Lan-Huong Thai1, Pierre Charles1, Matthieu Resche-Rigon2, Kristell Desseaux2, Loïc Guillevin3. 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.
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 GPApatients, 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.
Authors: Justyna Fijolek; E Wiatr; V Petroniec; E Augustynowicz-Kopec; M Bednarek; D Gawryluk; K Roszkowski-Sliz Journal: Clin Rheumatol Date: 2019-07-23 Impact factor: 2.980
Authors: Michael J Kemna; Jan Damoiseaux; Jos Austen; Bjorn Winkens; Jim Peters; Pieter van Paassen; Jan Willem Cohen Tervaert Journal: J Am Soc Nephrol Date: 2014-10-16 Impact factor: 10.121
Authors: Lynn A Fussner; Amber M Hummel; Darrell R Schroeder; Francisco Silva; Rodrigo Cartin-Ceba; Melissa R Snyder; Gary S Hoffman; Cees G M Kallenberg; Carol A Langford; Peter A Merkel; Paul A Monach; Philip Seo; Robert F Spiera; E William St Clair; Nadia K Tchao; John H Stone; Ulrich Specks Journal: Arthritis Rheumatol Date: 2016-07 Impact factor: 10.995