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Ivy Ng itn20@ic.ac.uk Chronic SOB MedED Y3 Lecture Series S l i d e s a d a p t e d f r o m M e d E d 2 0 2 3 l e c t u r e Cough Breathlessness Diaphragm Pulmonary embolus/ A systemic disorder? weakness? Psychogenic Heart disease? infarction? dyspnoea? Check the patient is not taking an ACE Inhibitor. If yes Lung disease? stop, ifnot determine length of history of cough Irregular pulse? Clinical signs of left ventricular hypertrophy or •Anaemia – check haemoglobin Is the breathlessness worseon failure? •Is amajor risk factor present? •Obesity – check BMI lying flat or in water? Does the Ndiagnosis of exclusionly a Personal or family history of ischaemic heart •Recent immobility •Overactivethyroid – check T4 vital capacity fall onlying disease? ECG abnormal? •Recent lower limb trauma/surgery compared with sittingor •Clinical DVT standing? Coughhas lasted •Previous or FH of VTE more than12 •Pregnancy or puerperium Coughhas lasted less weeks •Major medical illness If indoubt refer than 12 weeks - if no Chest radiograph shows cardiac to a respiratory sinister features (and enlargement? Treat or refer physician not a Smoker or ex- smoker) may be post Send for Refer to a respiratory physician viral Chest Radiograph Refer urgently for investigation Treat or refer to cardiologist AIRWAY DISEASE? SMALL LUNG DISEASE? Chest radiograph is Chest Radiograph is INFECTION? Pulmonary abnormal; normal embolus/ infarction? Is this an airway disorder? Is there a cough, wheeze, breathlessness or obstructive spirometry or reduced peak flow? If so is it a localisedor ageneralised obstruction? Is this a small lung disorder, (added sounds?Altered Is therea fever, a cough percussion note?) productive of purulent sputum and if so is it duetoanabnormality Within the of the chest?s on examination lung : Symptoms of Nasal stuffiness Localised obstruction? Sarcoidosis Abnormal ? Stridor Generalised obstruction? Asbestosis gastro- or catarrh or ?monophonic wheeze ?Polyphonic wheezing spirometry or oesophageal reflux other suggestion ?unilateral wheezing ? Bilateral wheezing Hypersensitivity pneumonitis personal or family Extrinsic allergic alveolitis or overweight – of post nasal drip Treat &/or refer for a Eosinophilic pneumonia history of other consider trial of disease - Idiopathic pulmonary Fibrosis atopic disease? - chest radiograph Lymphangioleiomyomatosis consider asthma proton pump consider trial of and/or a respiratory Langerhans Cell Histiocytosis inhibitor in highish intranasal Alveolarproteinosis or COPD opinion Localised obstruction : Generalised obstruction : Or dosage for 2/12 steroids Laryngeal carcinoma? Asthma? Retrosternal thyroid? Chronic obstructive pulmonary disease? Outside the lung : Relapsing polychondritis? Bronchiectasis? Pleural effusions A bronchial or tracheal tumour? Cystic fibrosis? Mesothelioma Post tracheostomy stenosis? Obliterative bronchiolitis? Scoliosis Advise smoking Inhaled foreign body? Respiratory muscle weakness cessation or trial of No response → refer respiratory Bronchopulmonary dysplasia? Obesity physician Obstructivesleep apnoea syndrome anti-asthma therapy as appropriate Treat or refer to a Refer to a respiratory Refer to an ENT or respiratory physician respiratory physician physician SYMPTOMS and CLINICAL EXAMINATION IN THE DIAGNOSIS OF LUNG DISEASET opics • Asthma • COPD • Interstitial Lung Disease(ILD) • Sarcoidosis • IPF • Occupational Lung Disease • Extrinsic allergic alveolitis • Obstructive Sleep ApnoeaCategorising Lung Disease Airways Consider other relevant systems: Alveolar - Cardiac - Anaemia (GI, Renal) - Metabolicacid-base imbalances (eg. DKA) Parenchyma Pleura Symptoms in Respiratory Problem Questions indicating respiratory problems Questions that rule in specific diagnoses Red flags to exclude Tend to be very non-specific. Wet cough The history should elicit the: Haemoptysis Dry cough • Narrative Pleuritic pain SOB – at rest vs exertional dyspnoea • Characteristics DVT Sx Chest pain – quality • Timing Cardiac chest pain Pleuritis • Triggers (radiations) “Sounds” stridor, sturtor, wheeze, etc • Exacerbators and alleviators Vomiting/pancreatitis Haemoptysis • Risk factors for conditionsyou have in Thoracic back pain Tiredness mind Travel Fever Lethargy Appetite loss This can form the general respiratory focussed history or systems Weight loss Sweats at night review structure. SBA 1 A 43 year-old woman presents with SOB, dry cough worse at night which started during an URTI 6 weeks ago. Her symptoms come and go and are worse when examination polyphonic expiratory wheeze is heard bilaterallyhaemoptysis. On Given the likely diagnosis what lung function results best support diagnosis A)FEV1/FVC <0.7 B)FEV1/FVC >0.7 C)FEV1/FVC <0.7 with Bronchodilator reversibility D)FEV1/FVC >0.7 with Bronchodilator reversibility E)FEV1/FVC <0.7 with reduced DLCO SBA 1 A 43 year-old woman presents with a 2-month history of SOB, dry cough worse at night which started after an URTI 2 months ago. Her symptoms come and go and are worse when she walks in the cold air to work. She has no weight loss or haemoptysis. On examination polyphonic expiratory wheeze is heard bilaterally Given the likely diagnosis what lung function results best support diagnosis A)FEV1/FVC <0.7 Learning point: asthma lung function B)FEV1/FVC >0.7 tests will show reversible obstructive C)FEV1/FVC <0.7 with BDR lung disease D)FEV1/FVC >0.7 with BDR Therefore, FEV1/FVC <0.7 and BDR E)FEV1/FVC <0.7 with reduced DLCOAetiology of Asthma Epithelial airway damage Reversible airway Inflammation obstruction largely mast cell, Vascular smooth muscle WITH Intact lung parenchyma IgE and hypertrophy Allergens eosinophil mediated (Type 1 Airway hyperresponsiveness Airway hyper- Hypersensitivity) responsiveness Mucus plugging (normally in exacerbations) exacerbations Page C. Pathogenesis of COPD and Asthma2016 Basics of Asthma History Asthmais aVERY COMMON chronic airway inflammationthat causes variable airway obstruction Shortness of breath Dry cough Wheeze (tight chest) HISTORY Rest of the HxestablishRF You should establish the following • FHx of asthma • Must be variability in symptoms!!!! “Are these problems always there” • FHx orpersonal history of atopy • Diurnal“Do you wake up at night breathless ofcoughing • Food allergy • Episodic history: Intermittent dyspnoea (shortness ofbreath), coughing • Hay fever (worse at morning and night) • Eczema • Are there triggers and if so what are they? • GORD can make asthma worse • House dust mite “are there carpets in yourbedroom, are you often in dusty environments?” Signs: • Pets:there are NO hypoallergenic pets • Expiratory polyphonic wheeze • Smoking “Do you or anyone at home smoke?” • Use of accessory muscles • Exercise:SOB isusually after exercise • Previous asthma care -> previousattacks? hospital? IV? Intubation? • Hyperinflated chest https://www.nice.org.uk/guidance/ng245/chapter/Recommendations#initial-clinical-assessment ASTHMA - Investigations ASTHMA - Investigations • Can we prove that there is a variable airway obstruction and airway inflammation? Investigation Suggestive of asthma Basic obs/sats Should be normal unless asthma attack Fractional Exhaled Nitric oxide ≥40ppm FEV 1 reduced FEV1decreases with less change in FVC lower than normal Spirometry FEV1/FVC <0.7 Bronchodilator reversibility >12% and 200ml increase in FEV 1ost SABA Peak flow diary (twice daily for2 weeks) >20% variability over 2-4 weeks 2 or more of the tests being positive is diagnostic https://www.nice.org.uk/guidance/ng245/chapter/Recommendations#initial-clinical-assessment ASTHMA - Investigations https://www.nice.org.uk/guidance/ng245/chapter/Recommendations#initial-clinical-assessment ASTHMA - Management MART (MaintenanceAnd Reliver Therapy)/Spacers One inhaler: Combination ICS with a LABA that has short and long acting affects Spacers: improve lung deposition of drug LABA to feel good through bronchodilation! ICS to address inflammation! (short and long acting LABA) Reliever: take when they get symptoms Maintenance: take the inhaler daily LABA helps improve ICS suppresses LABA has short acting airway obstruction inflammation! function that relieves and can make people Sx. ICS to suppress feel good. This may ICS will be taken Avoids people using when asthma is SABA for symptoms inflammation! help with adherence especially if they’re worse because without ICS for the symptomatic looking people want to take inflammation for relief the LABA for relief causing the problemACUTE Asthma Exacerbation In primary care - Oxygen - Salbutamol by spacer one puff every 60s, max 10 puff - Oral prednisolone (min 5 days or until recovery) In hospital (Severe/Life-threateningasthma) High flow Oxygen (Aim for 94-98% satyration) Nebulised salbutamol 5mg Nebulisied ipratropium bromide 0.5mg Oral prednisolone 50mg/ IV hydrocortisone 100mgACUTE Asthma Exacerbation SBA 2 A 45-year-old non-smoking man presents with a 9-month history of episodic wheeze, chest tightness, and nocturnal cough. He works in acar manufacturing plant and reportsthat his symptoms tend to worsen toward the end of his work week and improve after weekends or time off. His past medical history is unremarkable. Examination is normal. Initial spirometry reveals an FEV₁/FVC ratio of 0.72,with a16% improvement in FEV₁after bronchodilator use. Which of the followingis the most appropriate next step in confirmingthe diagnosis? A)High resolution CT chest B) Total IgE C) Serial peak expiratory flow monitoring with occupational exposure correlation D)Induced sputum eosinophil count E) FeNO SBA 2 A 45-year-old non-smoking man presents with a 9-month history of episodic wheeze, chest tightness, and nocturnal cough. He works in acar manufacturing plant and reportsthat his symptOccupational asthma toward the end of his work week and improve after weekends or time off. His past medical history is unremarkable. Examination is normal. • Most common industrial lung disease in the developed world Initial spirometry reveals an FEV₁/FVC ratio of 0.72,with a16% improvement in FEV₁af er bronchodilator use. Suspect In people with adult-onset asthma, poorly controlled established asthma, or Which of the followingis the most appropriate next step in confirmingthe diagnosis?ce of childhood asthma A)High resolution CT chest - Are symptoms the same, better or worse on B) T otal IgE days away from work? - Are symptoms the same, better or worse C) Serial peak expiratory flow monitoring with occupational exposure cwhen on holiday (time away from work, D)Induced sputum eosinophil count longer than usual breaks, at weekends or between shifts)? E) FeNOAsthma: Summary slide Aetiology: History Investigations: ✶ Establish variable obstructive symptoms Airway inflammation and Spirometry: hypersensitivity.Often with ✶ SOB - FEV1/FVC <0.7 eosinophilic inflammation ✶ Dry cough (obstructive lung disease) ✶ Wheeze -Bronchodilator ✶ often diurnal and the patient is usually completely well chestwise reversibility (BDR) >12% and Risk factors between episodes if they have good asthma control 200ml increase in FEV1 after salbutamol ✶ FHx asthma ✶ Establish triggers and risk factors ✶ Personal or FHx of ✶ Viral infections, cold air, exercise, allergic exposure, smoking -FeNO (measure of eosinophilic inflammation) ≥40ppm atopy, food allergy ✶ Establish current treatment(+technique and adherence) -Peak flow diary with >20% or hayfever ✶ Establish frequency, recency and severity ofany previous asthma attacksvariability over 2-4 weeks Differentials (DW too muchabout thses) Management: Complications: ✶ Viral URTI ✶ Conservative: Stopsmokingandavoidtriggers ✶ COPD (baseline ✶ Asthma attacks symptoms always ✶ Anti-inflammatoryreliever (AIR) therapy there) ✶ LowdoseMART ✶ Death ✶ Chronic sinusitis and ✶ ModeratedoseMART ✶ Oral candidiasis after post-nasal drip (will ✶ + LTRA/ LAMA 8-12 week trial have runny nose and oral steroid use (rinse secretions with worse symptoms when flat) mouth after steroid ✶ Tracheomalacia – inhalers) Positional symptoms ✶ vocal cord dysfunction SBA 3 A 67-year-old man presents to the GPwith increasingbreathlessness on exertion and achronic productive prolonged expiration.His oxygen saturation is 94% on room air. Spirometry shows an FEV₁/FVC ratio of 0.64 and a post-bronchodilator FEV₁of 65% predicted. He has had two exacerbations in the past year but has never been hospitalised. Which of the followingis the most appropriate next step in management? A. Short-acting beta-agonist (SABA) only B. Long-acting beta-agonist (LABA) C. LABA+ inhaled corticosteroid (ICS) D. Long-acting muscarinic antagonist (LAMA) + LABA E. Oral corticosteroids SBA 3 A 67-year-old man presents to the GPwith increasingbreathlessness on exertion and achronic productive cough.He has a45-pack-year smoking history.On examination, he has decreased breath sounds and prolonged expiration.His oxygen saturation is 94% on room air. Spirometry shows an FEV₁/FVC ratio of 0.64 and a post-bronchodilator FEV₁of 65% predicted. He has had two exacerbations in the past year but has never been hospitalised. Which of the followingis the most appropriate next step in management? A. Short-acting beta-agonist (SABA) only B. Long-acting beta-agonist (LABA) C. LABA+ inhaled corticosteroid (ICS) 2 moderate episode of COPD exacerbation, D. Long-acting muscarinic antagonist (LAMA) + LABA withoutadmission E. Oral corticosteroids No-asthmatic features → LAMA + LABA *IF there were asthmatic features → LABA + ICS What is COPD (hugely simplified) related (pun intended)oosely o Chronic bronchitis: Small airway obstruction continuous cough + → hyperinflation sputum production for at Irreversible least 3 months per year over 2 yrs airway Emphysema obstruction o Emphysema: Permanent Page C. Pathogenesis of COPD and Asthma2016 enlarged air spaces distal with reduced to the terminal Reduced recoil gas exchange bronchioles, with and less air destruction of alveolar pressure upon wall expiration when More goblet cells -> more elastin is broken mucus down What puts you at risk for COPD Disease caused by declining lung function •Lung function declines with increasing age Accelerated by inflammatory damage to the lungs • SMOKING • lack of anti-inflammatoriesto prevent inflammation - a1- antitrypsin deficiency) • Respiratory infections/chronic bronchitis? • susceptibility to inflammation (FHx), environmentalexposures COPD History Sounds like COPD Qs to ask to exclude asthma Consistent Chronic Cough symptoms Patient +/- sputum older than No night Dyspnoea - productive in only 35 years time - Persistent 30% of patients!! waking - mucus is - Progressive produced but not Qs to ask to consider heart failure - Exercise induced always coughed Pink frothy Leg up sputum swelling Cardiac history Smoking history Qs to ask to consider malignancy failure Wheeze Previous exacerbations Weight loss fatigue Metastatic symptoms haemoptysis COPD examination • Peripheral: • Increased respiratory effort: tripod, flared nostrils, accessory muscles • Hypercapnia (CO2 retention): Flap, bounding pulse • Chest: • Wheeze • Tachypnoea with prolongued expiration • Barrel chest → loss of percussion dullness over heart and liver • Cor pulmonale: Right ventricular heave, JVP elevated and ankle oedema • Hoover’s sign GrepMed COPD Diagnosis Suspect a diagnosis of COPD in people over 35 who have a risk factor (generally smoking or a history of smoking) and who present with 1 or more of the following symptoms: • exertional breathlessness • chronic cough • regular sputum production • frequent winter 'bronchitis' • wheeze Investigations Spirometry: post-bronchodilator FEV /FVC <1.7 with no BDR Basic obs: low saturations CXR: hyper-expansion, air trapping FBC: exclude anaemia Serum alpha-1 antitrypsin - if early onset, minimal smoking history or family history Stage FEV1 % MRC Dyspnoea scale Quantify symptom burden predicted (Grade 1-5) and impact on quality of I – mild >80 life II – moderate 50-79 - Score ≥2 suggest high • (CAT) COPD symptom burden Assessment Test III – severe 30-49 • scores≥10 suggest IV – very severe <30 or<50+ pharmacologicalTx resp failure SBA 4 What is thefirst step of pharmacological COPD management? a) SABA b) LABA c) LAMA d) LTRA e) ICS SBA 4 What is thefirst step of pharmacological COPD management? a) SABA Learning point: b) LABA COPD -> smoking cessation c) LAMA d) LTRA SABA/SAMA first line e) ICS Further treatment depends on whether there are asthmatic featuresCOPD Management Educate Reduce risk factors: • Smoking cessation • Pneumococcal and influen za vaccines • Consider pulmonary rehab (aerobic ex/ strength training) If breathless and has exercise limitation --> SABA or SAMA to use as neededCOPD management Individualised Long term oxygen therapy exacerbation plan Refer toa specialist • Identify at risk patients 2 ABGs3 weeksapartin non-smoking stable patientsonoptimal • Previous exacerbationinlastyear pharmacological treatment • Educate and make sure patients are Giveshort courseof oral OCS and antibiotics for aexacerbations them Consider LTOT ifpatient has • PaO2<7.3when stable • Ensure patientsare aware totell HCP when they have hadto use rescue packs Or • PaO 2etween7.3 and 8kPa whenstablewith ≥1of • Prescribe short course oral antibiotics and • Secondarypolycythaemia oral corticosteroids to take at home when • Peripheral oedema they have anexacerbation • PulmonaryhypertensionCOPD: Summary slide Aetiology: History Differentials (DW too muchabout thses) ✶ Establish irreversible chronic obstructive symptoms Complicated but in brief ✶ SOB pulmonary inflammation with ✶ Cough (wetordry; only 1/3 have a wet cough) ✶ Left Heart Failure smoking being a major cause ✶ Wheeze ✶ Ashthma and driver that causes ✶ Often exercise induced ✶ Bronchitis irreversible obstructive air✶ Progressive disease and alveolardamage. ✶ Establish risk factors: smoking, FHx especially if early onset ✶ Ask RHF signs and symptoms as COP can cause RHF Risk factors ✶ COPD patients are often the same demographics as those with HF and LHF can mimic COPD. Exclude LHF signs and symptoms ✶ Smoking ✶ Increasingage ✶ Examine for respiratory effort,T2 resp failure flap, reducedcricosternal distance and hyperexpansion. Investigations: Management: Complications: CATorMRC questionnaire ✶ Conservative: Stopsmoking, educate, create an individualised ✶ Exacerbations Spirometry: - FEV1/FVC <0.7 exacerbation plan andoptimize vaccination status ✶ Death (obstructive lung disease) ✶ 1 line: SABA/ SAMA ✶ Oral candidiasis after - No bronchodilator ✶ 2 ndline: LAMA + LABA(if noasthmatic features) reversibility LABA + ICS(if asthmatic features) oral steroid use (rinse rd mouth after steroid CXR ✶ 3 line: LABA+ LAMA + ICS inhalers) -hyper-expansion -air trapping SBA 5 Mr Carter Lage is a 78 year old man is referred to resp medics with chronic SOB, progressive exertional dyspnoea and a dry cough with no improvement after repeated treatment for Heart Failure. He is saturating on room air, has stage 2 clubbing, bibasal inspiratory crepitations. Imaging studies show bibasal reticular fibrosis What’s your top differential? A)Asthma induced degranulating emphysema B) Bronchiectasis C) COPD D) IPF E) Langerhans cell histiocysotis SBA 5 Mr Carter Lage is a 78 year old man is referred to resp medics with chronic SOB, progressive exertional dyspnoea and a dry cough with no improvement after repeated treatment for Heart Failure. He is saturating on room air, has stage 2 clubbing, bibasal inspiratory crepitations. Imaging studies show bibasal reticular fibrosis What’s your top differential? A)Asthma induced degranulating emphysemaThis one is made up B) Bronchiectasis C) COPD D) IPF E) Langerhans cell histiocysotis Interstitial lung disease • Broad term to describe any disease that affects the interstitium/parenchyma • There are, literally, 100s. Hundreds. Multos. Abundances. So many. ATS/ERS classification, 2001. ILD general cheat sheet Umbrella term to describe fibrosis of the lung parenchyma which isusually categorised by cause and radiology Pathology: Key causes: History: • Dry cough with shortness of breath Damage to the lungparenchyma, formation of • Idiopathic Pulmonary Fibrosis • Gradual onset scar tissue within alveoli and interstitium. • Systemic diseases: Rheumatoid • NO wheeze Causingimpaired gas exchange and restrictive arthritis, SLE, Scleroderma, • Ask about symptoms of lung disease. Sarcoidosis autoimmune disease, occupations, medication and unusual hobbies • Medications: Amiodarone, • Weight loss Nitrofurantoin, Methotrexate-ish Investigations: Exam: • Observations: hypoxic, tachypnoeic • Fine end inspiratory crackles (bibasal) • CXR: may appear normal in early disease, may see ground glass shadowing • Reduced lung expansion • High resolution CT thorax: fibrosis (honeycombing) • Clubbing • Lungfunction tests: restrictive and reduced DLCO Management: • MDT • Pulmonary rehab, manage any underlying causes, steroids/antifibrotics Idiopathic pulmonary fibrosis Symptoms Idiopathicpulmonary fibrosis = lung • Progressive SOB scarring of an unknown cause. • Exertional dyspnoea Signs • Dry cough • may have weight loss, fatigue and • Clubbing (notsuper common) malaise • Bi-basal fine end inspiratory • Slow insidious onset crepitations • Your patient may well have previous suspected COPD etc and usually a delayed diagnosis (often years late) Classic SBA • Old man with progressive dyspnoea and cough (often dry) with clubbing and bibasal crepitations IPF investigations What is the gold standard investigation for IPF? • MDT Approach • Rule out common mimics (COPD/pneumonias etc) • CXR – often mucky and non-specific by the time of presentation but can be clear • Spirometry and gas transfer: restrictive FEV1:FVC >0.7 with reduced gastransfer GOLD STANDARD – High resolution CT • IPF pattern of fibrosis (bi-basal sub-pleural fibrosis) • Rule out any other cause of pulmonary fibrosis • Rule out all other ILDs (exposure history, ANA panel, Rheumatoid factor, anti cyclic-citrullinated peptide and myositis panel for collagen vascular disease) • BAL +/- Lungbiopsy if MDT aren’t sure IPF Management Smoking cessation!!!! Lung cancer risk increased with IPF and IPF & smokingincreases risk up to 100 fold Continue monitoringand assess for Treatment How bad are they and how fast are they declining? Baseline, 6 month and 12month: Lung function (spirometry and gas transfer) 6 minute walking test, SO2 and Quality of Life Specialist care • Pulmonary rehabilitation • Supportive care – ambulatory or long term oxygen • Antifibrotics: Pirfenidone or Nintedanib • Lungtransplant in select patients Idiopathic Pulmonary Fibrosis: Summary slide Aetiology: RF: History: Examination: Differentials: ✶ Idiopathic… ✶ Increased ✶ ANY FIBROTICLUNG ✶ Path involves age ✶ Progressive dyspnoea ✶ Bilateral bibasal DISEASEWITHA dysregulated ✶ Male ✶ Dry cough end inspiratory KNOWN CAUSE (cause inflammatory ✶ Smoking crepitations thenits not idiopathic) pathways ✶ Clubbing ✶ Weight loss ✶ Investigations will exclude ✶ Malaise/fatigue these (radiography fibrotic patternis alsodistinctive too) Investigations: Management: HRCT is gold standard: usual ✶ Pulmonary rehab interstitial pneumonia pattern (bibasal ✶ Home oxygen subpleural fibrosis with honeycombing) ✶ Antifibrotics if <50 % FVC <80 % predicted • Blood panels to exclude other causes Prognosis and complications of lung fibrosis • Connective tissue disease markers • ANAAb ✶ Pulmonary hypertension ✶ Terminal • Rheumatoid factor ✶ 2-3 year median survival • Anti-cyclic citrullinated peptide • Myositis panels SBA 6 A 32-year-old African-Caribbean woman presents to her GP with a 3-month history of dry cough, On examination, there are no abnormal chest sounds, but there is mild bilateral parotid glandker. swelling and some tender red nodules over her shins. A chest X-ray reveals bilateral hilar lymphadenopathy. Which of the following is the most likely diagnosis? A. Tuberculosis B. Sarcoidosis C. Hodgkin lymphoma D. Systemic lupus erythematosus (SLE) E. Sjögren’s syndrome SBA 6 A 32-year-old African-Caribbean woman presents to her GP with a 3-month history of dry cough, fatigue, and occasional shortness of breath. She has no past medical history and is a non-smoker. On examination, there are no abnormal chest sounds, but there is mild bilateral parotid gland swelling and some tender red nodules over her shins. A chest X-ray reveals bilateral hilar lymphadenopathy. Which of the following is the most likely diagnosis? Learning point: A. Tuberculosis B. Sarcoidosis While sarcoidosismost commonly affects the C. Hodgkin lymphoma lungs it has extrapulmonary complications D. Systemic lupus erythematosus (SLE) which strongly support diagnosis and affect E. Sjögren’s syndrome patients QoL dramatically Sarcoidosis Chronic granulomatous disorder of unknown aetiology, affecting the lungs, skin and eyes Charactersied by accumulation of lymphocytes and macrophages and formation of non-caseating granulomas in the lungs This happens most commonly in youngwomen of Afro- Caribbean ethnicity(20-40 years old) If it’s in an SBA it will be likely be “afro Caribbean woman with a dry cough and skin patch on her shin” May include things like: - CXR bilateral lymphadenopathy - Lupus pernio (cheek, tip of nose and lobe of ear rash) - Erythema nodosum - High calcium or serum ACE Key manifestations of sarcoidosis Pulmonary (occurs in 90% macrophagesroxylase in Eyes of cases) • Anteriorand posterior Uveitis • Persistentdrycough • Bilateral lymphadenopathy • Red painfuleyewith • Lung fibrosis may occur following photophobia suggestsanterior inflammation around granulomas. Non-caseating uveitis. EMERGENCYwhich Often middle/upper zone granulomas can causeblindness. Loefgrens’s (30%) Heerfordt • Bilateral hilar • Rarer lymphadenopathy Skin • Fever • Erythemanodosum Parotids • Uveitis And/or • Parotiditis • Parotiditis • Bilateral ankle pain Erythema Nodosum • Can causefacial • Facial nerve nerve palsy palsy 75% resolve • Lupus Pernio: pathognomonic red Fevers blue lesionson cheeks, ears and tip of nose. Arthralgia • Migrating polyarthritis • Often ankles butcan bewrists/knees Cardiacissues(significant cause of death)Key manifestations of sarcoidosis 1-alpha hydroxylase in macrophages Pulmonary manifestations of Sarcoidosis Stage Stage 1 Bilateral hilar lymphadenopathy Stage 2 Bilateral hilar lymphadenopathy + pulmonary infiltrates Sarcoidosis Investigations: everything…. • No single diagnostic test, big picture interpretation of some key serum,imaging and biopsy results investigating for granulomas and organ improvement • Remember to go least invasive to more invasive and focus on the system causing symptoms in the question Systemic Bloods: FBC, blood film, CRP, TB testing: investigateinfection or leukaemias/lymphomas Bone profile: High serum calcium High Serum ACE Organ specific Lungs • CXR Kidneys: Urea and creatinine may be high • Bihilar lymphadenopathy (differentials areTB,sarcoidosisLiver: AST may be high • Upper lobeinfiltrates • findings do not correlate with symptoms Skin: skin biopsy • HRCT: ground glass -> reversible. Cystic -> irreversible • Bronchoalveolar lavagetive but can show obstruction Heart: ECG +/- ECHO • LungbiopsyManagement of sarcoid Conservative: -Educate and counsel -Refer to respiratory/specialist ILD clinic/sarcoid -Most acute disease resolves on its own (90% stage 1 lung disease and 50% stage 2) Medical: Erythema Nodosum can be managed with NSAIDs or short course steroids If there is • severe organ dysfunction (pulmonary fibrosis, worsening spirometry, pulmonary HTN etc, hypercalcaemia) • threat to life • unacceptable impact of QoL The major treatment is long term, low dose corticosteroids (specialist) Further immunosuppression escalation is very specialist with limited evidence base Sarcoidosis emergencies that require IV steroids Acute optic neuritis Cardiac disease Neurological DiseaseSarcoidosis Aetiology: Examination: Investigations: Resp: wheeze or rhonci Not clearly known but Bedside: may be immune Pyrexia Resp/cardio exam, Obs, ECG response to a residual Erythema nodosum/lupus pernio antigens from Cardio: may be arrhythmias or HF infection Differentials: Bloods: FBC, CRP, U&Es, LFT, ?ACE Young woman TB Asthma Imaging: CXR, Afro-Caribbean Lymphoma COPD Specialist: LFT, BAL History: Management: Complications: ✶ Persistent dry ✶ Pulmonary fibrosis cough and SOB ✶ Conservative– educate, refer to specialist clinic Variable others but from head ✶ Anterior uveitis to toe: Eye problems especially acutecal: watery, red eye +/- vision ✶ Oral steroids if severe organ disease or low QoL ✶ Cardiac arrhythmias or Parotid enlargement +/- facialIV steroids if: anterior uveitis, cardiac or diastolic heart failure nerve palsy neurological involvement Heart problems ✶ Erythema nodosum – NSAID or short course Erythema nodosum Joint problems especially steroids ankles SBA 7 A 58-year-old retired construction worker presentswith progressive shortness of breath and adry cough.He bibasal inspiratory crackles and finger clubbing. Achest X-ray shows diffuse interstitial markings. High-e resolution CT shows subpleural reticulation and pleural plaques. Which of the followingis the most likely diagnosis? A. Idiopathic pulmonary fibrosis B. Asbestosis C. Silicosis D. Mesothelioma E. Occupational asthma SBA 7 A 58-year-old retired construction worker presents with progressive shortness of breath and a dry cough. He reports no smoking history.He worked with insulation materials for over 30 years. Examination reveals fine bibasal inspiratory crackles and finger clubbing. A chest X-ray shows diffuse interstitial markings. High- resolution CT showssubpleural reticulation and pleural plaques. Which of the followingis the most likely diagnosis? Learning point: A. Idiopathic pulmonary fibrosis Asbestos can cause mesothelioma – which will cause pleural B. Asbestosis thickening/ effusion! C. Silicosis D. Mesothelioma Jobs in ship yards, power stations, working with insulation E. Occupational asthma and roofing are risk factors. For mesothelioma only light exposure isrequired and disease occurs20-40 years later. Occupational lung disease Jobs with inhaled particulates that cause fibrosis silica Asbestos coal ILDs can take decades to develop (10-20 years later)! Simple pneumoconiosis • Coal miners • Coal deposits in the lungs • Exertional dyspnoea and cough Progressive Massive Fibrosis • Considerable exertional dyspnoea + Silicosis Cough +/- black sputum Asbestos related lung disease • CXR: Large round fibrotic masses in Sx: Cough, SOB upper lobes Occupations: Stonemason, including asbestosis and pottery, ceramics mesothelioma • Mixed picture in spirometry Imaging: Upper lobe round fibrotic masses often with hilar “egg shell”calcificationsAsbestosis RF: • Working closely with asbestos (shipyard, mining and aerospace) • Bystander working near asbestos(electricians, painters and masons) • Background exposure (most common) PC: Signs: Progressive dyspnoea (often exertional) Clubbing Dry cough Reduced expansion Asbestos warts Pleuritic chest pain Bibasal crackles FLAWS + haemoptysis -> malignancy RHF Asbestosis Asbestos bodies Presence of asbestosis in the lungs with no symptoms or CXR findingsafter light exposure to asbestos Pleural plaques Thickening and calcification of the pleura visible on CXR (seen early and that causes slight restrictive lung disease and rarely at low exposure) causes occasional exertional dyspnoea. Can be diffuse. Asbestos exposure Generalised lungfibrosis 5-10 yearsafter heavy exposure that causes severe restrictive lungdisease, reduced gas Asbestosis transfer in alveoli, diffuse lung fibrosis on CXR and progressive dyspnoea Cancer Mesothelioma (pleural effusion, chest pain, SOB) Decades after Usually occurs 20-40 years after exposure which can be exposure. Does light not need Asbestos related lung cancer asbestosis to be Increased cumulative exposure = increased risk of cancer present and cancersdevelop >10 yearsafter first exposure Adapted fromKumarand Clarke. Xrays fromatsdr.cdc (see comments) Asbestos, Investigations Investigation Result Hx ofexposure, timing of exposure and exclusion ofother conditions are important CXR May show pleural plaques, effusions, bilateral lower lobe fibrosis (if there is asbestosis) or masses (cancer) Lungfunction Restrictive: Low FEV1, Low FVC, Low Residual volume Normal or high FEV1/FVC Alveolardamage: Reduced DLCO ABG if acute May show hypoxia (normally T1 Resp failure) HRCT Basal diffuse reticulonodular infiltrates Shaggy heart border Ground glass and diffuse interstitial fibrosis LungBiopsy For malignancies No specific management Compensation is available if they apply within 3 years ofdiagnosis Corticosteroids Support groups CXR Investigations Silicosis Coal’sworker – small round nodular Enlarged hilar lymph nodes with opacities (less well-defined margins) “eggshell’calcification SBA 8 A 45-year-old man presents with progressive breathlessness,dry cough, and fatigue over several months.He works as a farmer and reportsworsening symptoms after cleaning out mouldy hay. Examination reveals fine inspiratory crackles at the lungbases.Spirometry shows a restrictive pattern. High-resolution CT (HRCT) shows ground-glass opacities and centrilobular nodules in the upper lobes. Which of the followingis the most likely diagnosis? A. Hypersensitivity pneumonitis B. Chronic obstructive pulmonary disease (COPD) C. Sarcoidosis D. Idiopathic pulmonary fibrosis (IPF) E. Asthma SBA 8 A 45-year-old man presents with progressive breathlessness,dry cough, and fatigue over several months.He works as a farmer and reports worseningsymptoms after cleaningout mouldy hay. Examination reveals fine inspiratory crackles at the lungbases.Spirometry shows a restrictive pattern. High-resolution CT (HRCT) shows ground-glass opacities and centrilobular nodules in the upper lobes. Which of the followingis the most likely diagnosis? Classic features of hypersensitivity pneumonitis: A. Hypersensitivity pneumonitis B. Chronic obstructive pulmonary disease (COPD) - Exposure to organic antigens (e.g. mouldy hay C. Sarcoidosis → “farmer’s lung”) D. Idiopathic pulmonary fibrosis (IPF) - Restrictive lung pattern E. Asthma - Upper lobe predominant HRCT findings: centrilobular nodules and ground-glass opacities - Symptoms often subacute orchronic in presentationHypersensitivity Pneumonitis (extrinsic allergic alveolitis) History: Aetiology: Inhaled microscopic allergens that ✶ Exposure to ✶ Acute deposit in small airways and alevoli. ✶ 4-6 hours after exposure allergen ✶ Fever/flu like symptoms This leads to allergic response in ✶ Resolves within 48 hours small airways and alveoli ✶ Dyspnoea ✶ Subacute ✶ Symptoms after lower dose exposure with ✶ Farmer’s lung: mouldy hay orvegetablesough +/- ✶ Bird fancier’s lung: pigeons esp. their poom slow recovery over weeks/months ✶ Maltworker’s lung ✶ Malaise ✶ Chronic ✶ Cheese washer’s ✶ Insidious onset + weightloss +/- clubbing and is progressive with fibrosis which can be fatal Examination: Investigations: Differential Management: ✶ (nothing distinct) ✶ Nothing pathopneumonic Asthma triggered Avoid the allergen ✶ FBC/CRP/ESR for by the antigen ✶ Bilateral diffuse or infection/inflammation basal end ✶ CXR: patchynodular infiltrates in Steroids if symptoms inspiratory creps acuteor subacute. Fibrosis in chronic persist after allergen ✶ May be inspiratory ✶ HRCT: ground glass removal wheezes ✶ Antibody testing: not ideal as many people haveAbtooffending allergens ✶ clubbing without disease Farmer’s lung sufferers ✶ Lungfunction@ non-specific are eligible to restrictive with lowDLCO compensation SBA 9 A 52-year-old man presents to the GPwith excessive daytime sleepiness,morning headaches,and poor concentration. His wife reportsthat he snores loudly and occasionally stops breathing during sleep. He has a BMI of 36kg/m² and a neck circumference of 44 cm.Blood pressure is 150/95mmHg. Which of the followingis the most appropriate initial investigation to confirm the diagnosis? A. Full overnight polysomnography B. CT scan of the neck C. Overnight pulse oximetry D. Spirometry with bronchodilator response E. Epworth Sleepiness Scale SBA 9 A 52-year-old man presents to the GPwith excessive daytime sleepiness,morning headaches,and poor concentration. His wife reportsthat he snores loudly and occasionally stops breathing during sleep. He has a BMI of 36kg/m² and a neck circumference of 44 cm.Blood pressure is 150/95mmHg. Which of the followingis the most appropriate initial investigation to confirm the diagnosis? A. Full overnight polysomnography C. Overnight pulse oximetry GOLD standard diagnosis for OSA: D. Spirometry with bronchodilator response overnight polysomnography E. Epworth Sleepiness Scale Assess multiple physiological variables during sleep, including resp effort, airflow, O2 sats, EEG, EMG, Eye movement Obstructive Sleep Apnoea • Apnoea is complete loss of inspiration for at least 10 seconds • Hypopnoea is reduced inspiratory air flow (>30% for at least 10 seconds) • These are caused by complete or partial obstruction of the airway due to reduced muscle tone during sleep. Epi:fairly common! 3-5%. “classic” patient is middle agedoverweight man RF: Obesity, Cushing’s, Acromegaly, smoking, alcohol, macroglossia PC/HPC • Daytime sleepiness • Loud snoring! • Snoring→ Silence → Snoringoften grunting awake • Restless sleep • They wake hundredsoftimes but can be completelyunaware of repeated wake-ups Sleep Apnoea Clinical presentation Assess risk of sleep apnoea with STOP-BANG score Snoring BMI >35 Tired Age >35 Observed apnoea Neck circumference >40cm Pressure (BP) Gender - male Scores > 3 should be referred for polysomnography Night time in-laboratory Polysomnography Main outcome: Apnoea Hypopnoea Index which is the average number of obstructive events per hour. Mild OSA Moderate OSA Severe OSA 5 ≤ AHI ≤14 15 ≤ AHI ≤30 AHI > 30Management Mild OSA Moderate OSA Severe OSA 5 ≤ AHI ≤14 15 ≤ AHI ≤30 AHI > 30 Addressrisk factors Weight loss, smoking cessation, alcohol reduction and sleep on side ifpossible Asymptomatic Symptomatic Intra-oral CPAP mandibular If Tx failure orpatient Symptomatic sleep apnoea is advancement preference treated with CPAP at night device There are some surgical treatments but it depends a lot on what site or sites in the airway are causing apnoea Sleep apnoea is common, dangerous and worsens cardiovascular disease and is very important to identify and treat!!!OSA: Summary slide Aetiology: Examination: Investigations: ✶ Macroglossia Collapse of the upper ✶ Large neck Night-time in laboratory polysomnography airways • >5 apnoeic episodes per hour ✶ CVD • Mild OSA = 5 ≤ AHI ≤14 ✶ Obesity complications ✶ Acromegaly Differentials: • Moderate = 15 ≤ AHI ≤30 ✶ Commonlyaffects • Severe = >30AHI middle aged, ✶ Central sleep overweight men apnoea ✶ narcolepsy History: Management: Complications: ✶ Restless sleep ✶ Conservative: Lose weight, stop smoking and alcohol ✶ CVD Snoring -> silence a✶ Medical: ✶ Road traffic accident apnoea -> snoring ✶ If asymptomatic and mild intra-oral possibly with waking ✶ Impaired glucose mandibular device ✶ Sleepiness ✶ If mild symptomatic, moderate or severe tolerance during the day CPAP ✶ Depression ✶ TirednessFeedback