This case was a typical presentation for Wegener's granulomatosis with respect to the pulmonary and renal findings. Over the course of the past ten years this patient has done relatively well with only two major exacerbations requiring hospitalization. His renal function has apparently not been compromised to date. The finding of limited or no significant upper respiratory involvement is interesting in this case and is rather atypical for this disease. This may well represent a limited variant of Wegener's granulomatosis or possibly an excellent response of the Wegener's syndrome to cytotoxic chemotherapy. Finally, this case illustrates how appropriate clinical and pharmacological management has greatly improved the morbidity and mortality of this once uniformly fatal disease.
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