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Abstract Number: 1792

Clinical-Biological and Pathological Spectrum and Outcome of IgA Vasculitis in Adults: A French Study

Alexandra Audemard1, Evangeline Pillebout2, Patrice Cacoub3, Noémie Jourde-Chiche Sr.4, Zahir Amoura5, Noemie Le Gouellec6, Francois Maurier7, Boris Bienvenu8, Geoffrey Urbanski9, Sébastien Sanges10, Aurélie Hummel11, Alban Deroux12, Loic Raffray13, Luc Mouthon14, Loïc Guillevin for the French Vasculitis Study Group14, Eric Thervet15 and Benjamin Terrier16, 1Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France, 2Nephrology, Saint Louis, Paris, France, 3Groupe Hospitalier Pitié Salpétrière, Service de Médecine Interne, DHU i2B, Paris, France, 4Nephrology, CHU, Marseille, France, 5Internal medicine 2, French National Reference Center for Systemic Lupus and Antiphospholipid Syndrome, Pitié-Salpêtrière Hospital (AP-HP), Paris, France, 6Internal Medicine, Lille, France, 7HP Metz Belle Isle Hospital, Department of Internal Medicine, Metz, France, 8Médecine interne, CHU Côte de Nacre, CAEN, France, 9Internal Medicine, CHRU, Lille, France, 10Service de médecine interne, Centre National de Référence de la Sclérodermie Systémique, Hôpital Claude Huriez, CHRU Lille, Lille, France, 11Nephrology, Necker Hospital, Paris, France, 12Internal Medicine, CHU Grenoble, Grenoble, France, 13INTERNAL MEDICINE, bordeaux, France, 14National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, 15Nephrology, Hopital Européen Georges Pompidou, APHP, PARIS, France, 16National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: Arthritis, glomerulonephritis and skin, Henoch-Schönlein purpura

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Session Information

Session Title: Vasculitis

Session Type: Abstract Submissions (ACR)

Background/Purpose

IgA vasculitis is an immune-complex small-vessel vasculitis that mainly affects children and, more rarely, adults, in whom it seems to be more severe, because of gastrointestinal and renal involvements. Data on therapeutic management are lacking. The IGAVAS study was designed to describe IgA-vasculitis presentation and evaluate treatment efficacies. Here, we characterize its presentation and outcome. 

Methods

French centers in university and general hospitals retrospectively included 235 patients with IgA vasculitis. We collected information on clinical-biological presentation and outcomes, particularly renal involvement, and analyzed the features associated with renal involvement from diagnosis through the last follow-up visit, and renal insufficiency, defined as estimated glomerular filtration rate (eGFR) using MDRD <60 ml/min/1.73 m2.

Results

235 patients (143 men and 92 women, mean age 50±19 years) were enrolled. At disease onset, the most common manifestations were: purpura in all patients with skin necrosis (28%), glomerular nephropathy (64%), arthralgias/arthritis (63%) and gastrointestinal involvement (53%), mainly abdominal pain and intestinal bleeding; 34% had constitutional symptoms. Mean serum IgA level was 3.9±1.8 g/L, with only 37% exceeding the upper the limit of normal (>3.5 g/L). Among the 150 patients with renal involvement, mean eGFR was 80±34 ml/min/1.73 m², including 19% with eGFR <60 mL/min/1.73 m2. Mean proteinuria was 2.56 g/24 h and 85% had microscopic hematuria. IgA vasculitis was always histologically confirmed, mainly in skin and kidney biopsies. The most frequent histological findings in the skin were leukocytoclastic vasculitis (92%), IgA deposits (81%) and fibrinoid necrosis (28%). In the kidney, IgA deposits were seen in 98%, and endocapillary and extracapillary glomerulonephritis in 51% and 42%, respectively. Among the 76% of the patients treated, 82% received corticosteroids alone, with immunosuppressants combined for 18%. After median follow-up of 31 months, 98% achieved initial renal remission but 23% relapsed. Six patients died, 3 of vasculitis flares. Among patients with renal involvement, mean eGFR at last follow-up was 81±31 ml/min/1.73 m2, including 27% with eGFR <60 mL/min/1.73 m2. Three patients required dialysis and 1 had kidney transplantation. Older age, tubulointerstitial lesions on kidney biopsy and baseline eGFR were associated with a poor renal prognosis.

Conclusion

This large population enabled description of the clinical-biological presentation and outcome of IgA vasculitis in adults, and showed high frequencies of renal and gastrointestinal involvements and relapse rates. The analysis of the different therapeutic strategies is ongoing.


Disclosure:

A. Audemard,
None;

E. Pillebout,
None;

P. Cacoub,

Astra Zeneca, Bayer, Boehringer Ingelheim, Gilead, Glaxo Smith Kline, Janssen, Merck Sharp Dohme, Roche, Servier, Vifor.,

5;

N. Jourde-Chiche Sr.,
None;

Z. Amoura,
None;

N. Le Gouellec,
None;

F. Maurier,
None;

B. Bienvenu,
None;

G. Urbanski,
None;

S. Sanges,
None;

A. Hummel,
None;

A. Deroux,
None;

L. Raffray,
None;

L. Mouthon,
None;

L. Guillevin for the French Vasculitis Study Group,
None;

E. Thervet,
None;

B. Terrier,
None.

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