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Restrictive Disease

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Introduction

Restrictive Lung diseases are a group of diseases, that cause direct damage to the alveoli. This normally occurs due to a group of diseases known as Interstitial Lung Diseases.

Otherwise they occur due to extra-pulmonary factors (Obesity, Neuro-muscular disease weakening the diaphragm (both beyond the scope of this guide) and pleural effusions [covered elsewhere])

Restrictive Lung diseases have a particular pattern on spirometry- covered: Spirometry Guide

Common Symptoms

  • All Interstitial Lung Diseases have the potential to cause FIBROSIS within the lungs. -SCARRING
    • Due to this- there is reduced elasticity within the alveoli causing reduced overall lung expansion.
      • This in turn results in reduced gas exchange leading to:
        • Cough
        • Shortness of breath
        • Fatigue
        • Weight loss
  • All also have common signs that can occur:
    • Finger Clubbing
    • Crepitations on auscultation

Idiopathic Pulmonary Fibrosis

What:

  • Idiopathic = UNKOWN CAUSE [We are idio-ts for not knowing]
  • The most common Interstitial Lung Disease

Who:

  • 70-75 year olds
  • VERY rare < 50 year old

Investigations:

  • Identify this condition through CXR:
  • Characteristic GROUND GLASS APPEARANCE
  • Thickened interstitium

Management:

  • Pirfenidone -slows progression, unknown why exactly

Sarcoidosis

What:

  • An autoimmune disorder causing SYSTEMIC NON-CASEATING GRANULOMATOUS INFLAMMATION
  • SELF LIMITING (usually) – can be chronic but rare

Who:

  • Most common in the young (20s-40s)- AFTER INFECTION

Specific Symptoms

Usually does not cause fibrosis – but 20% progress into fibrosis – making symptoms and disease prognosis much worse* – however common symptoms still true

  • Also have SWOLLEN SKIN RASHES – due to systemic inflammation- swollen fat:
    • ERETHEMA NODOSUM – on SHINS normally
      • A sign in other inflammatory conditions – but sarcoidosis should be on differential if present

Investigation:

  • CT scan – Bilateral Hilar Lymphadenopathy
  • ++ Serum ACE level
  • ++Ca2+
  • Skin rashes evident- Systemic inflammation causes ERETHEMA NODOSUM (Sign in other conditions but if have sarcoidosis a differential

Management :

  • Since self-limiting nothing specific- symptom management and inflammation reduction
  • Oral steroids
  • Analgesia
  • NSAIDs

Occupational Lung Disease

Pneumoconiosis

What

  • Coal workers lung
  • Can be:
    • Simple – No symptoms – just evidence on CXR
    • Complicated- Progressive symptoms due to fibrosis

Who

  • Coal Workers

Symptoms

  • Same as common symptoms
  • Additionally – BLACK SPUTUM

Investigations

CXR- Nodules evident +/- Fibrosis

Spirometry

Management

Nothing Helps – just manage symptoms

Just prevent through appropriate PPE

Asbestosis

What

  • Fibrosis as a result of heavy asbestos exposure
    • First Asymptomatic – with pleural plaqs
    • Progresses to Acute Pleuritis
    • Then restrictive impairment develops – with pleural thickening
    • Eventually MESOTHELIOMA CANCER – incurable – death in 2 years

Who

  • Lots of Occupations involved in asbestos work:
    • Mining
    • Construction
    • Ship building
    • Boilers + Piping
    • Car Building

Investigations

  • CXR – shows pleural plaqs, pleural thickening, pleural effusion, diffuse fibrosis
  • Sputum sample- asbestos bodies
  • Lung biopsies – asbestos fibres

Management

  • None

Cancer Associated Asbestosis

  • Bronchial cancer- Double risk in asbestosis+smoker
  • Mesothelioma – pleural cancer
    • AGGRESSIVE