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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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-biological-and-pathological-spectrum-and-outcome-of-iga-vasculitis-in-adults-a-french-study/