Abstract
BackgroundHypocomplementemic 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.MethodsAll 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.ResultsTwelve 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.ConclusionRenal 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.
Highlights
Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis, called “McDuffie syndrome” or “anti-C1q vasculitis”
We describe here the clinical presentation, the pathological lesions and the therapeutic management of a multicentre cohort of 12 patients with biopsy-proven kidney involvement associated with HUV, identified through the French Vasculitis Study Group (FVSG) network
anti-neutrophil cytoplasmic antibodies (ANCA) antibodies were detected in 6 patients at some point during the follow-up, among whom 3 had antimyeloperoxidase (MPO) antibodies, 1 anti-proteinase 3 (PR3) antibodies, and 2 no antigenic specificity
Summary
Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis, called “McDuffie syndrome” or “anti-C1q vasculitis”. It was classified among immune complex small vessel vasculitides in the revised international Chapel Hill consensus conference in 2012 [1]. It was first described by McDuffie et al in 1973 and well defined in 1982 by Schwartz et al [2, 3]. We describe here the clinical presentation, the pathological lesions and the therapeutic management of a multicentre cohort of 12 patients with biopsy-proven kidney involvement associated with HUV, identified through the French Vasculitis Study Group (FVSG) network. We aimed to describe the kidney involvement of HUV in a multicenter national cohort with an extended follow-up
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