Are antineutrophil cytoplasmic antibodies a marker predictive of relapse in Wegener's granulomatosis? A prospective study

Objectives. To investigate the predictive value of testing for antineutrophil cytoplasmic antibodies (ANCA) in 55 patients with systemic Wegener's granulomatosis (WG) included in a randomized, prospective trial comparing corticosteroids and oral or pulse cyclophosphamide. Methods. All 55 patien...

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Published in:Rheumatology (Oxford, England) England), 2001-02, Vol.40 (2), p.147-151
Main Authors: Girard, T., Mahr, A., Noël, L.‐H., Cordier, J.‐F., Lesavre, P., André, M.‐H., Guillevin, L.
Format: Article
Language:English
Subjects:
Antibodies, Antineutrophil Cytoplasmic - blood
Antineutrophil cytoplasmic antibodies
Biological and medical sciences
Corticosteroids
Cyclophosphamide
Female
Granulomatosis with Polyangiitis - blood
Granulomatosis with Polyangiitis - drug therapy
Granulomatosis with Polyangiitis - immunology
Humans
Male
Medical sciences
Middle Aged
Predictive Value of Tests
Prognosis
Prospective Studies
Recurrence
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Wegener's granulomatosis
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Summary:Objectives. To investigate the predictive value of testing for antineutrophil cytoplasmic antibodies (ANCA) in 55 patients with systemic Wegener's granulomatosis (WG) included in a randomized, prospective trial comparing corticosteroids and oral or pulse cyclophosphamide. Methods. All 55 patients received corticosteroids. A cyclophosphamide pulse of 0.7 g/m2 was given at the time of diagnosis. After the first pulse, the patients were assigned at random to receive either pulse or oral cyclophosphamide (2 mg/kg/day), independently of ANCA results. ANCA were sought using an immunofluorescence assay and an attempt was made to correlate them with relapse of WG. ANCA were monitored throughout the study. Results. At the time of diagnosis, ANCA were detected in 48 (87%) patients, with a cytoplasmic labelling pattern in 44 and a perinuclear pattern in four. ANCA follow‐up was available for 50 patients. ANCA disappeared in 34 patients and persisted in nine. For 79% of the patients, the clinical course improved with the disappearance of ANCA and deteriorated with their persistence or increased titre. Among the patients who were initially ANCA‐positive, 23 relapses occurred. Relapses were more frequent when ANCA remained positive or reappeared [13/19 ANCA‐positive patients vs 3/29 ANCA‐negative patients (P
ISSN:1462-0324
1462-0332
DOI:10.1093/rheumatology/40.2.147