A Case of Hypocomplementemic Urticarial Vasculitis Syndrome With Severe Renal and Gastrointestinal Involvement

Lorena Jost; Birgit M Helmchen; Michael Osthoff; Luzia Nigg Calanca

doi:10.7759/cureus.72113

A Case of Hypocomplementemic Urticarial Vasculitis Syndrome With Severe Renal and Gastrointestinal Involvement

Cureus. 2024 Oct 22;16(10):e72113. doi: 10.7759/cureus.72113. eCollection 2024 Oct.

Authors

Lorena Jost¹, Birgit M Helmchen², Michael Osthoff¹, Luzia Nigg Calanca³

Affiliations

¹ Department of Internal Medicine, Kantonsspital Winterthur, Winterthur, CHE.
² Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich, CHE.
³ Department of Nephrology, Kantonsspital Winterthur, Winterthur, CHE.

Abstract

We present a severe case of hypocomplementemic urticarial vasculitis syndrome (HUVS) and its diagnostic and therapeutic challenges. A 56-year-old male presenting with fever and impaired kidney function was diagnosed with HUVS. Before the initiated treatment was effective, he developed severe colon ischemia, and a subtotal colectomy was required. We discuss other affected organs, such as kidneys, lungs, the heart, and the skin. Pathophysiology is briefly reviewed and the difficulty of overlapping autoimmune diseases is discussed. Treatment continues to be challenging, as there is no consensus about the optimal immunosuppressive therapy.

Keywords: anti-c1q antibody; colon ischemia; huvs; hypocomplementemic urticarial vasculitis; hypocomplementemic urticarial vasculitis syndrome; impaired kidney function; low complement.

Publication types

Case Reports