1. 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

2. RESTRICTIVE THORACIC DISEASE Due to disease within the lungs –ie Interstitial Lung Disease Disease of alveolar structuresDisease of alveolar structures - alveolar walls/lumen ( lung interstitium ) PathophysiologyPathophysiology  impaired alveolar gas exchange - alveolar barrier to O 2 exchange (ie Alveolar-Arteriolar barrier ) - CO 2 exchange unimpaired as alveolar ventilation normal (CO2 v soluble and blown off ) :  PaO 2 (  normal PaCO 2 :  PaO 2 (  SaO2) normal PaCO 2

3. 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

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

5. AETIOLGY OF ILD Inflam Infiltrate of alveolar walls (ie Alveolitis): Inflam Infiltrate of alveolar walls (ie Alveolitis): Granulomatous-alveolitis Extrinsic-Allergic-Alveolitis (Hypersensitivity Pneumonitis-Type 3 reaction)Granulomatous-alveolitis Extrinsic-Allergic-Alveolitis (Hypersensitivity Pneumonitis-Type 3 reaction) - Farmers lung - Avian (pigeon, budgie) Sarcoidosis- Multisystem disease- Lymphadenopathy/Erythema nodosum Uveitis/Myocarditis/Neuropathy Sarcoidosis- Multisystem disease- Lymphadenopathy/Erythema nodosum Uveitis/Myocarditis/Neuropathy

6. Aetiology of ILD Alveolitis continued Drug induced alveolitisDrug induced alveolitis - Amiodarone - Bleomycin, Methotrexate - Gold Fibrosing alveolitis - Rheumatoid, CryptogenicFibrosing alveolitis - Rheumatoid, Cryptogenic Autoimmune(multisystem) -SLE, Polyarteritis,Wegeners,Churg-StraussAutoimmune(multisystem) -SLE, Polyarteritis,Wegeners,Churg-Strauss

7. AETIOLGY OF ILD Dust-disease (Pneumoconiosis)Pulmonary fibrosis - asbestosis - silicosis

8. AETIOLGY OF ILD Carcinomatosis Carcinomatosis Lymphatic (adenoca) - bronchus,breast,prostate,colon,stomachLymphatic (adenoca) - bronchus,breast,prostate,colon,stomachEosinophilic Drugs- NitrofurantoinDrugs- Nitrofurantoin Fungal- AspergillosisFungal- Aspergillosis Parasites- Ascaris, FilariasisParasites- Ascaris, Filariasis Autoimmune vasculitis -Churg-Strauss,PolyarteritisAutoimmune vasculitis -Churg-Strauss,Polyarteritis

9. CLINICAL 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) End stage response to any inflammatory process

10. DIAGNOSIS OF ILD #1 History-eg occupation,drugs,pets,arthritisHistory-eg occupation,drugs,pets,arthritis Reduced lung volumesReduced lung volumes :  FEV 1  FVC 1 normal ratio > 75% : Peak flow normal Reduced gas diffusion (TLCO)Reduced gas diffusion (TLCO) Arterial oxygen desaturation (  PaO 2  SaO 2 )Arterial oxygen desaturation (  PaO 2  SaO 2 ) - at rest or on exercise

11. DIAGNOSIS OF ILD #2 Antibodies:Avian,Fungal,Auto-antibodies (Rheumatoid,Antinuclear)Antibodies:Avian,Fungal,Auto-antibodies (Rheumatoid,Antinuclear) Serum ACE and Ca raised in SarcoidSerum 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-Inflammatoryground glass vs Fibrotic nodular components of alveolar infiltratesHigh resolution CT scan-Inflammatoryground glass vs Fibrotic nodular components of alveolar infiltrates Transbronchial or thoracoscopic lung biopsy-rarely indicatedTransbronchial or thoracoscopic lung biopsy-rarely indicated

12. TREATMENT OF ILD Remove any trigger factorRemove any trigger factor - dust, drug, allergen Treat any inflammation-immunosuppressivesTreat any inflammation-immunosuppressives 1 st line Prednisolone1 st line Prednisolone 2 nd line2 nd line Azathioprine Cyclophosphamide Cylcosporin O 2 if hypoxaemicO 2 if hypoxaemic

13. Erythema Nodosum-Sarcoidosis

14. Bilateral hilar lymphadenopathy and lung infiltrares -Sarcoidosis

15. Non caseating granuloma -Sarcoidosis

16. DIP-pre steroids

17. DIP-post steroids

18. Lymphocytic alveolitis and intralumenal macrophages