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A struggle to breathe

A struggle to breathe. Dr Hardy Firm: Verena Walsh, Sofia Thorell , Caroline Sargeant , Patrick Birch, Helen Thornton, Hitesh Khanna , Alex Lomax & Michael Shipton. Case Presentation.

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A struggle to breathe

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  1. A struggle to breathe Dr Hardy Firm: Verena Walsh, Sofia Thorell, Caroline Sargeant, Patrick Birch, Helen Thornton, Hitesh Khanna, Alex Lomax & Michael Shipton

  2. Case Presentation • A 71 year old retired manual labourer was admitted to AM1 on 25th February 2013 with severe shortness of breath despite home oxygen

  3. History of Presenting Complaint • 2 day history of increasing shortness of breath

  4. Differential Diagnosis? • Pneumonia • Pulmonary fibrosis • Exacerbation of COPD • Pulmonary embolism • Pneumothorax • Left ventricular failure/CCF • Pericarditis

  5. Past Medical History • Rheumatoid arthritis • Systemic lupus erythematosus • Type 2 diabetes • Hypertension • NSTEMI June 2012 – IHD • Avascular necrosis of the left femur hip 2003 • Osteoporosis • GORD

  6. Medication • Current • Lansoprazole • Risendronate • Prednisolone • Diazepam • Co-codamol • Previously on • Adalimumab • Methotrexate • Current • Sitagliptin/Metformin (Janumet 50/1000) • Furosemide • Bisoprolol • Atorvastatin • Clopidogrel • Aspirin

  7. Family and Social History • No relevant family history • Social history • Manual labourer retired in 1997 • Lives at home with wife • Has no carers • Is on home oxygen

  8. Examination Findings • Flapping tremor • Raised JVP • RR: 22 breaths/minute • HR: 90 bpm; BP: 135/85 mm Hg • Temperature: 38.2°C • Finger clubbing • Dull note to percussion over the right middle lobe • Decreased breath sounds • Coarse crackles bilaterally over the lung bases

  9. Differential Diagnosis • Pneumonia • Pulmonary fibrosis ± superimposed infection • Exacerbation of COPD • Pulmonary embolism • Pneumothorax • Left ventricular failure/CCF • Pericarditis

  10. Investigations • Arterial Blood Gas • pH 7.32 • PaO2 : 7.13 kPa (on 28% O2) • PaCO2: 6.9 kPa • Bicarbonate: 37 mmol/L • Base excess: +14.3 • Lactate: <2 mmol/L

  11. Laboratory results to note • CRP 62 mg/L • WCC 15.3 x 109 cells/L (neutrophils 11.1 x 109 cells/L) • U&Es – no abnormal values • LFTs – nothing of note except low albumin

  12. Further Investigations • Chest X-ray showed • Right middle lobe pneumonia • Bilateral pleural effusion

  13. Diagnosis • Pulmonary fibrosis with superimposed infection • His risk factors include: • Occupational exposure • Rheumatoid arthritis • Systemic lupus erythematosus • Drugs • Methotrexate • Monoclonal antibodies

  14. Pulmonary Fibrosis: The Pathophysiology • Exchange of normal lung parenchyma with fibrotic tissue • Fibrotic tissue deposition is irreversible • Causes a decrease in oxygen diffusion capacity • Restrictive lung disease • Reduced air intake

  15. Pulmonary Fibrosis:Aetiology May be idiopathic – idiopathic pulmonary fibrosis (IPF) Risk factors: • Environmental/occupational pollutants - asbestos, silica etc. • Tobacco smoke • Connective tissue diseases - RA, SLE, scleroderma • Granulomatous diseases - Wegener’s, sarcoidosis • Infections - TB, viral, fungi etc. • Drugs - amiodarone, methotrexate, nitrofurantoin, busulfan, bleomycin, monoclonal antibodies • Radiation therapy to the chest

  16. Epidemiology Prevalence 6.8-16.3 per 100,000 person-years Increasing incidence of IPF – Up 10% between 1991-2003 128,100 people in USA UK - 5,000 deaths a year Men > Women (M:F ratio 1.7:1) Age - Between 50 and 70 years old

  17. IPF has been recognized in animals. • Legendary stuntman EvelKneivel died of IPF

  18. Symptoms • Commonly - increasing SOB and dry cough. • 5% - asymptomatic • 50% - systemically unwell with flu-like illness, fatigue or weight loss. • Extrapulmonaryfeatures – arthralgia, muscle pains and skin rashes. • Obstructive sleep apnoea may be a common presenting feature.

  19. Signs • Exertional dyspnoea progressing to breathlessness at rest • Tachypnoea • Cough • Clubbing • Cyanosis • Fine bilateral basal crepitations particularly at the end of expiration • Signs of cor pulmonale and right heart failure in the later stages

  20. Investigations • Blood • ABG • Raised CRP • Raised immunoglobulins • 30% of IPF are ANA +ve • 10% IPF are rheumatoid factor +ve • CXR • Reduced lung volume • Bilateral lower zone reticulo-nodular shadows • Advanced disease: honeycomb lung • HR-CT shows similar changes, but is more sensitive

  21. More Investigations • Bronchoalveolar lavage • Indicates activity of alveolitis • Raised lymphocytes = good response or prognosis • Raised neutrophils and eosinophils = poor response/prognosis • Lung biopsy • May be needed for diagnosis • Usual interstitial pneumonia (UIP)

  22. Obstructive vs. restrictive lung disease • Spirometry used to measure FEV1 and FVC • FEV1 – forced expiratory volume in 1 second • FVC – forced vital capacity • Both related to height, age and sex • FEV1/FVC – good measure of airflow limitation. Normal is 75%

  23. Obstructive lung disease • FEV1/FVC is reduced as obstruction to airflow results in greater decrease in FEV1 than FVC • For example, FEV1 = 1 L and FVC = 3 L. FEV1/FVC = 33.3% • Causes – COPD, asthma

  24. Restrictive lung disease • FEV1/FVC may be normal or even increased – FEV1 and FVC both reduced; ratio may rise due to greater elastic recoil • For example, FEV1 = 1.8 L, FVC = 2 L. FEV1/FVC = 90% • Causes – lung fibrosis, connective tissue diseases, pleural effusion, kyphoscoliosis, neuromuscular disorders

  25. Management • No effective treatment • Especially poor prognosis for idiopathic pulmonary fibrosis • 3 aims: • Behaviour change • Symptom relief • Slow progression of disease

  26. Lifestyle changes • Smoking cessation • Regular exercise (pulmonary rehabilitation) • Eating a balanced diet • Flu jab/Pneumococcal vaccine • Avoid others with colds/chest infections

  27. Symptom Control • Stop causative agent if known to prevent further progression • Corticosteroid with • Azothioprine • Methotrexate • Cyclophosphamide • Cyclosporin • Penicillamine • Sildenafil (Viagra) • Oxygen • Anticoagulation • High risk for pulmonary emboli

  28. Slowing disease progression • N-acetylcysteine (an antioxidant) • Pirfenidone • Trials - Gamma interferon/mycophenolate mofetil Surgical Treatment • Lung transplant Prognosis: 50% 5 yr survival rate (range 1-20)

  29. Management • Co-amoxiclav i.v. • 120 mg BD furosemide p.o. • Pleural effusion • Prophylactic LMWH • High risk of PE • Echo showed congestive cardiac failure

  30. In Summary... • Progressive disease caused by irreversible fibrotic tissue deposition • Risk factors: environmental factors, autoimmune diseases, infections and medications • Presents with a dry cough, exertional dyspnoea and fine end-inspiratory crackles • Restrictive lung disease so FEV1/FVC ratio is normal or increased as both volumes are reduced • Treatment is by removing the cause (if there is one) and symptomatic relief

  31. References • http://www.healthline.com/hlcmsresource/images/slideshow/heart_attack/slide03-sweating.jpg • http://almostadoctor.co.uk/content/systems/-respiratory-system/restrictive-vs-obstructive-lung-disease • Repiratory Disease. In Kumar P and Clark M (Eds) (2009) Kumar and Clark’s Clinical Medicine (7th edition). Edinburgh: Saunders Elsevier, 826

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