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Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis.

Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Research Abstract Details 

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  • Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Abstract Text:

    The clinical course of 15 patients with Wegener's granulomatosis (WG) and eight patients with microscopic polyangiitis (MPA) from one nephrological clinical center is presented for the period from 1984 to 1993, when testing for antineutrophil cytoplasmic antibodies (ANCA) was gradually introduced into routine clinical practice. We found a high degree of prolonged time periods with symptoms attributable to WG or MPA until the specific diagnosis was made. Nine patients with WG and one patient with MPA had symptomatic prediagnostic periods of more than three years, which extended in one case up to twenty years. In these prediagnostic periods, often even severe flares of vasculitic activity resulted in spontaneous remission without immunosuppressive therapy. One patient on chronic dialysis for four months because of rapidly progressive glomerulonephritis, experienced sufficient spontaneous regain of residual renal function to stay off dialysis for 6 years. Despite a high amount of spontaneous recovery, recurrent flares of disease eventually led to death in those cases without sufficient immunosuppressive therapy. Contrary to long courses of disease, one patient with WG had a fulminate exacerbation of disease with lethal hemoptysis after a prediagnostic period of only three months. Renal disease, respiratory and other symptoms did not occur sequentially, but each could precede the other. We conclude in agreement with published former experience, that WG and MPA show a highly variable spontaneous disease course, that requires extended observational periods for evaluating maintenance therapies.

    Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Publishing Authors By Initials

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    PUBMED ID PMID:

    MEDLINE DATE:

    Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Journal Published:

    PUBLICATION TYPE: Journal Article

    Journal: Renal failure

    VOLUME: 20

    Page Numbers: 519-32

    Journal Abbreviation: Ren Fail

    ISSN: 0886-022X

    DAY: 21

    MONTH: May

    YEAR: 1998

    Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Information

    Number of References:

    LANGUAGE: eng

    NlmUniqueID: 8701128

    Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Keywords Mesh Terms:

    KEYWORDS: Wegener Granulomatosis

    MESH TERMS: therapy

    Chemical & Substance for Abstract: Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis. Information

    Substance Name: Cyclophosphamide

    Registry Number: 50-18-0

    Grant and Affiliation Information for Clinical course and symptomatic prediagnostic period of patients with Wegener's granulomatosis and microscopic polyangiitis.

    AFFILIATION: Medical Department A, Hospital of Ludwigshafen, Academic Teaching Center of the Johannes-Gutenberg University Mainz, Bremserstrabe, Germany.

    Country: UNITED STATES

    UNITED STATES Research PublicationUNITED STATES Research Publication

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    MEDLINETA: Ren Fail

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