Clinical and pathologic features of polyarteritis nodosa and its renal-limited variant: primary crescentic and necrotizing glomerulonephritis.

This study supports the concept that primary necrotizing and crescentic glomerulonephritis is a kidney-limited form of polyarteritis nodosa. Thirty-four patients with necrotizing and crescentic glomerulonephritis were divided into three groups based on the presence or absence of systemic vasculitis as determined by clinical or histologic criteria. Laboratory studies demonstrated elevated erythrocyte sedimentation rates, anemia, mild eosinophilia, hematuria, and proteinuria in patients in each group; there were no significant differences in these data between the groups, however. Complement levels and antinuclear antibody screens were normal. Mean serum creatinine levels were markedly elevated but fell by a factor of two following therapy. There was a higher morbidity in the patients with kidney-limited disease. This was attributable to a higher percentage of these patients' having no symptoms and presenting for medical care only after they were in chronic renal failure. Most patients not experiencing chronic renal failure were treated with cyclophosphamide and prednisone, which seemed effective in this retrospective study.