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A man with Wegener’s granulomatosis and haemoptysis

BMJ 2009; 338 doi: http://dx.doi.org/10.1136/bmj.b1461 (Published 22 April 2009) Cite this as: BMJ 2009;338:b1461
  1. Richard Lee, ST2 academic clinical fellow, allergy and respiratory medicine,
  2. Rhodri Martin, ST1 core medical training ,
  3. David D’Cruz, consultant rheumatologist
  1. 1Lupus Research Unit, Rayne Institute, St Thomas’ Hospital, London SE1 7EH
  1. Correspondence to: D D’Cruz david.d’cruz{at}kcl.ac.uk

    A 60 year old man with Wegener’s granulomatosis who was clinically stable on methotrexate presented with a two day history of haemoptysis. On examination he was febrile, with bibasal crepitations and bronchial breath sounds in the left upper zone.

    Pulse oximetry showed an oxygen saturation of 96% on air. A full blood count showed that his haemoglobin was 134 g/l, white blood cells were 18.4×109/l (neutrophils 15.3×109/l), and serum creatinine was 74 μmol/l. He had a titre of 1:160 on recent serological testing for cytoplasmic staining antineutrophil cytoplasmic antibodies.

    Urinalysis detected trace amounts of protein and blood, but he had no dysmorphic red cells or casts on urine microscopy.

    He underwent computed tomography of the chest and chest radiography (figs 1 and 2).

    Fig 1 Chest radiograph

    Fig 2 Computed tomography of the chest

    Questions

    • 1 What are the likely causes of haemoptysis in this patient?

    • 2 How would you investigate this man further?

    • 3 What is this condition and how should it be treated?

    Answers

    Short answers

    • 1 Bilateral air space infiltration (figs 3 and 4) and haemoptysis suggest a relapse of Wegener’s granulomatosis, with pulmonary capillaritis causing diffuse alveolar haemorrhage. Pneumonia and pulmonary embolus are differential diagnoses. Haemoptysis could also be caused by bronchiectasis, malignancy, uraemia, coagulopathy, or congestive cardiac failure.

    Fig 3 Chest radiograph showing bibasal and right upper lobe air space shadowing

    Fig 4 Computed tomography of the chest confirming bilateral air space infiltrates (arrowheads) and surrounding ground glass shadowing

    • 2 Bronchoscopy is useful because lavage specimens are increasingly blood stained in diffuse alveolar haemorrhage; gas transfer is raised on pulmonary function testing in this condition. Microbiological analysis of sputum, blood, and bronchoalveolar lavage specimens is needed to identify an infective component.

    • 3 Diffuse alveolar haemorrhage—a severe complication of active vasculitis. …

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