Date: Tuesday, November 7, 2017
Session Title: Vasculitis Poster III: Other Vasculitis Syndromes
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Immunoglobulin A vasculitis (IgAV), formerly called Henoch–Schönlein purpura, is a small-vessel vasculitis characterized by immune-complex deposits with predominant IgA. Although the disease is often benign in children, adult IgAV has been described as being more severe. Moreover, renal involvement represents the main cause of morbidity and mortality in adults. This study was undertaken to determine whether age could impact IgAV initial manifestations and outcomes.
Methods: In this nationwide retrospective study, data from 260 IgAV patients were analyzed to describe their first symptoms and outcomes according to age at IgAV onset: 90 (35%) >60 years (late-onset) and 170 (65%) <60 years.
Results: Mean±SD age at IgAV diagnosis for the entire cohort was 50.1±18 years and 63% were male. Baseline manifestations included: purpura (100%), renal involvement (70%), arthralgias or arthritis (62%), gastrointestinal involvement (53%) and renal failure (30%), defined as estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2. Patients with late-onset IgAV, compared to those <60 years old, respectively, had more frequent renal involvement (89% vs. 60%; P<0.0001), more frequent microscopic hematuria (78% vs. 53%; P=0.0001); and more severe disease at diagnosis, with a higher renal failure rate (44% vs. 10%; P<0.0001) and higher urine protein excretion (1.20 vs 0.45 g/day; P=0.002). Only tubulointerstitial lesions in renal biopsies were more frequent in patients >60 years (44% vs. 21%; P=0.003). Median serum IgA was significantly higher for patients >60 years than those <60 years (4.4 vs. 3.3 g/L; P=0.0007). Therapeutic management was similar for both groups: glucocorticoids (70% vs. 60%; P=0.20) and cyclophosphamide (23% vs. 24%; P=0.85). Renal failure at the end of follow-up was more frequent for late-onset patients (42% vs. 13%; P<0.0001) and their vasculitis-related mortality was higher (4% vs. 0%; P=0.02). However, percentage±SD eGFR variation (deltaGFR) and annual DeltaGFR variation were similar for patients >60 and <60 years, respectively: 0.05±5.2% vs. 14.2±8.9% (P=0.27) and –2.2±10.9% vs. 11.1±8.3% (P=0.34).
Conclusion: Late onset of IgAV is associated with more frequent and severe renal involvement, and more frequent tubulointerstitial lesions. Comorbidities and physiological age might be involved.
To cite this abstract in AMA style:Audemard-Verger A, Baldolli A, Le Gouellec N, Raffray L, Deroux A, Goutte J, Lioger B, Amoura Z, Cacoub P, Sanges S, Pillebout E, Guillevin for the French Vasculitis Study Group L, Terrier B. Late Onset of IgA Vasculitis Is Associated with More Severe Renal Involvement [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/late-onset-of-iga-vasculitis-is-associated-with-more-severe-renal-involvement/. Accessed June 1, 2023.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/late-onset-of-iga-vasculitis-is-associated-with-more-severe-renal-involvement/