Endgames Case Review

A woman with upper and lower airway symptoms

BMJ 2015; 350 doi: https://doi.org/10.1136/bmj.h2708 (Published 21 May 2015) Cite this as: BMJ 2015;350:h2708
  1. Jennifer Capps, core medical trainee1,
  2. Constantina Chrysochou, renal medicine consultant2,
  3. William Flight, consultant respiratory physician3
  1. 1Royal Bolton Hospital, Bolton, BL4 0JR, UK
  2. 2Salford Royal Hospital NHS Foundation Trust, Salford, UK
  3. 3Oxford University Hospitals NHS Trust, Oxford, UK
  1. Correspondence to J Capps Jennifer.capps{at}doctors.org.uk

A 65 year old woman presented with a four week history of lethargy, cough, and feeling generally unwell. She had recently been treated for an ear infection and had occasional epistaxis but had no other medical history. She had never smoked. On examination she was did not have a fever and had crepitations in the base of her right lung. A chest radiograph showed right basal consolidation. Her blood tests showed: white blood cell count 17.5×109 cells/L (reference range 4-11), C reactive protein 3171 nmol/L (<47.6), urea 9.8 mmol/L (2.5-7.8), and creatinine 98 µmol/L (62-124). A diagnosis of community acquired pneumonia was made.

After seven days of treatment with antibiotics her symptoms had not improved. A repeat chest radiograph showed worsening bilateral inflammatory changes. Sputum and blood cultures were negative, as were tests for Legionella and Pneumococcus urinary antigens. Urine dipstick was positive for blood. At bronchoscopy, bloodstained mucus was found within the airways. Bronchial washings were negative for cancer cells and pathogens.

Her renal function then deteriorated (urea 10.6 mmol/L, creatinine 178 µmol/L) and intravenous fluids were started. A further chest radiograph showed progression of the inflammatory changes and a new left pleural effusion. A contrast enhanced computed tomogram of the chest was considered but not performed owing to worsening renal function. Further blood tests showed that she was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA).

Questions

  • 1. What is the likely diagnosis?

  • 2. What history or examination findings might have suggested the diagnosis?

  • 3. How is this diagnosis confirmed?

  • 4. How should this patient be managed once the diagnosis is confirmed?

Answers

1. What is the likely diagnosis?

Answer

Granulomatosis with polyangiitis (previously known as Wegener’s granulomatosis), a small vessel vasculitis that predominantly affects the kidneys and respiratory tract. Other possible differentials (before the positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) result) included atypical pneumonia, a complication …

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