10/25/19

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Intelligent FinalsRespiratory

Retesh Bajaj MRCP (Lond.)Interventional Cardiology SpR

@medicineinasuit

Case NumberLung Fibrosis 24

Bronchiectasis 14Old Lung Ca 11

Unilateral Pleural Effusion 10

Old TB 8COPD & Bronchiectasis 7

COPD 7Deviated Trachea 2

Asthma 2Sarcoidosis 2

Lobectomy/pneumonectomy scar 1Kyphosis 1

Apical lung fibrosis 1Upper Lobe Collapse 1

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Respiratory Presentations -INSPECT

• COPD – BIG CHEST, breathless, leaning forward, accessory muscles…. WET COUGH! Obstructive

• Bronchiectasis – CLUBBED, lots of sputum, cachexia. Obstructive

• Fibrosis – cachexia, cyanosis, oxygen, steroid signs… ? Rheumatic hands/sclerodactaly. Restrictive

In summary:

• Evidence of complications (almost everything leads to pulmonary HTN – MEMORISE the phrase ‘pulmonary component of the second heart sound’)

• Evidence of treatment – oxygen therapy!

• Evidence of associations/causes. Even saying: the cause is not evident, but common causes include xxx

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Ending the Presentation

• “To complete my exam”

• Peak flow/bedside spirometry, sputum, temperature

• Don’t leap to CXR unless investigating

Universally Relevant Respiratory Negatives

• Breathless at rest

• Clubbing

• Tar staining

• LNs• …Pulmonary HTN/Right heart failure – raised JVP, palpable, loud P2, TR, RV

heave

• Rheumatological disease (HANDS and FACE – RA and scleroderma), steroid therapy – more for fibrotic lung disease

Chest wall scars/signs

• Supra-clavicular fossa – LN biopsy

• Singular scars anywhere – thoracoscopy

• Scar over the suprasternal notch – mediastinoscopy

• Thoracotomy – from axillary line stretching to the back – lobectomy or pneumonectomy… rarely single lung transplant – these are normally really faded if old. LOOK carefully

• Note any kyphoscoliosis, pectus changes

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Restrictive vs Obstructive Defects

• Restrictive have a reduction of FEV1 and FVC in proportion –therefore the ratio is preserved (>70%)

• Obstructive have a greater deficit in FEV1

COPD - Signs

• Pursed lip breathing

• Plethoric – secondary polycythaemia

• CO2 retention from T2RF – bounding pulse, flapping tremor (on oxygen?)

COPD - Signs

• TAR staining, Hyperinflation? Cricoid-Sternal distance decreased less than 3 finger breadths. AP diameter

• Ask them to maximally exhale at the bedside - - - if >6 seconds = obstructive defect

• Hyperinflation, hyperresonant

• Signs of cor pulmonale

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COPD• Chronic bronchitis: clinical diagnosis – productive cough for 3 months of the year for 2

consecutive yrs

• Emphysema: histological diagnosis – permanent airspace dilatation beyond terminal bronchioles without fibrosis

• FEV1<80% and ratio < 70%

• Remember A1AT-D – MM is normal; SZ and ZZ have low levels

• Failure to inhibit the action of the protease neutrophil elastase:

• Early onset emphysema (lower lobe), bronchiectasis.

• Chronic hepatitis ± cirrhosis, increased risk of hepatocellular carcinoma.

Presenting COPD• Gentleman who is breathless at rest (RR 24) and unwell

• There is no peripheral cyanosis or CO2 retention flap but the pulse is 85 BPM RR and bounding in nature

• There is tar staining in his fingers but no clubbing. He is plethoric with a JVP elevated at 45 degrees to 4 cm

• the expiratory component of respiration is prolonged, with signs of hyperexpansion evidenced by increased AP diameter and use of accessory muscles of respiration and no lymphadenopathy

• Whilst expansion is globally reduced and percussion note is resonant, auscultation demonstrates good air entry, coarse pan-inspiratory and expiratory crackles throughout and expiratory polyphonic wheezes

• There is no focal consolidation and the pulmonary component of the second heart sound is not prominent

• There is minimal pedal oedema

• The chest findings are consistent with an obstructive defect. This gentleman likely has COPD with evidence of CO2 retention but no focal consolidation. There are some signs of PHT. He is currently not on supplementary oxygen therapy. I would like to check the temperature and sputum pot

• He needs a CV exam and bedside spirometry to confirm the obstructive defect

• His pharmacological therapy should be optimised. He may be a candidate for LTOT and should be assessed for this

COPD

• Chronic bronchitis: clinical diagnosis – productive cough for 3 months of the year for 2 consecutive yrs

• Emphysema: histological diagnosis – permanent airspace dilatation beyond terminal bronchioles without fibrosis

• FEV1 < 80% and ratio < 70%

• Remember A1AT-D – MM is normal; SZ and ZZ have low levels

Ix

• Spirometry with bronchodilator response (to differentiate from asthma)

• FEV1/FVC ratio <0.7 and not fully reversible

• Arterial blood gas (ABG) (type 1 or 2 respiratory failure is possible)

• CXR (hyperinflation, flat hemidiaphragms, bullae, large prominent pulmonary arteries)

• Echo (PH)

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Rx• Stop Smoking• Pulmonary rehab

• Bronchodilators and inhaled steroids, mucolytics (carbocysteine)

• Sats > 90%

• Surgery

• LTOT: carboxyHb <3%, PaO2 <7.3 twice at least 3 weeks apart in stable patients

• Or <8 wtih secondary polycythaemia, pulmonary HTN, cor pulmonale (oedema) and nocturnal hypoxaemia

• FEV1 < 30% predicted

• Sats < 92% at rest

• Flu + pneumococcal vaccine• Nutritional

NICE COPD SEVERITY

• Mild: FEV1 >80%

• Moderate: FEV1 50%–80%

• Severe: FEV1 30%–50%

• Very severe: FEV1 <30%

Causes of COPD

• SMOKING

• A1AT deficiency

• Environmental and occupational – pollution, coal cadmium

• Genetic polymorphisms

How do you manage an acute exacerbation?• Manage RF, infection and chronic management• RF – CONTROLLED oxygen therapy. Air-driven bronchodilators (if nebs

don’t work, consider theophylline)• Pts depend on hypoxia to drive their respiratory centre

• Chronic CO2 retention causes insensitivity to acidosis

• Therefore too much oxygen makes them retain more CO2 and worsens acidosis

• NIV – BiPAP – prevents airways collapse at the end of respiration • Expiratory pressure recruits more lung units

• Improved V/Q ratio

• Steroids and antibiotics for infection – steroids reduce inflammation

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COPD Management

• SOB + FEV1 > 50% - LABA (salmeterol) or LAMA (tiotropium)

• Combination therapy for those with 2+ exacerbations per year and FEV1<50% of predicted

• Seretide – salmeterol and fluticasone

• Symbicort – budesonide and formoterol

• Work because of increased steroid dose (with each ‘reliever’ use)

• Consider home nebulisers, theophyllines and anti-mucolytics

COPD surgery

• Bullectomy: large bullae and FEV1 <50% predicted

• Lung volume reduction surgery (LVRS): upper-zone dominant emphysema, FEV1 >20% predicted, PaCO2 <7.3 kPa and transfer factor of the lung for carbon monoxide (TLCO) >20%

• Lung transplantation

Smoking cessation

• Nicotine replacement therapy (NRT), bupropion (a noradrenaline-dopamine reuptake inhibitor), and varenicline (a partial agonist of the α4β2 nicotinic acetylcholine receptor)

• NEVER use these in any combination together

• Careful with CV disease

• Bupropion lowers seizure threshold and is contraindicated in epileptics.

Causes of a Dull Percussion Note• Consolidation – increased fremitus and bronchil breathing

• Collapse – decreased fremitus and sounds

• Fibrosis – increased fremitus, inspiratory crackles - - - non-productive cough

• Raised hemidiaphragm

• Pleural thickening

• Pleural effusion

• Surgery – lobectomy, pneumonectomy, thoracoplasty

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Pulmonary Fibrosis

• Dyspnoea, oxygen

• Cyanosis

• Clubbed

• Thoracotomy scars

• Steroid therapy – purpura

• Cyclosporin therapy – gingival hyperplasia

Presenting Fibrosis• This gentleman is breathless at rest with a respiratory rate of 24 BPM but looks otherwise well

• There is some peripheral cyanosis noted in his nails but no CO2 retention flap. The pulse is 85 BPM RR

• There is notable clubbing in his fingers but no tar staining or signs of rheumatological disease. I noted purpura which may be consistent with steroid therapy. His JVP is elevated at 45 degrees to 5 cm

• The chest is not hyperexpanded and there are no chest wall deformities

• Whilst expansion is globally reduced and percussion note is resonant, auscultation demonstrates fine end inspiratory crepitations best heard in the bases

• I listened for but did not note a prominent pulmonary component of the second heart sound

• There is some pedal oedema

• This gentleman has signs of fibrotic lung disease which is possibly being treated with corticosteroids. He is currently not on supplementary oxygen therapy. I would like to check the temperature and sputum pot

• He needs a CV exam and bedside spirometry to confirm the restrictive defect

• I would like to investigate for pulmonary hypertension as a complication with an echo and for secondary causes of fibrotic lung disease including autoimmune conditions and environmental causes in the history

Associations – clues to causes

• Rheumatoid hands

• Systemic sclerosis: telangiectasia, calcinosis, sclerodactyly, ATROPHIC nails, Raynaud’s phenomenon

• Ankylosing spondylitis

• Amiodarone – skin pigmentation… Is the patient in AF?!

Complications

• Chest Infection

• Respiratory failure

• Cor pulmonale: pulmonary HTN - raised JVP, loud, palpable P2, RV Heave, PR/TR, PERIPHERAL OEDEMA, ASCITES, PLEURAL EFFUSIONS

• Cancer

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• Group of conditions related to different pathologies

• Broadly:

• Idiopathic or CFA

• Allergic reaction to organic proteins – EAA

• Reaction to inorganic dusts - pneumoconioses

How to think about “fibrotic lung disease”

How to think about “fibrotic lung disease”• CFA

• IPF or CFA (ATS/ERS classification has 7 histological subtypes of which UIP is commonest)

• Rheumatological: RA, SLE, SS, CAH• Drugs: amiodarone, gold, methotrexate, cytotoxics (cyclophosphamide)

• Hamman-Rich syndrome: acute with a high mortality rate

• Eosinophilic: nitrofurantoin, imipramine, sulfasalazine, ABPA

• Vasculitis: PAN, Wegener’s (nasal septum, epistaxis), Goodpasture’s, Churg-Strauss (asthma, eosinophilia)

• SMOKING is the single biggest risk factor

• Pneumoconioses: inorganic dusts (iron, tin, silicon, coal)

EAA Causes of Apical Fibrosis

• Berylliosis

• Radiation

• EAA

• ABPA

• Sarcoidosis

• TB

• Coal workers’ pneumoconiosis

• Psoriasis

• Ankylosing spondylitis

• Langerhans Cell Histiocytosis

• Silicosis

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Differentiating Environmental ILD Causes• All cause apical fibrosis: silicosis, berylliosis, EAA, Coal worker’s,

ABPA

• EXCEPT Asbestosis – basal fibrosis

• “A goes with B”

• Basal also includes: rheumatoid arthritis, CTDs, idiopathic, drugs (methotrexate and amiodarone)

Investigations (Bloods)• FBC – secondary polycythaemia

• Eosinophil count

• ESR, RF

• ACE and Ca - sarcoidosis

• Antibodies: full autoimmune screen… anti-GBM, pANCA (PAN), cANCA(Wegener’s)

• Precipitins – hypersensitivity pneumonitis

• CK for polymyositis

Investigations

• ABG: Type 1 respiratory failure

• Lung function tests – restrictive defects

• HRCT: ground glass appearance

• MRI if upper lobes

• Lung biopsy (transbronchial or VATS… or open)

• BAL – lymphocytosis predicts steroid responsiveness and better prognosis

Management• Stop smoking! - - - COPD long-term can cause fibrosis• Treat the cause• Steroids• If not working – switch to azathioprine, methotrexate, cyclophosphamide

• Anti-fibrotic – pirfenidone and nintedanib

• Gastric protection (e.g. proton pump inhibitors) for any patients with symptoms of reflux disease

• Surgery• Lung transplant

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Rheumatoid Lungs

• Lung fibrosis (which may also be secondary to methotrexate/gold treatment)

• Pleural effusions (usually asymptomatic)

• Intrapulmonary nodules (including Caplan’s syndrome)

• usually asymptomatic but may become infected and cavitate

• Haemopytsis

• Obliterative bronchiolitis – high pitched wheeze

Respiratory Clubbing

• Infective: TB, bronchiectasis, empyema

• Ca: mesothelioma, bronchial

• ILD

• Cystic fibrosis

Pleural Effusions• Causes: lung Ca, renal, liver, cardiac, CTD (RA/lupus)

• Pleural tap (should be US guided) sent for lactate dehydrogenase (LDH), protein, pH, amylase, glucose, cytology, microscopy and culture

• Transudate vs Exudate

• Malignant recurrent - - - pleurodesis with talc, doxycycline, bleomycin

• Pleurodesis also used for recurrent pneumothorax

• Empyema (complication of pneumonia): pus in pleural cavity with a pH <7.2

Light’s Criteria

• Between 25 and 35 g/L protein

One of the following:

• Fluid to serum protein ratio > 0.5

• Fluid LDH: serum LDH > 0.6

• Fluid LDH > 2/3 upper limit of normal serum LDH

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Exudates and Transudates

• Exudates: 4 I’s: Infiltration (neoplasm), infection, infarction (pulmonary embolus), inflammation (rheumatoid arthritis and SLE)

• Transudates: Cardiac failure, chronic renal disease, chronic liver disease

• Rarer causes include Meigs’ syndrome

Bronchiectasis

• Inspiratory clicks, clubbing

• Yellow nails, curved?

• Crackles ALTER with coughing (they don’t in fibrosis)

• Classically early to mid inspiratory crackles (as opposed to fibrosis)

Causes• Bronchial obstruction – foreign body, tumour, lymph nodes, granulomata (including

TB)

• Childhood infections: measles, pertussis, TB

• Immunodeficiency: immunoglobulin

• Mucociliary clearance defect: CF: Chromosome 7, deletion F508;

• Kartagener’s, Young’s, Primary Ciliary Dyskinesia (chronic upper respiratory tract problems/otitis media)

• ABPA

• Yellow nail syndrome – lymphoedema, sinusitis, exudative pleural effusions, bronchiectasis

• Traction bronchiectasis – after fibrosis

Investigations• CXR – tramline shadows – bronchial thickening

• Young patient - - - - genetic analysis delta F508

• Spirometry and HRCT – bronchial thickening and “signet ring” sign (airway size greater than accompanying artery size)

• Sputum MC&S• Bloods: Check serum immunoglobulin levels (immunoglobulin IgA,

IgG, IgM) - immunodeficiency as a cause, Aspergillus serology (total IgE, IgE to Aspergillus and Aspergillus precipitins)

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Specific causes

• CF: Two measurements of sweat chloride and cystic fibrosis transmembrane conductance regulator (CFTR) genetic mutation analysis.

• Ciliary dysfunction (e.g. primary ciliary dyskinesia): Saccharin test or cilial electron microscopy to assess ciliary function.

Rx• STOP/AVOID smoking

• Rotating antibiotics

• Acute: involves cover for pseudomonas (piperacillin, ceftazidime, carbapenems, aminoglycosides)

• Long-term antibiotics for patients with ≥3 exacerbations per year

• Nebulized colomycin/tobramycin if Pseudomonas colonization.

• Possible disease-modifying action of azithromycin.

• Carbocysteine

• Oxygen?

• Psychological support

• Chest physiotherapy

Presenting Bronchiectasis• Gentleman who is breathless at rest (RR 24) and unwell

• There is no peripheral cyanosis or CO2 retention flap but the pulse is 85 BPM RR and bounding in nature

• There is tar staining in his fingers which are clubbed. He is plethoric with a JVP elevated at 45 degrees to 4 cm

• the expiratory component of respiration is prolonged and occasional productive cough was noted throughout the exam with sputum production. There was no lymphadenopathy

• Expansion is normal bilaterally, percussion note is resonant globally, auscultation demonstrates good air entry with coarse pan-inspiratory and expiratory crackles throughout and expiratory polyphonic wheezes

• There is no focal consolidation and the pulmonary component of the second heart sound is not prominent

• There is minimal pedal oedema

• The chest findings are consistent with an obstructive defect. This gentleman likely has bronchiectasis with no focal consolidation. There are some signs of PHT. He is currently not on supplementary oxygen therapy. I would like to check the temperature and sputum pot

• He needs a CV exam and bedside spirometry to confirm the obstructive defect. Cross-sectional imaging can help to confirm the diagnosis of bronchiectasis

• If there is any evidence of infection, a prolonged course of antibiotics is indicated and recurrent infections may indicate a need for long-term rotating courses of antibiotics.

- Permanent dilatation of terminal bronchioles

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TB Medications

• Isoniazid – enzyme inhibitor

• Rifampicin – enzyme inducer

• Drug-induced hepatitis: Rifampicin, isoniazid and pyrazinamide (check LFTs prior to commencement)

• Optic neuritis: Ethambutol (visual acuity should be documented before starting treatment)

• Peripheral neuropathy: Isoniazid (coprescribe pyridoxine)

TB

• Skin – lupus vulgaris

• Pott’s disease

• Sputum staining: Ziehl–Neelsen staining for acid-fast bacilli.

Lobectomy

• TB

• Bronchiectasis – uncontrolled symptoms

• Malignancy ----- NSCLC

• Cystic fibrosis

• Lung Abscess

• Single pulmonary nodule – eg RA

Pneumonectomy

• Bronchiectasis

• TB

• Malignancy

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Lung Ca• Small cell

• SIADH

• ACTH

• Lambert-Eaton

• Squamous

• Hypercalcaemia – PTHrP

• Hyperthyroidism – TSH

• Adeno - Clubbing, HPOA and gynaecomastia (produces HCG)

Lung Ca• NSCLC (85%) – adeno, squamous, large cell

• Pancoast’s syndrome

• apical lung tumour involvement of the brachial plexus and cervical sympathetic nerves

• patients may complain of pain in the shoulder/anterior chest wall and arm weakness, and have wasting of the intrinsic muscles of the hand and an ipsilateral Horner’s syndrome

• (ptosis, miosis, anhidrosis and enophthalmos)

Surgery for lung cancer

• FEV1 >1.5

• a transfer factor >50%

• No evidence of severe PH

• Non-metastatic

Lung Transplant

• Emphysema (usually with alpha-1 antitrypsin deficiency)

• Idiopathic pulmonary fibrosis

• Idiopathic pulmonary hypertension

• Bronchiectasis

• Cystic fibrosis

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Sleep disordered breathing

• Daytime somnolence, unrefreshing sleep, morning headaches, impaired concentration, short- term memory loss, personality change, sexual dysfunction, nocturnal choking, snoring, nocturia.

• The Epworth Sleepiness Score (ESS)

• Total score >10, or sleepiness in dangerous situations, even with a normal ESS, warrants sleep evaluation.

• ESS validated for obstructive sleep apnoea (OSA), and narcolepsy.

Causes

• OSA – pharyngeal collapse

• Central sleep apnoea: a cessation or decrease in ventilatory effort during sleep ± wakefulness. Often related to cerebrovascular, cardiac, or neurological disease

• Mixed sleep apnoea: when OSA is severe and long-standing, central apnoea may develop.

• Chronic opiate use.

OSA

• Men, hypothyroid, diabetic, nasal and pharyngeal issues, acromegaly, alcohol

• Lifestyle advice, nocturnal CPAP

• Complications: Hypertension, arrhythmia, myocardial infarction, stroke, obesity, diabetes mellitus, pulmonary hypertension.

Obesity hypoventilation (Pickwickian) syndrome

• Characterized by obesity (BMI ≥30 kg/m2), chronic hypercapnia (PaCO2 >6 kPa), and sleep disordered breathing.

• Approximately 90% of patients also have OSA.

• Management: nocturnal CPAP.

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Hoarse Voice

• Laryngeal issues – polyp, carcinoma, inflammation (singing, GORD, steroid inhalers - Candida)

• Vocal cord paralysis: thyroid masses, lung cancer, Ortner’s syndrome

• Laryngoscopy