Background/Purpose: Data on granulomatosis with polyangiitis (GPA) sustained remission off-therapy (SROT) are limited and it is unknown whether disease characteristics or treatment regimen may affect it. This study aimed to assess SROT of GPA patients from the French Vasculitis Study Group registry, and identify factors associated with its occurrence and durability during follow-up.

Methods: GPA had to satisfy the 1990 ACR classification criteria and/or revised Chapel Hill Nomenclature for study inclusion. SROT was defined as remission (BVAS=0) without glucocorticoids (GC) or immunosuppressants (IS), the latter for ≥6 months (ie 2 consecutive visits). SROT and its duration were extracted from the database. Data from patients with 3-, 5- and 10-year SROT were analyzed. Baseline characteristics of patients with 3-year GPA SROT were compared to those of registry GPA patients with available data at 3 years but not in SROT (controls), and 3-year SROT achieving 5-year SROT vs those who relapsed between 3 & 5 years. Patients with 3-year GPA SROT follow-up +7 years were analyzed according to maintained SROT or not.

Results: Among 795 database patients with new-onset GPA, 259 achieved at least 1 SROT at some time during their disease, after a median [IQR] of 36 [28–63] months post-diagnosis. The first SROT lasted a median of 14 [I8-32] months. Among 202 of those patients who had follow-up, 73 (36%) remained in SROT for a median follow-up of 34 [14-45] months post-SROT. Among 434 (54%) patients followed for ≥3 years post-diagnosis, 82% had received GC and cyclophosphamide induction therapy. At 3 years post-diagnosis, 92 (21%) patients in SROT were compared to 342 (79%) controls who had relapsed or were still taking GC or IS. Patients achieving 3-year SROT vs controls, respectively, had more frequently received intravenous cyclophosphamide as induction therapy (89% vs 77%, P=0.01), with a higher median number of infusions (7.5 vs 6; P=0.05); no other clinical or biological baseline difference was found. Among those 92 3-year SROT patients, 74 had ≥2 years of additional follow-up: 46 (62%) attained 5-year SROT and 28 (38%) had relapsed after a mean follow-up of 13 months. Baseline clinical and biological characteristics of patients achieving 5-year SROT did not differ from those of 3-year SROT patients who relapsed. Among those 92 3-year SROT patients, 16 had ≥7 additional years of follow-up: 6 (38%) achieved 10-year SROT, ie 8% of 75 GPA with available data at 10 years, and 10 (63%) had relapsed a mean 35 ± 28 months after achieving 3-year SROT.

Conclusion: Only 8% of GPA patients achieved 10-year SROT after conventional induction and maintenance therapies. No baseline clinical or biological characteristics helped distinguish patients achieving or maintaining SROT and those who relapsed. However, patients achieving 3-year SROT had received more intensive induction therapy than those who relapsed or were still on GC or IS at 3 years.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/granulomatosis-with-polyangiitis-sustained-remission-off-therapy-data-from-the-french-vasculitis-study-group-registry/