Kesarwani Vartika, Phachu Deep, Trivedi Ruchir
Internal Medicine, University of Connecticut, Farmington, USA.
Department of Nephrology, University of Connecticut, Farmington, USA.
Cureus. 2022 Mar 23;14(3):e23429. doi: 10.7759/cureus.23429. eCollection 2022 Mar.
Hypocomplementemia urticarial vasculitis syndrome (HUVS) is a rare form of systemic vasculitis which is characterized by the presence of urticaria and hypocomplementemia. The presence of recurrent and chronic urticarial rash is the dominant clinical finding in HUVS. Other manifestations including angioedema, arthritis, gastrointestinal symptoms, ocular inflammation, pulmonary involvement, renal involvement, and central nervous system involvement are also seen. Although the pathophysiology of HUVS is yet to be fully understood, it has been demonstrated that immune complex-mediated injury is the predominant mechanism responsible for severe systemic manifestations; a mechanism of injury similar to systemic lupus erythematosus (SLE). HUVS shared many clinicopathological features with SLE and it is prudent to question whether HUVS is a separate disease entity or SLE in evolution. Herein we present a case of a male patient who was diagnosed with SLE a year after being diagnosed with HUVS.
低补体血症性荨麻疹性血管炎综合征（HUVS）是一种罕见的系统性血管炎，其特征为存在荨麻疹和低补体血症。反复出现的慢性荨麻疹皮疹是HUVS的主要临床发现。还可见其他表现，包括血管性水肿、关节炎、胃肠道症状、眼部炎症、肺部受累、肾脏受累和中枢神经系统受累。尽管HUVS的病理生理学尚未完全阐明，但已证明免疫复合物介导的损伤是导致严重全身表现的主要机制；这一损伤机制与系统性红斑狼疮（SLE）相似。HUVS与SLE有许多临床病理特征相同，因此谨慎质疑HUVS是否为一个独立的疾病实体或处于演变中的SLE是很有必要的。在此，我们报告一例男性患者，其在被诊断为HUVS一年后又被诊断为SLE。
