Characteristics and Management of IgA Vasculitis (Henoch-Schönlein purpura) in Adults: Data from the 260 Patients Included in the Igavas Survey

Alexandra Audemard-Verger¹, Evangeline Pillebout², Agnès Dechartres³, Johan Chanal⁴, Zahir Amoura⁵, Noemie Le Gouellec⁶, Patrice Cacoub⁷, Noémie Jourde-Chiche⁸, Geoffroy Urbanski⁹, Jean-Francois Augusto⁹, Guillaume Moulis¹⁰, Loic Raffray¹¹, Alban Deroux¹², Aurélie Hummel¹³, Bertrand Lioger¹⁴, Melanie Catroux¹⁵, Stanislas Faguer¹⁶, Julie Goutte¹⁷, Nihal Martis¹⁸, Francois Maurier¹⁹, Etienne Riviere²⁰, Sébastien Sanges²¹, Aurélie Baldolli²², Nathalie Costedoat-Chalumeau²³, Melanie Roriz²⁴, Xavier Puéchal²⁵, Marc Andre²⁶, Christian Lavigne²⁷, Boris Bienvenu²⁸, Arsène Mékinian²⁹, Elie Zagdoun³⁰, Charlotte Girard³¹, Alice Berezne³², Loïc Guillevin²⁵, Eric Thervet³³ and Benjamin Terrier³⁴, ¹Internal Medicine, Caen, France, ²Nephrology, Saint Louis, Paris, France, ³Epidemiology, Hotel Dieu, Paris, France, ⁴Dermatology, Cochin Hospital, Paris, France, ⁵Department of Internal Medicine 2. Referal center for SLE/APS, Hôpital Pitié-Salpêtrière, AP-HP, UPMC Univ Paris 06 & French National Reference Center For Systemic Lupus and Antiphospholipid Syndrome, Paris, France, ⁶Internal Medicine, Lille, France, ⁷Assistance Publique-Hôpitaux de Paris (AP-HP), Groupe Hospitalier Pitié-Salpêtrière, Département de Médecine Interne et d’Immunologie clinique, DHU i2B, Inflammation, Immunopathologie, Biothérapie, Université Pierre et Marie Curie, Paris 6, Paris, France, Paris, France, ⁸Vascular Research Center of Marseille, Aix-Marseille Univ., Vascular Research Center of Marseille, Marseille, France, ⁹Internal Medicine, CHU, Angers, France, ¹⁰CHU Purpan, Toulouse, France, ¹¹Internal Medicine, CHU de Bordeaux, Bordeaux, France, ¹²Internal Medicine, CHU Grenoble, Grenoble, France, ¹³Necker, paris, France, ¹⁴GICC UMR 7292, University François Rabelais, Tours, France, ¹⁵Internal Medicine, Cochin Hospital, Paris, France, ¹⁶Nephrology, CHU, Toulouse, France, ¹⁷Internal Medicine, CHU, Paris, France, ¹⁸Internal Medicine, CHU, Nice, France, ¹⁹Department of Internal Medicine, HP Metz Belle Isle Hospital, Metz, France, ²⁰Internal Medicine, CHU, Bordeaux, France, ²¹Université Lille Nord de France, Faculté de Médecine Henri Warembourg, Lille, Lille, France, ²²Internal Medicine, CHU, Caen, France, ²³Internal Medicine, Cochin University Hospital, Paris, France, ²⁴internal Medicine, Lariboisière, Paris, France, ²⁵Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, Paris, France, ²⁶Internal Medicine CHU G Montpied, Internal Medicine, Clermont Ferrand, France, ²⁷CHU Angers, department of Internal Medicine, Angers, France, ²⁸Caen University Hospital, Caen, France, ²⁹Service de médecine interne. Hôpital Saint-Antoine., Paris, France, ³⁰Internal Medicine, CH, Saint-Lo, France, ³¹Internal Medicine, CHU, Lyon, France, ³²Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, France, ³³Nephrology, Hopital Européen Georges Pompidou, APHP, PARIS, France, ³⁴National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Colchicine, cyclophosphamide, glucocorticoids and vasculitis, Henoch-Schönlein purpura

Session Information

Date: Tuesday, November 15, 2016

Title: Vasculitis - Poster III: Rarer Vasculitides

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.

Disclosure: A. Audemard-Verger, None; E. Pillebout, None; A. Dechartres, None; J. Chanal, None; Z. Amoura, None; N. Le Gouellec, None; P. Cacoub, None; N. Jourde-Chiche, None; G. Urbanski, None; J. F. Augusto, None; G. Moulis, None; L. Raffray, None; A. Deroux, None; A. Hummel, None; B. Lioger, None; M. Catroux, None; S. Faguer, None; J. Goutte, None; N. Martis, None; F. Maurier, None; E. Riviere, None; S. Sanges, None; A. Baldolli, None; N. Costedoat-Chalumeau, Biogen Idec, 2,UCB, 2; M. Roriz, None; X. Puéchal, None; M. Andre, None; C. Lavigne, None; B. Bienvenu, None; A. Mékinian, None; E. Zagdoun, None; C. Girard, None; A. Berezne, None; L. Guillevin, None; E. Thervet, None; B. Terrier, None.

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 .

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