Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis - Inserm - Institut national de la santé et de la recherche médicale Access content directly
Journal Articles BMC Nephrology Year : 2022

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis

Daniel Bertin
Marie-Sylvie Doutre
  • Function : Author


Background: Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis. We aimed to describe the kidney involvement of HUV in a multicenter national cohort with an extended follow-up. Methods: All patients with HUV (international Schwartz criteria) with a biopsy-proven kidney involvement, identified through a survey of the French Vasculitis Study Group (FVSG), were included. A systematic literature review on kidney involvement of HUV was performed. Results: Twelve patients were included, among whom 8 had positive anti-C1q antibodies. All presented with proteinuria, from mild to nephrotic, and 8 displayed acute kidney injury (AKI), requiring temporary haemodialysis in 2. Kidney biopsy showed membrano-proliferative glomerulonephritis (MPGN) in 8 patients, pauci-immune crescentic GN or necrotizing vasculitis in 3 patients (with a mild to severe interstitial inflammation), and an isolated interstitial nephritis in 1 patient. C1q deposits were observed in the glomeruli (n = 6), tubules (n = 4) or renal arterioles (n = 3) of 8 patients. All patients received corticosteroids, and 9 were also treated with immunosuppressants or apheresis. After a mean follow-up of 8.9 years, 6 patients had a preserved renal function, but 2 patients had developed stage 3-4 chronic kidney disease (CKD) and 4 patients had reached end-stage kidney disease (ESKD), among whom 1 had received a kidney transplant. Conclusion: Renal involvement of HUV can be responsible for severe AKI, CKD and ESRD. It is not always associated with circulating anti-C1q antibodies. Kidney biopsy shows mostly MPGN or crescentic GN, with frequent C1q deposits in the glomeruli, tubules or arterioles.
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Dates and versions

inserm-03690745 , version 1 (08-06-2022)



Alice Corthier, Marie Jachiet, Daniel Bertin, Aude Servais, Christelle Barbet, et al.. Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis. BMC Nephrology, 2022, 23 (1), pp.67. ⟨10.1186/s12882-022-02689-8⟩. ⟨inserm-03690745⟩
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