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
• Fungal
• Parasites
• Autoimmune vasculitis
- Nitrofurantoin
- Aspergillosis
- Ascaris, Filariasis
-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