Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review
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 membranoprol...
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MDPI AG
2020-07-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/9/7/2131 |
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| author | Oana Ion Bogdan Obrișcă Gener Ismail Bogdan Sorohan Sonia Bălănică Gabriel Mircescu Ioanel Sinescu |
| author_facet | Oana Ion Bogdan Obrișcă Gener Ismail Bogdan Sorohan Sonia Bălănică Gabriel Mircescu Ioanel Sinescu |
| author_sort | Oana Ion |
| collection | DOAJ |
| description | 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 m<sup>2</sup>). 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. |
| first_indexed | 2024-03-10T18:38:54Z |
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| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-10T18:38:54Z |
| publishDate | 2020-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-d13706677ef5483eb49af6157d5818092023-11-20T06:00:43ZengMDPI AGJournal of Clinical Medicine2077-03832020-07-0197213110.3390/jcm9072131Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based ReviewOana Ion0Bogdan Obrișcă1Gener Ismail2Bogdan Sorohan3Sonia Bălănică4Gabriel Mircescu5Ioanel Sinescu6Department of Nephrology, Fundeni Clinical Institute, 022328 Bucharest, RomaniaDepartment of Nephrology, Fundeni Clinical Institute, 022328 Bucharest, RomaniaDepartment of Nephrology, Fundeni Clinical Institute, 022328 Bucharest, RomaniaDepartment of Nephrology, Fundeni Clinical Institute, 022328 Bucharest, RomaniaDepartment of Nephrology, Fundeni Clinical Institute, 022328 Bucharest, RomaniaDepartment of Uronephrology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, RomaniaDepartment of Uronephrology, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, RomaniaHypocomplementemic 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 m<sup>2</sup>). 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.https://www.mdpi.com/2077-0383/9/7/2131hypocomplementemic urticarial vasculitis syndrome (HUVS)kidney involvementcrescentic glomerulonephritisnephrotic syndromeanti-C1q antibodies |
| spellingShingle | Oana Ion Bogdan Obrișcă Gener Ismail Bogdan Sorohan Sonia Bălănică Gabriel Mircescu Ioanel Sinescu Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review Journal of Clinical Medicine hypocomplementemic urticarial vasculitis syndrome (HUVS) kidney involvement crescentic glomerulonephritis nephrotic syndrome anti-C1q antibodies |
| title | Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review |
| title_full | Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review |
| title_fullStr | Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review |
| title_full_unstemmed | Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review |
| title_short | Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review |
| title_sort | kidney involvement in hypocomplementemic urticarial vasculitis syndrome a case based review |
| topic | hypocomplementemic urticarial vasculitis syndrome (HUVS) kidney involvement crescentic glomerulonephritis nephrotic syndrome anti-C1q antibodies |
| url | https://www.mdpi.com/2077-0383/9/7/2131 |
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