Comprehensive Guide to Respiratory Medicine: Symptoms, Diseases & Treatments

1. Respiratory Medicine Alice Hart-George

2. Contents • ABCDE & symptoms: SOB, chest pain, cough/haemoptysis • 1. Respiratory Failure • 2. Asthma & COPD • 3. Bronchietctasis • 4. Granulomatous & Vasculitic Lung Disease - TB, Sarcoidosis, Wegener’s, Churg Strauss, Hypersensitivity pneumonitis (EAA) • 5. Infections • 6. Restrictive Lung Disease (Intrinsic v Extrinsic) • 7. Lung Cancer

3. A..B..CDE • A: Is the patient talking?  Airway patent. - Brief PC/PMH/Drugs&Allergy • B: Look: Colour, SOB, Accessory Muscles, RR, SpO2. Listen: Auscultate, Percuss. Cough, Wheeze, Silent. Feel: Trachea, Equal chest expansion • OXYGEN on (COPD: Venturi 24% SpO2 88-92%) - Sit them up • Peak flow, ABG, CXR, Temperature (+/- Blood culture), Sputum sample

4. Classic OSCE Presentations Pleural rub: non-specific sign occurring with pleural inflammation of any cause

7. *Haemoptysis • PE • FB • ‘Crack’ lung

8. LUNG FUNCTION: Spirometry results in lung disease…. Influenced by Age, Sex, Height, (Race)

9. 1. Respiratory Failure • Failure of Gas Exchange (O2 in, CO2 out) • Need blood (perfusion, Q) to come into close proximity to air (ventilation, V) • 2 Types: • HypoxaemicType I – low oxygen levels (ABG SaO2 <8kPa) • Hypercapnic Type II – low oxygen levels and high CO2 levels (ABG SaCO2 >6.7 kPa)

10. Hypoxaemic (Type I) • Causes: • V/Q mismatch, the air isn’t going to the same bits as the blood. E.g. Pneumonia, PE, Asthma, Pulmonary oedema. • Shunting of blood from pulmonary to systemic circulation, blood bypasses the lungs. • Hypercapnea. O2 and CO2 levels in alveoli interrelated. The more CO2, the less space for O2 • High altitude. Less O2 in the air. • Management: • Give more. High flow O2. Make sure it’s not Type II! • CPAP – Continuous Positive Airway Pressure • Reverse underlying cause. Treat the infection, break the clot

11. Hypercapnic (Type II) • Causes: • Ventilation. Either total ventilation (i.e. resp rate) or in dead space of lung. COPD, opiates, respiratory exhaustion • Inspired CO2. Only really an issue in intubated patients rebreathing their own CO2 • Management: • Natural Ventilation. Reverse sedating drugs, Give O2 • COPD: Restrict O2 Venturi 24% SpO2 88-92%, Neb Salbut. & Ipatrop Br. • Assisted Ventilation • Invasive – Intubate and give positive pressure ventilation to keep alveoli open and better ventilated. • Non Invasive (NIV) – BiPAP. Two different pressures, one for inhalation, one for exhalation. Bi PAP for type II.

13. 2. Asthma • Common chronic disease characterised by reversible expiratory airflow limitation, cough, SOB, wheeze, chest tightness, especially at night/with exercise. • HxAtopy (Eczema, Asthma, Hay fever, Allergies, IgE) • Diurnal variation in symptoms (& peak flow) • Examination can be normal. • Very common; 5-10% of population. Causes 2000 deaths/year, prevalence increasing in West.

14. Asthma • Can be classified as: • Extrinsic. Positive IgE, (‘allergic’). Occurs in childhood, response to environmental irritants, often associated with atopy. Often remits during teens, but may recur in later life. • Intrinsic. Normal IgE (‘non-allergic’), Develops later in life or post URTI. More progressive and less responsive to treatment.

15. Pathophysiology • Irritants cause bronchospasm and inflammation. Inflammation leads to oedema, mucus secretion, smooth muscle hypertrophy and fibrosis. • Stimuli : House dust mite, pollen, cat hair, viral infection, cold air, cigarette smoke, exercise, emotional stress. • Drugs contraindicated: BB, NSAIDs.

16. Lung function tests +/- reversibility study can help establish a diagnosis. • Peak flow diary, to demonstrate the morning dip. • Management • Education, especially re trigger avoidance and recognising deterioration. • BTS Therapeutic Ladder BTS/SIGN British Guideline on the Management of Asthma : 2012

17. BTS Stepwise approach to asthma BTS/SIGN British Guideline on the Management of Asthma : 2012

18. COPD • Chronic, progressive respiratory disorder characterised by airflow limitation of only limited reversibility • Very common, kills >20,000 people per year in UK • Risk factors: Smoking, smoking, smoking, atmospheric pollutants, alpha1 antitrypsin deficiency. • Chronic productive cough for 3 months each year for 2 consecutive years, or abnormal permanent dilatation of terminal air spaces.

19. COPD • Smoking causes inflammation, mucus gland hypertrophy and  mucus production. Airflow limitation in small airways. • Destruction of lung tissue and dilatation of distal airspaces which collapse. • Lungs lose pliability, loss of elastic recoil and gain more dead air space/trapping (hence hyperinflation), increasing the work of breathing and causing SOB. • As disease progresses, the poorly ventilated lung  tendency to accumulate CO2, so to compensate, the respiratory centres become tolerant to high blood CO2 and focus shifts to respiratory drive on hypoxaemia; ‘hypoxic drive’.

20. Presentation • Progressive productive cough (yellow/white), SOB, often over many years. Strong smoking history. Be aware of ‘asthma’ in older adults! Get a smoking hx! • Often exercise tolerance and wheeze • O/E: Hyperinflation, coarse crackles, wheeze, breath sounds, hyperresonance on percussion. Prolonged expiration, pursed lips, rail grabbing. • Spirometry helps confirm disease and assess severity. FEV1/FVC <70% (FEV180% predicted) • (Post nebs PEF can help differentiate from asthma if doubtful)

21. COPD • Hyperinflation >6 anterior ribs • Hyperlucent lung fields • Flattened hemidiaphragms • Exclude Lung Ca

22. Management • STOP SMOKING! • Short acting B2 Agonists, LAB2A, LAmuscarinic antagonist, Inhaled Corticosteroids, Anticholinergics. • Influenza & Pneumococcal vaccines • Pulmonary Rehab, especially if recent exacerbation • Complications: Respiratory failure, CorPulmonale, Pneumothorax, Polycythemia. • Discuss ceilings of treatment sooner rather than later. • Home O2 (24-28% Venturi: 88-92%)

23. Acute exacerbation of COPD • Like Pneumonia but NO CONSOLIDATION on CXR • Different Abx to CAP/HAP • Often worsening symptoms & purulent sputum • Ix: Basic obs, SpO2, Peak flow, ABG, CXR, Bloods (FBC, U&E), Sputum, Urine, Blood culture if febrile. • Rx: O2 Venturi 24-28% SpO288-92% • Neb Salbutamol 5mg & Ipatropium bromide 500mcg • Oral/IV steroid (Prednisolone 30mg PO 14/7) • Abx trust guidelines e.g. Amox 500mg TDS/ Doxy 200mg OD • Non invasive ventilation (NIV aka BiPAP) for ACIDOTIC CO2 retainers, during hypercapnoeic exacerbations PaCO2 ≥6 / pH ≤7.35 to  acidosis  mortality

24. 3. Bronchiectasis • Abnormal and Permanent dilatation of bronchi • Focal or diffuse impaired clearance of bronchial secretions 2 infection (chronic, recurrent). • Cough, Purulent sputum, Haemoptysis, Wheeze, SOB, Clubbing, Coarse inspiratory creps at bases.

25. Bronchiectasis • Ix: Sputum, Spirometry (Obstructive), CXR, HRCT (thick large airways larger than blood vessels), Blood Immunoglobulins, Sweat test. • Rx: Physio, bronchodilator, steroids, Abx if 2 infection. High resolution CT • Bronchial wall thickening • Dilated bronchi appear ‘beaded’ • Signet ring sign

26. 4. Granulomatous & Vasculitic Lung Disease • Granuloma: An organised collection of mature macrophages (aka histiocytes, often called epithelioid), accompanied by accessory features such as necrosis or infiltration by inflammatory leucocytes. Form when the immune system attempts to wall off a substance that it perceives as foreign but can’t eliminate it. The epithelioid macrophages fuse  Giant Cells (aka Langerhans Cells)

27. Granulomatous & Vasculitic Lung Disease • Many causes, differentiated by clinical phenotype/accessory features. • Infection: • TB • PCP/PJP • Autoimmune: • Sarcoidosis • Wegener’s Granulomatosis • Churg Strauss • Inflammation from foreign substance: Hypersensitivity Pneumonitis (aka EAA: extrinsic allergic alveolitis) • Bird fancier’s lung • Farmer’s lung • Mushroom Picker’s lung

28. TB • Most common ID killer worldwide • 1.4 million deaths 2010 • M tuberculosis, Acid-Fast Bacilli smear and culture. • Slow growing, obligate aerobic, intracellular parasite. Non spore forming, non-motile, only living in humans. • Recognised by time of Hippocrates as infectious, termed phthisis (wasting disease), used to be called consumption in Europe for same reason. • Rare in West unless risk factor present

29. TB • Mycobacteria are highly antigenic, promote vigorous non specific response, which is exactly what they need to do to get into phagocytes, where they set up home. Also provides means of spread via lymph nodes. • Extrapulmonary disease is rare except in immunocompromised, who get disease in Liver, Bone marrow, spleen, kidneys, bones (Pott’s Disease), and Brain. • Typical lesions are granulomas with epithelioid macrophages and Langerhans cells with central caseation and necrosis with a rim of fibrosis. • Initial lesions may heal and develop latent infection, before reactivating as pulmonary or generalised disease (anti TNF drugsCXR) • If unable to halt the initial infection, pt develops primary progressive TB, rapid onset of tuberculous pneumonia with purulent exudate.

30. Presentation • Cough, haemoptysis, weight loss, fever, night sweats • Risk: HIV, Exposure, Endemic area, Homeless/Prison, Overcrowding, Malnutrition, Immunocompromised. • Elderlycan just present with non resolving lung symptoms. • CXR: Consolidation, Apical cavitation/granuloma (Ghon focus), Hilar lymphadenopathy, fibrosis, calcification • TB meningitis: 2-3/52 headache, change mental state  Coma • Pott’s disease: Spinal lesions, pain, weakness • GU TB: UTI Sx or PID Sx/Epididymitis Sx • GI TB: Non healing ulcers, pain, malabsorption, diarrhoea, PR blood.

31. Management: RIPEs • Isolate and barrier nurse • 4 drug regimen: • E or S can be stopped once know not MDR • Check FBC, LFT, U&E, colour vision/acuity, peripheral neuropathy • DOT: direct observation of treatment

32. Sarcoidosis • Granulomas form in multiple organs, cause unknown. ?changes in immune system following exposure to an agent. • Usually affects lungs or lymph nodes, but can affect any organ, generally gradual onset. • Women slightly > Men, > African descent, in whom disease is both more prevalent and more likely to be symptomatic. • 2/3 people with radiological signs of Sarcoid are asymptomatic.

33. Diagnosis of exclusion • Differentials: • Lymphoma • Mets • Wegeners • 4 stages on CXR • 1: bilhilar LNs • 2: AND reticulonodular infiltrates • 3: bilateral infiltrates • 4: Fibrocystic; hilar retraction. Cystic and bullous changes

34. Sarcoidosis Mangement • NSAIDs – in mild disease • Steroids • Common steroid alternatives (Methotrexate, azathioprine) • Hydroxychloroquine (for bone involvement)

35. Wegener’s Granulomatosis(aka Granulomatosis with polyangiitis aka ANCA-associated granulomatous vasculitis) • Systemic Necrotizing Vasculitis • Autoimmune attack of small/medium vessels • via Anti-neutrophil cytoplasmic antibodies (c-ANCA+ 75-90%) either Cytoplasmic or Perinuclear activate neutrophils in affected tissues, targeting vessel endothelium Vasculitis and granuloma formation. • Unknown aetiology • AffectsURT, nose, LRT, lungs, kidneys • Rare (10 : 1,000,000) M=F > N Europeans (Caucasian 90%). Symptoms: Respiratory: Recurrent LRTI, Cough, SOB, Haemoptysis, nasal congestion, epistaxis, ulcerated mucosa, otitis media, saddle nose = late presentation Renal: Haematuria, fever, night sweat, weight loss Eyes: Episcleritis, Uveitis, Optic nerve vasculitis

36. Management • Immunosuppression • Steroids and cytotoxics (mainly cyclophosphamide) • Rituximab if refractory to conventional treatment.

37. Churg Strauss syndrome(aka allergic granulomatous angiitis) • Systemic Necrotizing Vasculitis • Autoimmune attack of small/medium vessels • via Anti-neutrophil cytoplasmic antibodies (pANCA+ 75%) • Eosinophilic infiltrative disease and Granulomatous inflammation • Rare. Unknown cause Key features/stages: • Allergies (new) • Asthma (esp mature onset) and Allergic rhinitis • Organ involvement • Sinusitis • Eosinophilic pneumonia • Mononeuritis multiplex (vasculitic neuropathy) • Gastroenteritis and necrotised intestines • Systemic small vessel vasculitis and granulomatosis, with pulmonary, cardiac, derm, renal and peripheral nerve involvement, causing sx related to those systems. Progresses to a coronary arteritis and MI.

38. Churg Strauss syndrome Management • Specialist involvement for each organ system affected, resp cardio derm etc. • Immunosuppression via Steroids • Cytotoxics, esp Cyclophosphamide or azathioprine. • Life long follow up, with monitoring of ESR and degree of eosinophilia. • 5 year survival with treatment approx 60%, with death due to MI, CRF, GI bleed, Cerebral Haemorrhage or Respiratory failure

39. Hypersensitivity pneumonitis aka EAA Extrinsic Allergic Alveolitis • Inflammation within the alveoli due to hypersensitivity to inhaled allergens, often occupational exposure. • Combination of type III (immune complex) and type IV (cell mediated) hypersensitivity • More of a syndrome, a clinical phenotype with lots of causes • Famous examples include farmer’s lung, mushroom picker’s lung or pigeon fancier’s lung

40. Hypersensitivity pneumonitis • Diffuse inflammation of lung parenchyma, characterised by poorly formed non-caseating granuloma with Langerhans cells and interstitial fibrosis. • Can progress to alveolar destruction; ‘Honeycombing’ • The condition where granuloma meets fibrosis • IgG mediated, patients have high levels. • 3 ways to present: • Acute: 4-6 hours post heavy exposure to agent, with chills, malaise, cough, SOB and headache. Resolves on cessation of exposure • Subacute: Intermittent episodes of mini version of above, with fatigue and weight loss • Chronic: Often no history of above episodes, present with insidious onset cough, SOB and weight loss. Removing agent gives only partial relief.

41. Hypersensitivity pneumonitis Management • Cease exposing yourself to pigeons, no matter how much you fancy them. No mushroom picking, even if it’s magical. • Steroids if severe

42. 5. Infections • Pneumonia • Aspergillosis

43. Pneumonia • Infection of pulmonary parenchyma • Inflammation causes alveolar spaces fill with inflammatory cells exudate. • Cause can be bacteria, virus, fungi, parasite and chemicals or trauma. • Range of conditions with different microbial pathogen spectrum, characterised by acute respiratory illness accompanied by radiological features. • CXR: diffuse/lobar consolidation with air bronchograms.

44. Pneumonia • Generally classified by the context in which it develops • Community Acquired (CAP): Includes within 48 hours admission • Hospital Acquired (HAP): Occuring >48hrs of admission • Aspiration • Pneumonia of Immunocompromise • Alternatively, by anatomic distribution of disease • Lobar • Bronchopneumonia • Interstitial

45. Spectra

46. Medically important bacteria Anaerobes shown in green

47. Pneumonia Symptoms: • Cough, SOB +/- purulent sputum. • Fever, Pleuritic chest pain, haemoptysis • Headaches, confusion, myalgia, malaise • May be vague presentation in immunocompromised or elderly. O/E: • Crackles, bronchial breathing, pleural rub • ↑ RR, Hypotension, Confusion Ix: • Bloods – FBC, CRP, U&E (Urea & CURB 65) • CXR, Sputum culture, VBG Rx: • Supportive – IV Fluids, O2, Chest physio. • Antibiotics – Local guidelines, pending culture

48. Pleural effusion • Excess fluid between the 2 pleural layers • Excessive amounts impair breathingSOB, pleuritic chest pain. • Breath sounds, vocal resonance, plural rub. • Stony dull percussion • Serous fluid (hydrothorax) • Pus (empyema) • Blood (haemothorax) • Chyle (chylothorax) CXR: Basal opacity obscuring hemidiaphragm, concave upper boarder

49. Transudate v Exudate

50. 6. Restrictive Lung Disease (RLD)(aka restrictive ventilatory defects) • Essentially effects due to reduced lung volume, TLC, VC, without changes to airway resistance. • Can be divided anatomically into: • Intrinsic. Parenchymal Disease. Inflammation and scarring of lung tissue (Interstitial lung disease), or filling of airspaces with exudate and debris (pneumonitis). Impairs gas exchange (Exercise O2) • Extrinsic. Extraparenchymal. Diseases affecting the bits that work outside the organ e.g. neuro, leading to inadequate ventilation and respiratory failure.