A man aged 78 years was hospitalized 4 weeks before his death with a history over 3 months of increasing dyspnea and malaise. Examination revealed atrial flutter and signs of chronic obstructive airways disease. Initial tests showed acute renal failure in association with a raised ESR (59mm/hr) and rheumatoid factor (92iu/ml; normal range, 0 - 40iu/ml). Tests for perinuclear anti-neutrophil cytoplasmic auto-antibodies (pANCA) were strongly positive, but negative for various anti-nuclear antibodies and cANCA. The patient's renal failure deteriorated markedly, and he died despite treatment with steroids.
At autopsy, both kidneys (left, 165g; right 135g) showed mild nephrosclerosis and pale areas of infarction throughout their cortices. There was no evidence of urinary obstruction, or of macroscopic renal vascular occlusion. Moderate atrophy of the cerebellar vermis was the only abnormality on sectioning the brain (1335g). There was no macroscopic infarction. Arteries of the circle of Willis and vertebrobasilar system contained some plaques of atherosclerosis, but were otherwise normal. A large subarachnoid hemorrhage (SAH) surrounded the lower half of the spinal cord and cauda equina (Fig. 1). No arteriovenous malformation was found upon dissection of the hematoma, and the cord appeared normal.
DIAGNOSIS AND DISCUSSION