Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis.

Area of Science:

• Nephrology
• Rheumatology
• Autoimmunology

Background:

• Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder.
• HUVS can involve multiple organ systems, including the kidneys, and is associated with hypocomplementemia.

Observation:

• A 31-year-old woman presented with nonspecific symptoms and acute renal failure.
• Laboratory findings included hematuria, nephrotic proteinuria, elevated creatinine, and low C1q levels.
• Kidney biopsy revealed proliferative glomerulonephritis with crescent formation, indicative of severe kidney injury.

Findings:

• The patient experienced end-stage kidney disease requiring dialysis.
• Treatment involved cyclophosphamide, rituximab, mycophenolate mofetil, and glucocorticoids.
• A delayed but excellent recovery of renal function was observed after 12 months of dialysis.

Implications:

• This case underscores the potential for significant renal recovery in HUVS with crescentic glomerulonephritis.
• It adds to the limited literature on this rare condition, emphasizing the importance of aggressive immunosuppressive therapy.
• The successful outcome suggests that timely and appropriate treatment can lead to favorable long-term renal outcomes in HUVS patients.