A case report of hypocomplementemic urticarial vasculitis presenting with membranoproliferative glomerulonephritis
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CASE REPORT
Open Access
A case report of hypocomplementemic urticarial vasculitis presenting with membranoproliferative glomerulonephritis Kalliopi Vallianou1, Chrysanthi Skalioti1, George Liapis2, John N. Boletis1* and Smaragdi Marinaki1
Abstract Background: Hypocomplementemic urticarial vasculitis syndrome is an infrequent condition characterized by ocular, renal, gastrointestinal and pulmonary involvement with low serum complement levels and autoantibodies. Renal manifestations vary from microscopic hematuria to nephrotic syndrome and acute kidney injury. Accordingly differing histologic patterns have been reported. Case presentation: We present the case of a 65 years old woman with a history of chronic uveitis who presented with arthralgias, urticarial rush, nephrotic syndrome, glomerular hematuria and low serum complement. Kidney biopsy revealed an immune-complex membranoproliferative glomerulonephritis. The patient received induction therapy with steroids, cyclophosphamide and hydroxychloroquine followed by rapid clinical improvement and remission of proteinuria. Maintenance treatment consisted of rituximab pulses. Conclusions: The majority of hypocomplementemic urticarial vasculitis syndrome cases is idiopathic, although an association to drugs, infections or other autoimmune disorders has been recorded. Given the rarity and heterogeneity of the disease, no standard treatment is established. Keywords: Complement, Case report, Hypocomplementemic urticarial vasculitis syndrome, Renal disease, Vasculitis
Background Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare condition characterised by consistent urticarial lesions with histologic findings of leukocytoclastic vasculitis, as well as low serum complement levels and usual ocular, renal, gastrointestinal and pulmonary involvement. It represents the most severe of three distinct syndromes of the urticarial vasculitis (UV) spectrum. Normocomplementemic urticarial vasculitis (NUV) is a benign cutaneous vasculitis and hypocomplementemic urticarial vasculitis (HUV) is a low complement vasculitis also limited to the skin. HUVS, that is * Correspondence: [email protected] 1 Department of Nephrology and Renal Transplantation Unit, Faculty of Medicine, Laiko Hospital, National and Kapodistrian University of Athens, Athens, Greece Full list of author information is available at the end of the article
characterized by systemic involvement and usually circulating anti-C1q antibodies, was first described by McDuffie et al. in 1973 and the diagnostic criteria were defined in 1982 by Schwarz et al. Urticarial vasculitis is a rare disorder, with an incidence of 0,5/100.000persons, with only 1–2% of them, usually middle-aged women, developping the complete syndrome [1, 2]. It comprises a type III hypersensitivity reaction mediated by immune complex deposits on capillaries and postcapillary venules. Although most cases are idiopathic, the syndrome may be secondary to drugs, infections or other autoimmune disorders [3]. Given the limited number of cases and
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