Jost Lorena, Helmchen Birgit M, Osthoff Michael, Nigg Calanca Luzia
Department of Internal Medicine, Kantonsspital Winterthur, Winterthur, CHE.
Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich, CHE.
Cureus. 2024 Oct 22;16(10):e72113. doi: 10.7759/cureus.72113. eCollection 2024 Oct.
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.
我们报告了一例严重的低补体血症性荨麻疹性血管炎综合征（HUVS）病例及其诊断和治疗挑战。一名56岁男性，出现发热和肾功能损害，被诊断为HUVS。在开始的治疗有效之前，他发生了严重的结肠缺血，需要进行次全结肠切除术。我们讨论了其他受影响的器官，如肾脏、肺、心脏和皮肤。简要回顾了病理生理学，并讨论了自身免疫性疾病重叠的难点。由于对于最佳免疫抑制治疗尚无共识，治疗仍然具有挑战性。
