapy
• Management of pulmonary hypertension and cardiac
  failure
• Pulm. Vasodilators
• Diuretics
• 4. Antibiotics for infections
• 5. Miscellaneous

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End Stage Disease

• Lung Transplantation
• Palliative End of Life Care
• Domicilliary Oxygen
• Symptomatic relief
• Discontinuation of steroids and immunosuppressive
  drugs

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Sarcoidosis

• Multi-system granulomatous disorder
• Unknown etiology
• Pulmonary features gt 80 (Interstitial
  infiltrates/fibrosis)
• Extrapulmonary (Eyes, skin, liver, spleen,
  nervous systme, cardiac etc) Approx. 30
• Increasing recognition
• Diagnosis primarily on lung biopsy Serum ACE
• Treatment Corticosteroids and other
  anti-inflammatory drugs

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