title: Clinical and immunological follow-up of patients with severe renal disease in Wegener's granulomatosis.
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.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal; D001379:Azathioprine / Q000627:therapeutic use; D001706:Biopsy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005455:Fluorescent Antibody Technique; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000188:drug therapy / Q000276:immunology* / Q000401:mortality / Q000473:pathology; D006801:Humans; D007111:Immunity, Cellular; D007668:Kidney / Q000276:immunology / Q000473:pathology; D007674:Kidney Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009846:Oliguria / Q000209:etiology / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use; D013601:T-Lymphocytes / Q000145:classification
chemical_list: D000911:Antibodies, Monoclonal; D003520:Cyclophosphamide; D001379:Azathioprine; D011241:Prednisone