CLASSICAL VERSUS NONRENAL WEGENERS GRANULOMATOSIS

We investigated whether 'limited' or 'non-renal' Wegener's granulomato sis (WG) differs from classical or 'renal' WG. Renal WG is characteriz ed by necrotizing granulomatosis of the upper and or lower respiratory tract, accompanied by systemic vasculitis and focal segmental necroti zing glomerulonephritis. This last feature is absent in nonrenal WG. I n a prospective follow-up study of all identified cases presenting to a single teaching hospital, we reviewed 22 patients with non-renal WG, and compared their presentation and outcome with that of 28 patients with renal WG. Clinical and laboratory assessment of disease activity, frequency of death, relapse and end-stage renal disease were assessed . The two groups differed in clinical presentation, laboratory feature s and outcome. The group with non-renal WG had less cutaneous and pulm onary disease; the haemoglobin, white cell count and platelet count te nded to be normal. Residual mortality was confined to the renal group. However, the groups shared many features, particularly their requirem ent for immunosuppressive therapy, since WG causes major tissue destru ction regardless of whether it is a localized or widespread process. A t the immunopathological level, the two groups appear to be part of a single disease spectrum. Importantly, the non-renal WG group may chang e the pattern of their disease to involve the kidney. Long-term follow -up of such patients is therefore essential.