Late Onset of IgA Vasculitis Is Associated with More Severe Renal Involvement

Alexandra Audemard-Verger¹, Aurélie Baldolli², Noemie Le Gouellec³, Loic Raffray⁴, Alban Deroux⁵, Julie Goutte⁶, Bertrand Lioger⁷, Zahir Amoura⁸, Patrice Cacoub⁹, Sébastien Sanges¹⁰, Evangeline Pillebout¹¹, Loïc Guillevin for the French Vasculitis Study Group¹² and Benjamin Terrier¹³, ¹Internal Medicine, Caen, France, ²Internal Medicine, CHU, Caen, France, ³Internal Medicine, Lille, France, ⁴Internal Medicine, CHU de Bordeaux, Bordeaux, France, ⁵Internal Medicine, CHU de Grenoble, Grenoble, France, ⁶Internal Medicine, Saint Etienne, France, ⁷GICC UMR 7292, University François Rabelais, Tours, France, ⁸Department 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, ⁹Internal Medicine Department, University Hospital “Pitié-Salpêtrière”, “Pierre et Marie Curie Paris VI” University, Paris, France, ¹⁰Université Lille Nord de France, Faculté de Médecine Henri Warembourg, Lille, Lille, France, ¹¹Nephrology, Saint Louis, Paris, France, ¹²Service de Médecine Interne, Centre de Référence Maladies Auto-Immunes et Auto-Inflammatoires Systémiques Rares, Hôpital Cochin, Paris, France, ¹³Internal Medicine, Cochin University Hospital, Paris, France

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Aging, Henoch-Schönlein purpura, outcomes and vasculitis

Session Information

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.

Disclosure: A. Audemard-Verger, None; A. Baldolli, None; N. Le Gouellec, None; L. Raffray, None; A. Deroux, None; J. Goutte, None; B. Lioger, None; Z. Amoura, None; P. Cacoub, None; S. Sanges, None; E. Pillebout, None; L. Guillevin for the French Vasculitis Study Group, None; B. Terrier, None.

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 2, 2020.

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