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 DISEASERESTRICTIVE THORACIC DISEASE Due to disease within the lungs Due to disease within the lungs

–ie Interstitial Lung Disease –ie Interstitial Lung Disease

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

• PathophysiologyPathophysiology impaired alveolar gas exchangeimpaired alveolar gas exchange- alveolar barrier to O- alveolar barrier to O22 exchange exchange

(ie Alveolar-Arteriolar barrier )(ie Alveolar-Arteriolar barrier )- CO- CO22 exchange unimpaired as alveolar ventilation exchange unimpaired as alveolar ventilation

normal (CO2 v soluble and blown off )normal (CO2 v soluble and blown off ): : PaOPaO22 ( ( SaO2) normal PaCOnormal PaCO22

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 ILDAETIOLGY OF ILD

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

AETIOLGY OF ILDAETIOLGY OF ILD

Inflam Infiltrate of alveolar walls (ie Alveolitis):Inflam Infiltrate of alveolar walls (ie Alveolitis): ••Granulomatous-alveolitisGranulomatous-alveolitis •Extrinsic- •Extrinsic-Allergic-Alveolitis Allergic-Alveolitis (Hypersensitivity (Hypersensitivity Pneumonitis-Type 3 reaction)Pneumonitis-Type 3 reaction)

- Farmers lung- Farmers lung- Avian (pigeon, budgie)- Avian (pigeon, budgie)

• • SarcoidosisSarcoidosis-Multisystem disease-Multisystem disease-Lymphadenopathy/Erythema nodosum -Lymphadenopathy/Erythema nodosum Uveitis/Myocarditis/NeuropathyUveitis/Myocarditis/Neuropathy

Aetiology of ILD

Alveolitis continuedAlveolitis continued•• Drug induced alveolitisDrug induced alveolitis

- Amiodarone- Amiodarone- Bleomycin, Methotrexate- Bleomycin, Methotrexate- Gold- Gold

•• Fibrosing alveolitis Fibrosing alveolitis - Rheumatoid , Cryptogenic - Rheumatoid , Cryptogenic

• Autoimmune(multisystem)Autoimmune(multisystem) -SLE, Polyarteritis,Wegeners,Churg-Strauss -SLE, Polyarteritis,Wegeners,Churg-Strauss

AETIOLGY OF ILD AETIOLGY OF ILD

Dust-diseaseDust-disease (Pneumoconiosis) (Pneumoconiosis)•• Pulmonary Pulmonary fibrosis fibrosis

- asbestosis- asbestosis- silicosis- silicosis

AETIOLGY OF ILD AETIOLGY OF ILD

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

EosinophilicEosinophilic•• DrugsDrugs - Nitrofurantoin- Nitrofurantoin•• FungalFungal - Aspergillosis- Aspergillosis•• ParasitesParasites - Ascaris, Filariasis- Ascaris, Filariasis•• Autoimmune vasculitis Autoimmune vasculitis -Churg-Strauss,Polyarteritis -Churg-Strauss,Polyarteritis

CLINICAL SYNDROME OF ILDCLINICAL SYNDROME OF ILD

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

Pulmonary fibrosis(honeycomb lung)Pulmonary fibrosis(honeycomb lung)End stage response to any inflammatory processEnd stage response to any inflammatory process

DIAGNOSIS OF ILD #1DIAGNOSIS OF ILD #1• History-eg occupation,drugs,pets,arthritisHistory-eg occupation,drugs,pets,arthritis

• Reduced lung volumesReduced lung volumes: : FEVFEV11 FVCFVC11 normal ratio > 75% normal ratio > 75%

: Peak flow normal: Peak flow normal

• Reduced gas diffusion (TLCO)Reduced gas diffusion (TLCO)

• Arterial oxygen desaturation (Arterial oxygen desaturation (PaOPaO22 SaOSaO22))

- at rest or on exercise- at rest or on exercise

DIAGNOSIS OF ILD #2DIAGNOSIS OF ILD #2

• Antibodies:Avian,Fungal,Auto-antibodies Antibodies:Avian,Fungal,Auto-antibodies (Rheumatoid,Antinuclear)(Rheumatoid,Antinuclear)

• Serum ACE and Ca raised in Sarcoid Serum ACE and Ca raised in Sarcoid

• Bilateral diffuse alveolar infiltrates on chest X-rayBilateral diffuse alveolar infiltrates on chest X-ray

• Echocardiogram to excl LVFEchocardiogram to excl LVF

• High resolution CT scan-InflammatoryHigh resolution CT scan-Inflammatory ground glass vsground glass vs Fibrotic nodular components of alveolar infiltrates Fibrotic nodular components of alveolar infiltrates

• Transbronchial or thoracoscopic lung biopsyTransbronchial or thoracoscopic lung biopsy -rarely -rarely indicatedindicated

TREATMENT OF ILDTREATMENT OF ILD

• Remove any trigger factorRemove any trigger factor- dust, drug, allergen- dust, drug, allergen

• Treat any inflammation-immunosuppressivesTreat any inflammation-immunosuppressives• 11stst line line

Prednisolone Prednisolone • 22ndnd line line

Azathioprine Azathioprine CyclophosphamideCyclophosphamideCylcosporin Cylcosporin

• OO22 if hypoxaemic if hypoxaemic

Erythema Nodosum-Sarcoidosis

Bilateral hilar lymphadenopathyand lung infiltrares -Sarcoidosis

Non caseating granuloma -Sarcoidosis

DIP-pre steroids

DIP-post steroids

Lymphocytic alveolitis and intralumenal macrophages