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Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report
Jiten Prakash Mehta1, Charley Qi Hua Jang2, Peter Fahim1
1Division of Nephrology, Department of Medicine, University of California, Los Angeles, California, USA.
Glomerular Diseases
|February 23, 2023
Summary
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare immune condition that overlaps with systemic lupus erythematosus (SLE). Kidney biopsy aided in diagnosing HUVS in a patient initially thought to have SLE.
Area of Science:
- Rheumatology
- Immunology
- Nephrology
Background:
- Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare immune complex-mediated condition.
- HUVS shares overlapping features with systemic lupus erythematosus (SLE), complicating differential diagnosis.
- Established criteria for distinguishing HUVS from SLE are lacking, despite existing diagnostic guidelines.
Observation:
- A 40-year-old female with a history of SLE presented with odynophagia, rash, and acute kidney injury (AKI).
- Following AKI resolution, persistent hematuria and proteinuria prompted a kidney biopsy.
- The kidney biopsy was crucial in diagnosing HUVS in this patient.
Findings:
- The case highlights the diagnostic challenges in differentiating HUVS from SLE due to overlapping clinical and serological features.
- Kidney biopsy proved essential in confirming the HUVS diagnosis when clinical presentation was ambiguous.
- HUVS can present with severe systemic manifestations, including renal involvement.
Implications:
- Further case accumulation is needed to refine diagnostic modalities for HUVS.
- Improved diagnostic criteria are necessary to differentiate HUVS from SLE effectively.
- Understanding HUVS is critical for appropriate management, especially given potential for severe organ involvement and mortality.
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