Clinical Review Lesson of the week

Wegener's granulomatosis presenting as a pleural effusion

BMJ 2003; 327 doi: https://doi.org/10.1136/bmj.327.7406.95 (Published 10 July 2003) Cite this as: BMJ 2003;327:95
  1. Adrian G Blundell, senior house officer in medicine (adrianblundell@ntlworld.com)1,
  2. Simon Roe, consultant nephrologist1
  1. 1Nottingham Renal and Transplant Unit, Nottingham City Hospital, Nottingham NG5 1PB
  1. Correspondence to: A G Blundell
  • Accepted 5 March 2003

Introduction

Wegener's granulomatosis is one of the pauci-immune small vessel vasculitides. It classically presents with the triad of upper and lower respiratory tract granulomas and necrotising focal segmental glomerulonephritis. It is associated with the presence in the serum of autoantibodies againstcomponents of neutrophil cytoplasmantineutrophil cytoplasmic autoantibodies (ANCA). The illness can develop at any age but is more common in patients in their 50s and 60s and in men. The incidence of vasculitis is increasing, with about 10-20 people per million affected. We present a case that in retrospect had many clues at the initial time of admission, but it took five months and six different hospital teams to make the diagnosis.

Case report

A 64 year old woman, who had had breast carcinoma that had been treated with wide local excision and radio-therapy six years previously, was admitted to her local hospital at the end of March 2001. She presented with a two week history of an influenza-like illness, including a blocked nose and right ear, dry cough, and intermittent sweats. She was feverish and had a left pleural effusion, which was confirmed radiologically. Her inflammatory markers were raised (total white cell count 13.1x109/l, C reactive protein 322 mg/l (normal range < 5 mg/l)). Treated was started with intravenous antibiotics for a possible empyema. Despite three …

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