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Autoimmun Rev. 2014 Mar;13(3):313-8. doi: 10.1016/j.autrev.2013.11.003. Epub 2013 Nov 10.

Are anti-proteinase-3 ANCA a useful marker of granulomatosis with polyangiitis (Wegener's) relapses? Results of a retrospective study on 126 patients.

Author information

1
Department of Internal Medicine, French Vasculitis Study Group (FVSG), Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris, France.
2
Département de Biostatistique et Informatique Médicale, Hopital Saint-Louis, Université Paris Diderot, Paris, France.
3
Department of Internal Medicine, French Vasculitis Study Group (FVSG), Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris, France. Electronic address: loic.guillevin@cch.aphp.fr.

Abstract

OBJECTIVES:

Predicting granulomatosis with polyangiitis (Wegener's) (GPA) relapses based on ANCA titers remains a source of debate. Our objective was to evaluate the relevance of monitoring PR3-ANCA titers for GPA management.

METHODS:

This retrospective study included 126 patients fulfilling the 1990 ACR criteria for GPA and PR3-ANCA-positive at the time of diagnosis. Disease activity was assessed with BVAS/WG and Disease Extent Index. For each patient, a median of 12 serum samples was analyzed, i.e., one every 5.5months.

RESULTS:

Induction therapy obtained remission in 88% of the patients. ANCA became negative by IF for 70/115 (60.9%) patients and by ELISA for 90/115 (78.3%). After median follow-up of 70months, 85/126 (67.5%) patients had 154 clinical relapses associated with cANCA and PR3-ANCA-positivity for 122 (79.2%) and 102 (66.2%) of them, respectively. Relapse-free survival was significantly longer for patients who remained PR3-ANCA-negative (HR 0.60 [95% CI 0.39-0.92], P=0.02). Individual ANCA-profile analysis revealed that, for 60% of GPA patients, clinical outcomes and ANCA-titer changes were closely associated, i.e., ANCA were always positive during relapses and negative during remission. The 35 patients with fluctuating ANCA-positivity during remission were in partial remission or had developed grumbling GPA.

CONCLUSION:

Although ANCA were positive during most systemic relapses or residual disease, no strict clinical-immunological correspondence was observed for 25% of the patients. Thus, GPA management cannot be based on ANCA levels alone.

KEYWORDS:

ANCA; Disease activity; Granulomatosis with polyangiitis (Wegener's); Monitoring; Proteinase 3; Relapse

PMID:
24225075
DOI:
10.1016/j.autrev.2013.11.003
[Indexed for MEDLINE]
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