VASCULITIS AFFECTING THE KIDNEY - PRESENTATION, HISTOPATHOLOGY AND LONG-TERM OUTCOME

  • 1 January 1984
    • journal article
    • research article
    • Vol. 53  (210) , 181-207
Abstract
Patients (53) who by clinical and/or histological criteria were judged to have vasculitis affecting the kidney were reviewed. Patients with systemic lupus or Schoenlein-Henoch purpura were excluded. Segmental necrotizing glomerulitis was taken as a manifestation of vasculitis (microscopic polyarteritis). Those patients in whom vasculitis was confined to glomerular capillaries were compared with those who in addition had vascular lesions outside the glomeruli. The 2 groups were found to have identical clinical features. Clinical presentation was predominantly extrarenal and exclusively renal in only 2. Three patients were classified histologically as Wegener''s granulomatosis, 4 had malignant tumors and 2 relapsing polychondritis. Hepatitis B surface antigen was absent in all of 37 patients tested. Renal disease presented with microscopic hematuria and minor proteinuria in the majority (32) but 16 patients presented with rapid deterioration of renal function, including 7 with anuria. Histologically 42 patients showed segmental necrotizing glomerulitis (microscopic polyarteritis), 6 diffuse proliferative glomerulonephritis and in 5 only minor or ischemic glomerular changes were present. Crescents were found in 41/42 of those with segmental necrotizing glomerulitis, and involved more than 50 per cent of glomeruli in 15 patients. Immunohistochemical studies were generally negative except for fibrin. EM showed no evidence of immune deposits in necrotizing glomerulitis, but these were present in 2/6 patients with diffuse proliferative glomerulonephritis. Overall prognosis was poor, 35 patients having died, 20 early in the course of the disease and 15 later. Oligoanuria and extensive crescent formation were adverse signs. Survivals were 54, 38 and 34% at 1, 5 and 10 yr, respectively. Of those who survived the initial illness, 12 were stable with apparently inactive disease, while 16 continued to show clinical signs of activity and required treatment. A variety of treatments was employed including oral and i.v. corticosteroids, immunosuppressive agents, anticoagulants; only 1 patient was treated by plasmapheresis. It is difficult to draw any firm conclusions as to the efficacy of treatment and survival rates remain unsatisfactory.