Dataset Information

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis.

ABSTRACT:

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.

SUBMITTER: Corthier A

PROVIDER: S-EPMC8851735 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature

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Publications

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis.

Corthier Alice A Jachiet Marie M Bertin Daniel D Servais Aude A Barbet Christelle C Bigot Adrien A Doutre Marie-Sylvie MS Bessis Didier D Bouffandeau Ancuta A Moranne Olivier O Jarrot Pierre-André PA Bardin Nathalie N Terrier Benjamin B Burtey Stephane S Puéchal Xavier X Daniel Laurent L Jourde-Chiche Noémie N

BMC nephrology 20220216 1

<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>T ...[more]

PMID: 35172758

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Dataset Information

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis.

Background

Methods

Results

Conclusion

Publications

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis.

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