Clinical and immunological follow-up of patients with severe renal disease in Wegener's granulomatosis

Am J Nephrol. 1985;5(1):21-9. doi: 10.1159/000166898.

Abstract

Clinical and immunological data are reported of 12 patients suffering from Wegener's granulomatosis and severe renal involvement. Although 9 patients recovered from their acute illness, a long-term follow-up a relapse occurred in 4 of these 9 patients. Therefore, lifelong follow-up in this group patients seems to be mandatory. Extensive immunological investigations did not provide evidence for humoral mechanisms underlying the pathogenesis of this disease; T lymphocyte subsets in peripheral blood as well as functional reactivity of lymphocytes in vitro were also normal. However, none of the patients was able to mount a primary cellular immune response in vivo. On the other hand, kidney biopsy specimens obtained before the initiation of drug therapy revealed periglomerular and interstitial cellular infiltrations consisting predominantly of T lymphocytes with a ratio Leu 3a (OKT4)/Leu 2a (OKT8) of 5:1. This may indicate that a type IV (delayed-type) hypersensitivity reaction takes place in the kidney. These findings suggest that an abnormal cellular immunoreactivity plays a major role in the pathogenesis of Wegener's granulomatosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antibodies, Monoclonal
  • Azathioprine / therapeutic use
  • Biopsy
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Fluorescent Antibody Technique
  • Follow-Up Studies
  • Granulomatosis with Polyangiitis / complications
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / immunology*
  • Granulomatosis with Polyangiitis / mortality
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Immunity, Cellular
  • Kidney / immunology
  • Kidney / pathology
  • Kidney Diseases / etiology*
  • Kidney Diseases / pathology
  • Male
  • Middle Aged
  • Oliguria / etiology
  • Oliguria / therapy
  • Prednisone / therapeutic use
  • T-Lymphocytes / classification

Substances

  • Antibodies, Monoclonal
  • Cyclophosphamide
  • Azathioprine
  • Prednisone