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Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome-A Case-Based Review.
Oana Ion1, Bogdan Obrișcă1,2, Gener Ismail1,2
1Department of Nephrology, Fundeni Clinical Institute, 022328 Bucharest, Romania.
Journal of Clinical Medicine
|July 10, 2020
Summary
Hypocomplementemic urticarial vasculitis syndrome (HUVS) can cause severe kidney disease. Aggressive immunosuppressive therapy improved outcomes for most patients with HUVS-related glomerulonephritis, though some progressed to end-stage renal disease.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Hypocomplementemic urticarial vasculitis syndrome (HUVS), also known as McDuffie syndrome, is a rare condition.
- It is characterized by urticaria, low complement levels, and anti-C1q antibodies.
- HUVS can rarely present as immune-complex mediated glomerulonephritis.
Purpose of the Study:
- To describe three cases of HUVS with severe renal involvement.
- To review and analyze reported cases of HUVS with renal disease from 1976-2020.
- To understand the clinical presentation, pathogenesis, and outcomes of HUVS-related kidney disease.
Main Methods:
- Case series of three patients with HUVS and severe glomerulonephritis.
- Systematic literature review of HUVS cases with renal involvement.
- Analysis of clinical data and kidney biopsy findings from 60 reported patients.
Main Results:
- The most common renal manifestations were hematuria and proteinuria (70%).
- Membranoproliferative glomerulonephritis was the most frequent biopsy finding (35%).
- Two of three severe cases showed response to immunosuppressive therapy; one progressed to end-stage renal disease.
Conclusions:
- Severe renal involvement in HUVS, often membranoproliferative glomerulonephritis, requires aggressive immunosuppression.
- Early diagnosis and treatment may improve renal outcomes in HUVS patients.
- Further research is needed to fully elucidate HUVS pathogenesis and optimize treatment strategies.
Keywords:
anti-C1q antibodiescrescentic glomerulonephritishypocomplementemic urticarial vasculitis syndrome (HUVS)kidney involvementnephrotic syndromeMore Related Videos
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