[Nephropathy associated with hypocomplementemic urticarial vasculitis: A case report and literature review].

Nephrol Ther

Centre universitaire des maladies rénales, CHU de Caen, avenue de la côte de Nacre, 14033 Caen cedex 9, France; Unicaen, UFR de médecine, Normandie Université, 2, rue des Rochambelles, 14032 Caen cedex, France. Electronic address:

Published: March 2020


Category Ranking

98%

Total Visits

921

Avg Visit Duration

2 minutes

Citations

20

Article Abstract

Hypocomplementemic urticarial vasculitis is a rare systemic vasculitis, affecting small vessels, characterised by chronicle urticaria, hypocomplementemia, and systemic manifestations. Renal involvement, whose prevalence varies between 9% and 60%, is mainly glomerular. We here report the case of a 59 years old woman presenting kidney failure, associated with chronicle urticaria and arthralgias. Laboratory investigation showed haematuria, proteinuria, hypocomplementemia and anti-SSa antibody positivity. A percutaneous kidney biopsy revealed focal and segmental glomerulonephritis associated with an acute interstitial nephritis. Hypocomplementemic urticarial vasculitis diagnosis was established after identifying anti-C1q antibodies. The lack of a dry syndrome, the negativity of a Schirmer test and the lack of sialadenitis on a salivary gland biopsy excluded an associated Gougerot-Sjögren Syndrome. The patient was treated with hydroxychloroquine and low-dose steroids, enabling a clinical and biological recovery. Of the 82 cases in the literature describing hypocomplementemic urticarial vasculitis associated nephropathies, 72 (88%) were a glomerular impairment, most frequently secondary to membranoproliferative glomerulonephritis. Only 6 (7%) tubulo-interstitial nephritis have been reported, 4 of them being associated with a glomerulonephritis. Patients were more likely to be women, aged in their third decade. The most frequent renal manifestations were haematuria (60%), and proteinuria (52%). Kidney failure was rarely observed (22%), with a fairly good renal prognosis. Hypocomplementemic urticarial vasculitis was associated with a systemic disease in 11 (13%) patients. In the absence of recommendations, the treatment strategy remains to be defined.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nephro.2019.09.004DOI Listing

Publication Analysis

Top Keywords

hypocomplementemic urticarial
20
urticarial vasculitis
20
chronicle urticaria
8
kidney failure
8
vasculitis associated
8
vasculitis
6
associated
6
hypocomplementemic
5
urticarial
5
[nephropathy associated
4

Similar Publications

Background: Hypocomplementemic urticarial vasculitis (HUV) syndrome is a rare form of small-vessel vasculitis characterized by a heterogeneous spectrum of clinical and biological findings. It is typically marked by chronic urticarial eruptions, hypocomplementemia and histopathological evidence of leukocytoclastic vasculitis. It may also involve multiple organ systems, with frequent articular, gastrointestinal, renal, and other systemic manifestations.

View Article and Find Full Text PDF

Background: Chronic urticarial lesions, a common condition of mostly unknown cause, can occur in immune dysregulation disorders such as hypocomplementemic urticarial vasculitis syndrome (HUVS), neutrophilic urticarial dermatosis (NUD), and systemic autoinflammatory diseases (SAIDs), including Schnitzler syndrome.

Objective: This study aimed to identify the molecular basis of non-pruritic chronic urticarial eruptions in four unrelated sporadic patients initially diagnosed with neonatal NUD, Schnitzler syndrome, HUVS or giant cell arteritis.

Methods: Conventional next-generation sequencing (NGS) of leukocyte DNA was supplemented with high-depth NGS (>1500X) to improve detection of low-level mosaicism in SAID-related genes.

View Article and Find Full Text PDF

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare, severe form of urticarial vasculitis. It is characterized by persistent hypocomplementemia, chronic urticarial vasculitic lesions, and severe multiorgan involvement. Herein, we present long-term follow-up of two siblings diagnosed with HUVS at early ages, who were found to have DNASE1L3 mutations, and their subsequent 20-year follow-up.

View Article and Find Full Text PDF

Current therapeutic options for adult patients with urticarial vasculitis: A scoping review.

J Am Acad Dermatol

August 2025

Vasculitis Clinic, Department of Medicine, Sacre-Coeur Hospital, Université de Montréal, Montréal, Quebec, Canada.

Background: Urticarial vasculitis (UV) is a rare form of small vessel vasculitis, and there are limited published data on its management.

Objective: This study aims to review the current therapeutic options for UV.

Methods: A PubMed search was conducted, selecting articles published from 2000 to January 2024.

View Article and Find Full Text PDF

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterized by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. Systemic involvement can either be present at the onset of disease or develop later. Here, we present a rare case of a 22-year-old female, who initially presented with generalized rash and was eventually diagnosed with HUVS.

View Article and Find Full Text PDF