Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report

Jiten Prakash Mehta; Charley Qi Hua Jang; Peter Fahim; Minhtri Khac Nguyen; Jonathan Zuckerman; Rosha Mamita; Mohammad Kamgar

doi:10.1159/000525942

Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report

Glomerular Dis. 2022 Jul 19;2(4):189-193. doi: 10.1159/000525942. eCollection 2022.

Authors

Jiten Prakash Mehta¹, Charley Qi Hua Jang², Peter Fahim¹, Minhtri Khac Nguyen¹, Jonathan Zuckerman³, Rosha Mamita⁴, Mohammad Kamgar¹

Affiliations

¹ Division of Nephrology, Department of Medicine, University of California, Los Angeles, California, USA.
² David Geffen School of Medicine, University of California, Los Angeles, California, USA.
³ Department of Pathology and Laboratory Medicine, University of California, Los Angeles, California, USA.
⁴ Department of Psychology, University of California, Los Angeles, California, USA.

Abstract

Introduction: Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an infrequent immune complex-mediated condition characterized by nonpruritic urticarial lesions, low serum complement levels, and autoantibodies, associated with systemic manifestations like arthralgia/arthritis, angioedema, ocular inflammation with conjunctivitis, episcleritis, uveitis, renal, gastrointestinal, and pulmonary involvement. HUVS and systemic lupus erythematosus (SLE) overlap and the criteria for identifying HUVS as an entity distinct from SLE are lacking. Despite the diagnostic criteria established by Schwartz et al. [Curr Opin Rheumatol. 2014;26(5):502-9], differentiation from SLE is sometimes difficult as patients often also fulfill the classification criteria of the American College of Rheumatology (ACR). The prognosis of HUVS depends on the organ system involved. Lung disease results in significant morbidity and mortality and is made worse by smoking. Kidney involvement with glomerulonephritis may ultimately result in end-stage renal disease with the need for kidney transplant. Death may also occur due to acute laryngeal edema.

Case presentation: We pre-sent a case of a 40-year-old female who had a diagnosis of SLE, presented with severe odynophagia, was found to have an erythematous macular rash, and had acute kidney injury attributed to contrast-related injury and cardiorenal syndrome. After the resolution of the AKI, she continued to have hematuria and low-grade proteinuria that led to a kidney biopsy that aided in the diagnosis of HUVS.

Discussion/conclusion: Given the rarity of this disease and the difficulty in differentiating HUVS from other rheumatological diseases such as SLE, further accumulation of cases is necessary to understand the best diagnostic modality for this entity.

Keywords: Electron microscopy; Glomerulonephritis; Hypocomplementemic urticarial vasculitis syndrome; Rare disease; Urticarial vasculitis.

Publication types

Case Reports

Grants and funding

No funding sources were used during this case report by any of the authors.