Fitz Respiratory PACES Notes
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Respiratory 1. Interstitial lung disease (most frequent by a long way!) 2. Bronchiectasis 3. Cystic fibrosis 4. Pneumonectomy/lobectomy 5. Pleural effusion 6. Chronic obstructive airways disease 7. SVC obstruction 8. Cor pulmonale 9. Apical fibrosis Oral presentation • Tachypnoeic? • Peripheral signs • Key findings • Consistent with …DD • Is there evidence of… o Asterixis? o Cor pulmonale? o Infective exacerbation Interstitial lung disease History: • Dry cough • SoB esp on exertion • Leg swelling • Occupational Hx • Hobbies • Drugs now, in past Examination • Clubbing • Signs of steroid use • Central cyanosis • Decreased symmetrical chest expansion • Bilateral fine end inspiratory crackles Pulmonary hypertension – a wave with raised JVP, Left parasternal heave, loud P2 What are the causes of lung fibrosis? • Idiopathic pulmonary fibrosis (aka CFA, or histological diagnosis of UIP) o Hamman-Rich syndrome is an aggressive fatal from of interstitial pneumonitis in young, otherwise well patients with little obvious cause. Rx supportive. • Connective tissue diseases o Rheumatoid lung – hands, nodules o SLE – facies o Systemic sclerosis – facies and hands o Polymyositis – prox muscle weakness or tenderness o Dermatomyositis – heliotrope rash and gottron’s papules o Sjorgren’s syndrome o MCTD o Ankylosing spondylitis - posture • Drugs – many o Anti-cancer: esp Methotrexate, Cyclophosphamide, busulfan, bleomycin o Anti-arrhythmics: Amiodarone o Anti-inflammatory: Sulfasalazine, gold o Anti-biotics: Nitrofurantion, sulphonamides • Radiotherapy – scars, localised, tattoos • Pneumoconioses o Asbestosis o Coal workers’ pneumoconiosis o Silicosis (silica) o Beryliosis – Beryllium (lightbulbs) o Byssinosis – cotton o Chalicosis – stone o Bauxite fibrosis o Baritosis – Barium – generally benign with striking CXR abnormalities • Sarcoidosis • Extrinsic allergic alveolitis o Alveolar inflammation due to hypersensitivity to inhaled organic particles (Type III and IV hypersensitivity) o Bird fancier’s lung o Cheese washer’s lung o Farmer’s lung o Hot tub lung o Malt worker’s lung o Miller’s lung o Wood worker’s lung How would you investigate a patient with fibrosis? • ABG – hypoxia • CXR – reticulonodular shadowing and honeycombing • Bloods incl. ESR, RF, ANA, Igs • Formal spirometry with a restrictive pattern and decreased gas transfer • HRCT Treatment? • MDT – PT, OT, SW, CNS • Oxygen, treatment of infections early • NIV if indicated • No Dx modifying Rx for IPF – palliate • Reduce exposure to cause • Steroids/Aza, Cyclophosphamide • Rarely single Lung transplant • Inform about occupational health compensation Causes of pulmonary fibrosis by zone Upper zone • Ank spond • Carconoma • EAA • Pneumoconiosis (except Asbestosis) • TB Middle zone • Sarcoid Lower zone • Asbestosis • IPF/CFA • RA/SLE Generalised • Chemotherapy Anywhere • Radiotherapy Pleural effusion What are the causes? • Transudates? o CCF o Hypoalbuminaemia o Renal failure o Hypothyroidism o Meig’s syndrome – right sided pleural effusion with ovarian mass • Exudate? o Malignancy . Primary . Secondary o Infection . Parapneumonic . Empyema . TB o Inflammatory . Rheumatoid . SLE o PE How do you distinguish between a transudate or an exudates? • Classically ><30g/L • However Light’s criteria more accurate o Effusion/Serum protein >50% o Effusion/Serum LDH >60% o Effusion LDH > 2/3 normal range Pneumonectomy Examination • Deformity of chest • Flattening of lateral side • Thoracotomy scar • Tracheal deviation to that side • Decreased expansion on that side • No AE in bases • Bronchial breathing in upper zones (Lobectomy As above but central trachea, and diminuished AE and percussion note. ) Indications for lung resection • NSC Lung Ca • Solitary pulmonary nodules of uncertain cause • Bronchiectasis – localised • Massive haemoptysis from bronchiectasis or benign or malignant lesion • TB treatment in pre-antibiotic era Thoracoplasty Rib removal Less dull, more AE If with bronchiectasis or upper lobe signs - ?old TB What are the types of lung cancer? What are the treatment options for lung cancer? How do you diagnose and manage TB? What are the causes of haemoptysis? How would you manage haemoptysis? Bronchiectasis Permanent pathological dilatation of distal airways with chronic infection Hx • SoB • Cough productive of purulent sputum • Infections • DM, diarrhoea, wt loss – CF • Sinusitis – Kartagener’s • Inflammatory disorder • Hx childhood infections Examination • Thin • High RR • Cyanosis • Clubbing • Sputum pot with purulent sputum • Hyperexpanded chest • Coarse late insp and exp crackles with clicks and wheezes DD of clubbing and crackles • Fibrosing alveolitis (but no clicks, wheeze or sputum) • Bronchial Ca with infection • Lung abscess Causes • Congenital o Primary ciliary dyskinesia o Kartagener’s syndrome (as above with sinusitis, bronchiectasis, situs inversus and infertility) o Anatomical defects • Cystic fibrosis • Bronchial obstruction from foreign body, Cancer, granuloma, LNs • Childhood respiratory infections – esp Pertussis, measles, mycoplasma • ABPA • Hypogammaglobulinaemia • Yellow-nail syndrome • Chronic aspiration • Inflammatory disorders o RA, SLE, Sjogren’s, Marfans Investigations • Sputum MCS • CXR – tramlines, ring shadows • HRCT • A1AT • Aspergillus skin prick test for ABPA • RF or CCP in RA • HIV • Sweat chloride test for CF • Exhaled NO for ciliary disorders Treatment • PT • Abx – oral or nebulised • Bronchodilators • Surgery Findings on Pulm function tests? • Reduced FVC and FEV1 with a reduced FEV1/FVC > 70%. Cystic fibrosis History: • Recurrent chest infections since childhood • Productive cough with sputum • SOBOE • Symptoms of malabsorption with diarrhoea, short statue and under-weight • Symptoms of diabetes Examination: On observation • Short statue/ thin • Oxygen – wall/concentrator • Nebuliser/ NIV • Antibiotics, mucolytics • Insulin needles or BM equipment • ?Portacath/hickman line • ?PEG Gross Clubbing of fingers and toes (maybe insulin testing marks) • Lines/ scars from neck lines • Reduced expansion • Bilateral coarse crackles with pops, clicks and wheeze consistent with bronchiectasis • Possible Cor pulmonale What is cystic fibrosis? What are the major medical complications of cystic fibrosis? What is the management of cystic fibrosis? • Education o Patient, partner, family o Genetic testing • RFs o smoking o contact with MDR organisms • MDT o Dietitian – supplements/ feed o PT – postural drainage o OT – home modifications o CNS – Chest and DM o Drs – Chest, Endocrine, Gastro o Transplant coordinators • Medical o Bronchodilators and mucolytics o ABx – PO, IV, Nebs o Oxygen/NIV o Supplemental nutrition and creon o Diabetes care with insulin and .