Temporal arteritis with pauci-immune glomerulonephritis: a systemic disease.

Temporal arteritis is easily diagnosed and responds gratifyingly to treatment. Renal complications are unusual, but nevertheless occur. Earlier, an association between pauci-immune glomerulonephritis and temporal arteritis was shown. We present a patient who clearly had temporal arteritis but also developed cerebral hemorrhage, pulmonary infiltrates related to granulomatous pulmonary vasculitis, and pauci-immune glomerulonephritis. We suggest that temporal arteritis is neither always localized nor temporal. Instead, the condition can be a lethal, systemic disease. Renal involvement in patients with temporal arteritis is not common and the presence of glomerulonephritis is rare [Jennette and Falk 1994]. Lenz et al. [1998] described a patient who developed vision loss, optic nerve atrophy, elevated erythrocyte sedimentation rate, a positive rheumatoid factor and terminal glomerulonephritis. The renal biopsy showed focal and segmental necrotizing glomerulonephritis, despite negative antineutrophil cytoplasmatic antibodies (ANCA), antinuclear antibodies and antiglomerular basement membrane antibodies. Giant cells were identified in the necrotic vessel walls within the kidney. Immunofluorescence was negative and a diagnosis of ANCA-negative pauci-immune glomerulonephritis was made. The patient did not respond to immunosuppression and developed end-stage renal disease. Although the clinical attributes were consistent with temporal arteritis, no temporal artery biopsy was done in that patient. We recently treated a patient with temporal arteritis and pauci-immune glomerulonephritis. Our patient's course was somewhat different in comparison to the patient described by Lenz et al. [1998].