Abstract

The patient is a 45-yr-old Caucasian man with no significant past medical history, who in December 2006 developed arthralgias affecting both shoulders and hips and subsequently noticed shortness of breath with a cough, and a lower limb rash. These symptoms did not improve with diclofenac or with a course of antibiotic therapy prescribed by his general practitioner. The patient presented to his local hospital when he developed hemoptysis, epistaxis, and sinusitis, as well as a worsening of his shortness of breath, rash. and arthralgias. He had lost 3.5 kg in weight during the course of his illness. He was on no medication except for diclofenac, prescribed in the previous month, and a course of amoxicillin. His family history included rheumatoid arthritis and lung cancer in two family members, both of whom were heavy smokers. He was originally from Ireland but had been living in the United Kingdom for the previous 25 yr. He had a partner and three children and was an ex-smoker. At the local hospital, examination was unremarkable except for a lower limb vasculitic rash. Initial blood tests (Table 1) revealed significant renal impairment with a serum creatinine of 345 μmol/L (normal range 50 to 110 μmol/L) and an acute inflammatory response with a C-reactive protein (CRP) of 204 mg/dl and a serum albumin of 31 g/L. He was treated with antibiotics for a presumed chest infection and with intravenous fluids. On the sixth day of admission, creatinine had increased to 563 μmol/L and he was acidotic with serum bicarbonate 18 mmol/L, at which point he was admitted to the intensive care unit for hemofiltration. Hemoglobin had fallen to 9 from 12 g/L on admission. Further investigations revealed an antineutrophil cytoplasm antibody (ANCA) by immunofluorescence and an anti-PR3 antibody concentration of 728 IU/ml (normal range 0 to 19 …

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