Interstitial Lung Disease

Definition
1. ILD
  1. Disorders that result in disruption of the distal lung parenchyma
2. Interstitium
  1. alveolar walls and septae contains the lung connective tissue elements
3. Fibrosis
  1. extensive alteration of alveolar/airway architecture
    1. aka Lung Scaring
    2. Alveoli is replaced by fibrotic tissue
    3. cause loss of the tissue’s ability to transfer oxygen
Classification
1. Aetiology
  1. Idiopathic
    1. IPF
    2. sarcoidosis
    3. langerhans cells histiococytosis
  2. Collagen Vascular
    1. Scleroderma
    2. RA
  3. Drug
    1. MTX
    2. Radiation
  4. Occupational
    1. Inorganic
      1. Asbestosis
      2. Silicosis
    2. Organic
      1. Hypersensitivity Pnemonitis
        aka Extensive Allergic Alveolitis
        Antigen
        Thermophillic Actinomycetes
        Aspergillus
        Avian
        Type III Hypersensitivity
        Progress to chronic
        Type IV hypersensitivity
        IgE
        Allergen avoidance
        Onset after 6-8 hrs
        SnS
        Dry Cough
        SOB
        Fever
        Basal Creps
        Predictor
        Exposure
        increased after SnS exposure
        recurrent episode
        Crackles
        weight Loss
  5. Post Infection
    1. TB
      1. Upper Lobes
      2. No ongoing Inflammation
2. Radiology
  1. Upper Lobe
    1. CHHARTS
      1. Coalworkers
        Pneumoconiosis
        Caplan's Syndrome
        intrapulmonary nodules on CXR
        in addition to RA
      2. Hypersensitivity
      3. Histiocytosis
      4. Ankylosing Spondylitis
      5. Radiation
        Pneumonitis
        Dose : ~2500 rads
        6 mo after RTx
        responsive to steroids
      6. Tuberculosis
      7. Silicosis
  2. Lower Lobe
    1. RAIDS
      1. Rheumatoid Arthritis
        Pulmonary Nodules
        Pleuritis
        Pleural Effusion
      2. Asbestosis
      3. IPF
      4. Drugs
        MTX
        NItrofurantoin
        Amiodarone
        Prolonged O2
      5. Scleroderma
3. Histology
  1. more cellular the picture, the more treatment responsive
  2. UIP (Usual)
  3. NSIP (non specific)
  4. DIS (Desquamative)
  5. RD-ILD (Respiratory Bronchitis)
  6. LIP (Lymphocytic)
  7. AIP (Acute)
  8. BOOP (Bronchiolitis Obliterans Organizing Pneumo)
Investigation
1. Radiology
  1. CXR
    1. Bilat shadowing/infiltrates
    2. Patchy/Nodular
    3. Starts Peripheral
  2. HRes CT Thorax
    1. Gold standard
      1. Pattern
        IPF
        Subpleural
        Lower Lobe
        Peripheral
        Asbestosis
        COP
        Central
        Brocnhovascular thickening
    2. Reticular opacities
    3. Honeycomb cyst
    4. Traction bronchiectasis
2. PFT
  1. Spirometry
    1. Low FEV1
    2. Low FVC
    3. FEV1/FVC >80%
  2. Diffusion Lung Capacity
    1. Reduced
    2. Ddx
      1. Anemia
      2. Emphysema
  3. Lung Volume
    1. TLC reduced
3. Bloods
  1. FBC
  2. UNE
  3. LFT
  4. Inc Ca2+
  5. Rheumatoid Factor
  6. dsDNA
  7. anti-scl70
  8. anti-GBM
4. Functional Assess
  1. ABG
    1. type 1 resp fail
  2. 6 min walk
    1. +-Echo
    2. exertional desaturaion
5. Bronchoscopy
  1. EBUS-TPNA
    1. ENDOBRONCHIAL ULTRASOUND-GUIDED TRANSBRONCHIAL NEEDLE ASPIRATE
    2. for sarcoidosis
6. Bx
  1. VATS
    1. only atypical progressive
Signs
1. Pulmonary
  1. PFT
  2. Chest
    1. Rapid Shallow Breathing
    2. Central Cyanosis
    3. Tracheal Deviation
    4. Reduced chest expansion
    5. Ascultation
      1. FIne end crepitation
      2. crackle (velcro)
    6. Tactile Fremitus
      1. Increased
      2. Normal
    7. Percussion
2. Extra Pulmonary
  1. Hands
    1. Clubbing
    2. Slerodactaly
    3. Raynaud's Phenomena
    4. Onycholysis
  2. Head and Neck
    1. Butterfly Rash
    2. Lupus Perino
    3. Keratoconjuctivits
    4. Uveitis (red eye)
  3. Others
    1. Erythema Nodosum
    2. `Myopathy
    3. Radiation therapy marks
History Taking
1. PC
  1. Progressive Exertional Dyspnoea
    1. 6 to 12 months
  2. Cough
    1. not productive
  3. NO wheeze
  4. Impressive radiological with no SOB
  5. Musculoskeletal Pian
  6. Photosensitivity
  7. Pleuritis
  8. Scleroderma Symptoms
    1. tightening of skin @ hands
    2. Raynaud's phenomenon
    3. Precede long before lung symptoms
2. Px
  1. Age
    1. IPF >60
    2. Sarcoidosis 20-40
  2. Smoking
    1. IPF
    2. RB-ILD
      1. Reversible with cessation
  3. Gender
    1. Women > Men
  4. Duration
    1. Acute/Subacute misDx as pnuemo
  5. Meds
    1. OTC meds
    2. Protein Supplements
  6. Occupation
    1. Duration
      1. extent
      2. Latency
    2. Organic (HP)
      1. Hay farming
      2. Mushroom
      3. Pigeon Fancier
    3. Inorganic
      1. Coal/Sandblasting?
        Silica
        Asbestos
IPF
1. Signs
  1. Clubbing
  2. Crepitation
  3. Pathogenesis
    1. Basement membrane changes
    2. Oxidative stress
    3. Procoagulant activity
    4. Vascular Remodelling
2. Symptoms
  1. >60 y/o
  2. male > woman
  3. exertional SOB
3. Treatment
  1. Poor prognosis
  2. NO effective Tx
  3. Steroid
    1. on suspicion of NSIP
    2. NO EFFECT
  4. Lung transplant <65y/o
  5. Palliative care
Sarcoidosis
1. Signs
  1. BHL+ Erythema Nodosum
    1. Good Prognosis
    2. Lofgren's syndrome
  2. Bi-hilar adenopathy
  3. Hypercalcemia
  4. Uveitis
  5. Keratoconjuctivitis
  6. Lupus Pernio
  7. Hepatic Granuloma
  8. Multi Organ
2. Symptoms
  1. Fever
  2. Fatigue
  3. Weight Loss
3. Treatment
  1. Steroid Responsive
  2. WHO?
    1. Hypercalcemia
    2. Critical Organs
      1. Cardiac
      2. Neuroligical
      3. Occular
  3. 20-40mg/day Prednisone
    1. Taper to low or QOD
    2. 9 to 12 mo
    3. if CNS / Cardiac
      1. Higher dose
    4. Relapse?
      1. MTX
      2. Adalinumab
      3. Plaquenil
4. Investigation
  1. Hx/Exam
  2. CXR
  3. PFT
  4. Urinary Ca2+
  5. Tuberculin test
  6. HIstology
    1. Non caseating granuloma
5. Systemic Granulomatous Disease

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Interstitial Lung Disease

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	Created by Amirul Aiman about 6 years ago