CLINICAL COURSE AND SYMPTOMATIC PREDIAGNOSTIC PERIOD OF PATIENTS WITHWEGENERS GRANULOMATOSIS AND MICROSCOPIC POLYANGIITIS

The clinical course of 15 patients with Wegener's granulomatosis (WG) and eight patients with microscopic polyangiitis (MPA) from one nephro logical clinical center is presented for the period from 1984 to 1993, when testing for antineutrophil cytoplasmic antibodies (ANCA) was gra dually introduced into routine clinical practice. We found a high degr ee of prolonged time periods with symptoms attributable to WG or MPA u ntil the specific diagnosis was made. Nine patients with WG and one pa tient with MPA had symptomatic prediagnostic periods of more than thre e years, which extended in one case up to twenty years. In these predi agnostic periods, often even severe flares of vasculitic activity resu lted in spontaneous remission without immunosuppressive therapy. One p atient on chronic dialysis for four months because of rapidly progress ive glomerulonephritis, experienced sufficient spontaneous regain of r esidual renal function to stay of dialysis for 6 years. Despite a high amount of spontaneous recovery, recurrent flares of disease eventuall y led to death in those cases without sufficient immunosuppressive the rapy. Contrary to long courses of disease, one patient with WG had a f ulminate exacerbation of disease with lethal hemoptysis after a predia gnostic period of only three months. Renal disease, respiratory and ot her symptoms did not occur sequentially, but each could precede the ot her, We conclude in agreement with published former experience, that W G and MPA show a highly variable spontaneous disease course, that requ ires extended observational periods for evaluating maintenance therapi es.