Néphropathie associée à une vascularite urticarienne hypocomplémentémique : présentation d'un cas clinique et revue de la littérature

Abstract : Hypocomplementemic Urticarial Vasculitis (HUV) 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 a tubulo-interstitial nephritis. HUV diagnosis was established after identifying anti-C1q antibodies. The patient was treated with Hydroxychloroquine and low-dose steroids, enabling a clinical and biological recovery. Of the 82 cases in the literature describing HUV 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 thirties. The most frequent renal manifestations were haematuria (60%), and proteinuria (52%). Kidney failure was rarely observed (22%), with a fairly good renal prognosis. HUV was associated with a systemic disease in 11 (13%) patients. In the absence of recommendations, the treatment strategy remains to be defined.
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Annabel Boyer. Néphropathie associée à une vascularite urticarienne hypocomplémentémique : présentation d'un cas clinique et revue de la littérature. Médecine humaine et pathologie. 2018. ⟨dumas-02101028⟩

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