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Multicenter Study
. 2022 Feb 16;23(1):67.
doi: 10.1186/s12882-022-02689-8.

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis

Alice Corthier  1 , Marie Jachiet  2 , Daniel Bertin  3 , Aude Servais  4 , Christelle Barbet  5 , Adrien Bigot  6 , Marie-Sylvie Doutre  7 , Didier Bessis  8 , Ancuta Bouffandeau  9 , Olivier Moranne  10   11 , Pierre-André Jarrot  12   13 , Nathalie Bardin  3   13 , Benjamin Terrier  14   15 , Stephane Burtey  1   13 , Xavier Puéchal  14   15 , Laurent Daniel  13   16 , Noémie Jourde-Chiche  17   18
Affiliations
Multicenter Study

Biopsy-proven kidney involvement in hypocomplementemic urticarial vasculitis

Alice Corthier et al. BMC Nephrol. .

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.

Keywords: Anti-C1q antibody; C1q deposits; Glomerulonephritis; Hypocomplementemic urticarial vasculitis; McDuffie syndrome; Renal biopsy; Renal vasculitis.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Representative kidney pathological lesions observed in patients with Hypocomplementemic Urticarial Vasculitis from the present cohort. A Vasculitis with fibrinoid necrosis involving a pre-glomerular arteriole. Masson’s trichrome, × 200. B Global endocapillary proliferation with an interstitium containing a slight peri-venular inflammatory infiltrate. Masson’s trichrome, × 200. C Interstitial nephritis with mononuclear cells, oedema, and suffering tubules. Masson’s trichrome, × 200. D Abundant deposits of C1q within arteriole wall, capsule, and tubular basement membrane. Immunofluorescence, × 200
Fig. 2
Fig. 2
Treatment of the kidney involvement of HUV (first, second and third line therapies). A In the 12 patients from the present cohort. B In the 26 patients from the literature
Fig. 3
Fig. 3
Treatment and renal outcome in 3 patients representative of the present cohort (patients #1, #2 and #10)
See this image and copyright information in PMC

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