Abstract
Hypocomplementemic urticarial vasculitis syndrome (HUVS), or McDuffie syndrome, is a rare small vessel vasculitis associated with urticaria, hypocomplementemia and positivity of anti-C1q antibodies. In rare cases, HUVS can manifest as an immune-complex mediated glomerulonephritis with a membranoproliferative pattern of injury. Due to the rarity of this disorder, little is known about the clinical manifestation, pathogenesis, treatment response and outcome of such patients. We describe here three cases of HUVS with severe renal involvement. These patients had a rapidly progressive form of glomerulonephritis with severe nephrotic syndrome against a background of a membranoproliferative pattern of glomerular injury with extensive crescent formation. Therefore, these patients required aggressive induction and maintenance immunosuppressive therapy, with a clinical and renal response in two patients, while the third patient progressed to end-stage renal disease. Because of the rarity of this condition, there are few data regarding the clinical presentation, pathology and outcome of such patients. Accordingly, we provide an extensive literature review of cases reported from 1976 until 2020 and place them in the context of the current knowledge of HUVS pathogenesis. We identified 60 patients with HUVS and renal involvement that had adequate clinical data reported, out of which 52 patients underwent a percutaneous kidney biopsy. The most frequent renal manifestation was hematuria associated with proteinuria (70% of patients), while one third had abnormal kidney function on presentation (estimated glomerular filtration (GFR) below 60 mL/min/1.73 m2). The most frequent glomerular pattern of injury was membranoproliferative (35%), followed by mesangioproliferative (21%) and membranous (19%). Similar to other systemic vasculitis, renal involvement carries a poorer prognosis, but the outcome can be improved by aggressive immunosuppressive treatment.
Highlights
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune hypocomplementemic small vessels vasculitis, affecting skin, joints, eyes, lungs and kidneys, first described by McDuffie et al, (1973) [1,2,3,4]
HUVS was defined based on histopathology as “anti-C1q vasculitis” and classified as “small vessels vasculitis” accompanied by urticaria, hypocomplementemia and anti-C1q antibodies [5]
Anti-C1q antibodies and complement consumption are clearly involved, HUVS pathogenesis is unclear and what triggers these abnormalities is a matter of debate [6,7]
Summary
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune hypocomplementemic small vessels vasculitis, affecting skin, joints, eyes, lungs and kidneys, first described by McDuffie et al, (1973) [1,2,3,4]. Because HUVS is a rare condition and kidney biopsy was not consistently performed, kidney involvement was reported with variable frequency (14–50%), the histopathology and clinical pattern as well as outcome of kidney involvement are unclear [2,3,8]. We report three cases of hypocomplementemic urticarial vasculitis syndrome with renal involvement; we undertook a literature search (1976–2020), focusing on cases for which individual data on renal involvement were available, aiming to better profile the histopathology and clinical presentation as well as the outcome of kidney involvement in HUVS
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