Hypocomplementemic Urticarial Vasculitic Syndrome A Rare Case of Renal Failure

Introduction Hypocomplementemic urticarial vasculitic syndrome (HUVS) is a rare autoimmune disorder characterized by chronic urticarial vasculitis, low complement levels, and multi-organ involvement. This condition often presents diagnostic challenges due to its overlapping features with systemic lupus erythematosus (SLE) and other autoimmune diseases. In a recently published case study, a 66-year-old man developed severe renal failure as a complication of HUVS, requiring intensive care.

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A Rare and Severe Case of HUVS This case report describes a 66-year-old patient presenting with abdominal pain, rash, confusion, oliguria, and respiratory distress. Laboratory findings revealed acute renal failure with leukocytosis, elevated lactate, and hypocomplementemia. A skin biopsy confirmed leukocytoclastic vasculitis, and the patient was diagnosed with HUVS. Notably, he had not yet exhibited signs of SLE, which frequently coexists with HUVS.

Key Clinical Findings:

• Symptoms: Chronic urticarial rash, abdominal pain, shortness of breath, confusion, oliguria.
• Laboratory Results: Elevated white blood cell count, high lactate, low complement levels (C3 and C4), and proteinuria.
• Diagnosis Confirmation: Skin biopsy confirming leukocytoclastic vasculitis, fulfilling diagnostic criteria for HUVS.
• Treatment & Outcome: High-dose steroids and hemodialysis facilitated partial recovery; however, relapse occurred two months later, requiring further immunosuppressive therapy.

Broader Implications of HUVS and Treatment Approaches HUVS remains an interdisciplinary challenge due to its complex presentation and potential for multi-organ involvement. The American College of Rheumatology (ACR) underscores the importance of early recognition and appropriate immunosuppressive therapy to manage autoimmune vasculitic syndromes effectively.

Management Strategies:

• First-line treatment: High-dose steroids
• Immunosuppressants (methotrexate, azathioprine, mycophenolate mofetil) for refractory cases
• Plasmapheresis in severe relapses

Further Research and Clinical Awareness This case highlights the importance of considering HUVS in patients presenting with chronic urticaria and renal dysfunction. As the condition is often associated with SLE, long-term monitoring is crucial to detect evolving autoimmune complications.

Read the full study at https://doi.com/10.29328/journal.jcn.1001017.

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