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Restrictive Lung Disease Overview

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Restrictive Lung Disease Overview Powered By Docstoc
					      RESTRICTIVE THORACIC DISEASE
    Thoracic Restriction due to causes
           out with the lungs
• Skeletal :Vertebrae-eg Thoracic kyphoscoliosis , Ribs –
  eg Traumatic multiple rib #s
• Muscle Weakness - Intercostal or Diaphragmatic (due to
  Myopathy/Neuropathy/Myelopathy)
  –eg Myaesthenia Gravis ,Guillan Barre, Motor neurone
  disease ,Poliomyelitis
• Abdominal Obesity/Ascites –compression of thoracic
  contents
• Results in Chronic alveolar under ventilation with low
  PaO2 ( SaO2) and raised PaCO2 and reduced lung
  volumes
RESTRICTIVE THORACIC DISEASE
Due to disease within the lungs
 –ie Interstitial Lung Disease

• Disease of alveolar structures
  - alveolar walls/lumen ( lung interstitium )

• Pathophysiology
    impaired alveolar gas exchange
   - alveolar barrier to O2 exchange
(ie Alveolar-Arteriolar barrier )
   - CO2 exchange unimpaired as alveolar ventilation
   normal (CO2 v soluble and blown off )
   : PaO2 ( SaO2) normal PaCO2
          Aetiology of ILD
• Fluid in the alveolar air spaces
• Cardiac Po oedema (in alv walls and lumen)
  due to raised Po venous pressure –ie LVF
• Non Cardiac Po oedema –Normal Po
  venous pressure with leaky Po capillaries -
  due to sepsis or trauma (Shock lung or
  ARDS)-due to Altitude sickness
         AETIOLGY OF ILD

Consolidation of alveolar air spaces:
• Infective pneumonia
- viral, bacterial, fungal, protozoal
• Infarction
- pulmonary emboli/vasculitis
• Other causes (ie BOOP)
- rheumatoid disease
- drugs
- cryptogenic
AETIOLGY OF ILD

 Inflam Infiltrate of alveolar walls (ie Alveolitis):

 •Granulomatous-alveolitis
 •Extrinsic-Allergic-Alveolitis
   (Hypersensitivity Pneumonitis-Type 3 reaction)
   - Farmers lung
   - Avian (pigeon, budgie)
 • Sarcoidosis
   -Multisystem disease
   -Lymphadenopathy/Erythema nodosum
   Uveitis/Myocarditis/Neuropathy
           Aetiology of ILD
Alveolitis continued
• Drug induced alveolitis
      - Amiodarone
      - Bleomycin, Methotrexate
      - Gold
• Fibrosing alveolitis
  - Rheumatoid , Cryptogenic
• Autoimmune(multisystem)
  -SLE, Polyarteritis,Wegeners,Churg-Strauss
AETIOLGY OF ILD

 Dust-disease
 (Pneumoconiosis)
 • Pulmonary fibrosis

 - asbestosis
 - silicosis
AETIOLGY OF ILD

Carcinomatosis
  • Lymphatic (adenoca)                                             -
  bronchus,breast,prostate,colon,stomach

Eosinophilic
   • Drugs                           - Nitrofurantoin
   • Fungal                          - Aspergillosis
   • Parasites                       - Ascaris, Filariasis
   • Autoimmune vasculitis           -Churg-Strauss,Polyarteritis
CLINICAL SYNDROME OF ILD

•   Breathless on exertion
•   No cough or wheeze
•   Lung crackles (inspiratory)
•   Finger clubbing
•   Central cyanosis (if hypoxaemic)


Pulmonary fibrosis(honeycomb lung)
End stage response to any inflammatory process
DIAGNOSIS OF ILD #1
• History-eg occupation,drugs,pets,arthritis

• Reduced lung volumes
  : FEV1 FVC1 normal ratio > 75%
  : Peak flow normal
• Reduced gas diffusion (TLCO)
• Arterial oxygen desaturation (PaO2 SaO2)
  - at rest or on exercise
              DIAGNOSIS OF ILD #2

• Antibodies:Avian,Fungal,Auto-antibodies
  (Rheumatoid,Antinuclear)
• Serum ACE and Ca raised in Sarcoid

• Bilateral diffuse alveolar infiltrates on chest X-ray

• Echocardiogram to excl LVF

• High resolution CT scan-Inflammatory ground glass vs
  Fibrotic nodular components of alveolar infiltrates
• Transbronchial or thoracoscopic lung biopsy -rarely
  indicated
TREATMENT OF ILD

• Remove any trigger factor
  - dust, drug, allergen

• Treat any inflammation-immunosuppressives
• 1st line
  Prednisolone
• 2nd line
  Azathioprine
  Cyclophosphamide
  Cylcosporin

• O2 if hypoxaemic
Erythema Nodosum-Sarcoidosis
Bilateral hilar lymphadenopathy
and lung infiltrares -Sarcoidosis
Non caseating granuloma -Sarcoidosis
DIP-pre steroids
DIP-post steroids
Lymphocytic alveolitis and intralumenal macrophages

				
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posted:8/13/2011
language:English
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