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An unusual case of haemoptysis. Hardy Firm. Mr X is a 60yr old Caucasian man from Levenshulme. PC: Massive haemoptysis Short of breath Pleuritic chest pain localised to the upper zone of the left hemithorax. Initial concerns?. Cancer TB. HPC Non-productive cough for 1 week
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An unusual case of haemoptysis Hardy Firm
Mr X is a 60yr old Caucasian man from Levenshulme PC: • Massive haemoptysis • Short of breath • Pleuritic chest pain localised to the upper zone of the left hemithorax
Initial concerns? • Cancer • TB • HPC • Non-productive cough for 1 week • No previous haemoptysis • He is regularly SOB on exertion, but his SOB on this occasion is much worse • No weight loss • No night sweats
Differential Diagnosis? • Causes of haemoptysis....
PMH: • 2 x TIA • Hypertension • Peptic ulcer repair plus vagotomy • Cholecystectomy • Hernia repair • COPD - previous admission to hospital in Feb 2011 with cough, SOB, and green sputum
DIAGNOSIS? Emphysematous bullous disease
Treatment: • Left upper bullectomy - increased Mr X’s FEV1 by 10%
SH: • Ex-smoker - 35 pack years, stopped in 2010 • Retired at age of 53 - was a manager of a newspaper company • Drinks about 14 units of alcohol/week • Lives with his wife - both have no history of travel outside the UK DH: • Symbicort • Salbutamol • Tiotropium • Azithromycin • Simvastatin • Ramipril • Aspirin • Lansoprazole • No known allergies
On this admission Reminder of PC: • Massive haemoptysis • Short of breath • Pleuritic chest pain localised to the upper zone of the left hemithorax Examination findings: • Tachycardic • Tachypnoeic • Quiet, dull left upper zone • Expiratory wheeze • BP: 100/70 • GCS: 15 • Temp: 36.2
Investigations: Reminder of PC: • Massive haemoptysis • Short of breath • Pleuritic chest pain localised to the upper zone of the left hemithorax Bloods: • Hb:11.3 • WCC: 14.8 • Creatinine: 68 • CRP: 83 ABG’s: • pH: 7.66 • pO2: 10.1 • pCO2: 3.29 • BE: -0.7 • Bicarb: 24.7 02: 100% on 24% oxygen??
DIAGNOSIS Left upper lobe bulla containing acute large haematoma plus active haemorrhage
Treatment: • Embolisation of the arteries involved using PVA (polyvinyl alcohol) particles • Multiple blood transfusions • Discharged 10 days later
Emphysema • Emphysema = dilatation + destruction of lung tissue distal to terminal bronchiole • Loss of elastic recoil • Collapse of small airways on expiration = expiratory wheeze COPD = emphysema + chronic bronchitis • UK: causes 18m lost working days for men/y
Pathophysiology • Infiltration of bronchiole walls with inflammatory cells predominantly CD8+ • Scarring and thickening of walls = narrowing • Epithelial layer: columnar cells replaced by squamous cells • Emphysema causes expiratory flow limitation and air trapping = pursed lip breathing • VA/Q mismatch = tachypnoeic
Classifications of Emphysema • Centri-acinar • Around bronchioles affected, common • Pan-acinar • Whole lung affected, less common • Severe can become bullous • Occurs in alpha- antitrypsin deficiency • Irregular • Scarring and damage is patchy
Signs Symptoms • Often no signs if mild • Tachypnoea + prolonged expiration • Accessory muscle use • Hoover sign = paradoxical lower chest movement • Hyperinflation and loss of cardiac + liver dullness • Hypercapnia: bounding pulse and flapping tremor • Productive cough with white/clear sputum • Wheeze • Breathlessness • Frequent infective exacerbations • Worsened by cold weather and pollution • Severe breathlessness on exertion • Systemic effects
Diagnosis • Clinical history and examination • Pulmonary function tests • Chest X-ray • CT • Blood gases • ECG • Alpha- antitrypsin levels and genotype
Definition Bullous Lung Disease • Bulla (pl. bullae) – A thinly walled (<1mm thick) air filled space within the lung parenchyma of >1cm diameter. • Bleb – Gas containing space between lung parenchyma and visceral pleura or between the pleura. [1]
Bullous Lung Disease Aetiology • Chronic disease • Diffuse emphysema • Cystic Fibrosis • Sarcoidosis • Acquired • Cannabis smoking • Occupational exposure (carbon, silica) • Genetic • Marfan’s syndrome • Ehler-Danlos syndrome • Alpha 1-antitrypsin deficiency • Alpha 1- antichymotrypsin deficiency
Bullous lung disease Pathophysiology
Bullous Lung Disease Complications • Spontaneous pneumothorax • Infection • Haemorrhage • Lung cancer
Emphysematous lung disease: Vanishing lung syndrome, Chronic emphysema. Fibrotic lung disease: Sarcoidosis and Ankylosing Spondylitis are conditions characterised by fibrosis of the lung tissue which predisposes to bullae development. Genetic conditions: Birt-Hogg-Dube syndrome, Marfan Syndrome, Ehlers-Danlos Syndrome. Infective: HIV infection Bullous Lung Disease Aetiology
Bibliography • http://www.umm.edu/patiented/articles/emphysema_000195.htm • Kumar and Clark, p835-839