Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Data on adults IgA vasculitis (IgAV) are lacking. This survey was designed to better define clinical spectrum and efficacy of treatments in this population.
Methods: We analyzed data from 260 patients with IgAV included in a French multicenter retrospective IGAVAS survey.
Results: Mean age at diagnosis was 50.1±18 years, and 63% of patients were male. At baseline, manifestations included purpura (100%), arthralgia or arthritis (62%), glomerulonephritis (70%) or gastro-intestinal involvement (53%). Thirty percent of the patients showed renal failure (eGFR <60ml/min/1.73m2). Median eGFR in patients with renal involvement was 88 mL/min/1.73m2 (Q1-Q3: 55; 103), median proteinuria level 1.5 g/day (Q1-Q3: 0.6; 3), and hematuria was noted in 88%. Median serum IgA level was 3.6 g/L (Q1-Q3: 2.7; 4.8) and 85/159 patients (53%) presented with elevated IgA levels. Skin biopsy demonstrated leukocytoclastic vasculitis in 205 patients (92%). Direct immunofluorescence revealed IgA and complement deposition in dermis blood vessels in 174/216 (81%) and 47/222 (21%) of patients, respectively. Renal biopsy demonstrated mesangial IgA deposits in 142/144 patients (99%) and extracapillary proliferation in 59/143 patients (41%). Data concerning therapeutic efficacy was available and analyzed in 127 patients. In univariate analysis, global response (complete or partial) was achieved in 80% (64/80) in patients treated with corticosteroids (CS) alone compared to 77% (23/30) in patients treated with CS and cyclophosphamide (CYC) (p=0.17). Multivariate analysis using inverse weighting on propensity score revealed that patients treated with CS plus CYC had higher response rate than patients treated with CS alone [OR (95% CI) 2.33 (1.29-4.18), p=0.005]. Sensivity analysis excluding outliers confirmed this result [OR 1.79 (1.00-3.20), p=0.049]. Conversely, full multivariate model without propensity score did not demonstrate a benefit of CS plus CYC compared to CS alone [OR 0.88 (0.29-2.67), p=0.82], or after adjustment on propensity score [OR 0.90 (0.29-2.78), p=0.86]. After median follow-up of 17.2 months (Q1-Q3: 9.1-38.3) corresponding to 593 patient-years, 8 patients died, including 3 deaths directly related to IgAV (2 mesenteric ischemia and 1 multivisceral failure). Eight patients experienced end-stage renal failure treated by renal transplantation (n=2) or dialysis (n=6). Among patients who received a treatment, 15 experienced minor relapse (14%) and 9 (8%) major relapse during the first 12 months after treatment.
Conclusion: In conclusion, this series provides interesting data on clinical and histological presentation and therapeutic efficacy, suggesting that CS alone appears to be a reasonable first-line therapy in patients with systemic IgAV, while the benefit of adding CYC to CS remains uncertain.
To cite this abstract in AMA style:Audemard-Verger A, Pillebout E, Dechartres A, Chanal J, Amoura Z, Le Gouellec N, Cacoub P, Jourde-Chiche N, Urbanski G, Augusto JF, Moulis G, Raffray L, Deroux A, Hummel A, Lioger B, Catroux M, Faguer S, Goutte J, Martis N, Maurier F, Riviere E, Sanges S, Baldolli A, Costedoat-Chalumeau N, Roriz M, Puéchal X, Andre M, Lavigne C, Bienvenu B, Mékinian A, Zagdoun E, Girard C, Berezne A, Guillevin L, Thervet E, Terrier B. Characteristics and Management of IgA Vasculitis (Henoch-Schönlein purpura) in Adults: Data from the 260 Patients Included in the Igavas Survey [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/characteristics-and-management-of-iga-vasculitis-henoch-schonlein-purpura-in-adults-data-from-the-260-patients-included-in-the-igavas-survey/. Accessed October 28, 2020.
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