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Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report
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, o...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9936762/ https://www.ncbi.nlm.nih.gov/pubmed/36817292 http://dx.doi.org/10.1159/000525942 |
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author | Mehta, Jiten Prakash Jang, Charley Qi Hua Fahim, Peter Nguyen, Minhtri Khac Zuckerman, Jonathan Mamita, Rosha Kamgar, Mohammad |
author_facet | Mehta, Jiten Prakash Jang, Charley Qi Hua Fahim, Peter Nguyen, Minhtri Khac Zuckerman, Jonathan Mamita, Rosha Kamgar, Mohammad |
author_sort | Mehta, Jiten Prakash |
collection | PubMed |
description | 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 present 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. |
format | Online Article Text |
id | pubmed-9936762 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
spelling | pubmed-99367622023-02-18 Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report Mehta, Jiten Prakash Jang, Charley Qi Hua Fahim, Peter Nguyen, Minhtri Khac Zuckerman, Jonathan Mamita, Rosha Kamgar, Mohammad Glomerular Dis Case Report 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 present 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. S. Karger AG 2022-07-19 /pmc/articles/PMC9936762/ /pubmed/36817292 http://dx.doi.org/10.1159/000525942 Text en Copyright © 2022 by The Author(s). Published by S. Karger AG, Basel https://creativecommons.org/licenses/by-nc/4.0/This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. |
spellingShingle | Case Report Mehta, Jiten Prakash Jang, Charley Qi Hua Fahim, Peter Nguyen, Minhtri Khac Zuckerman, Jonathan Mamita, Rosha Kamgar, Mohammad Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report |
title | Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report |
title_full | Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report |
title_fullStr | Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report |
title_full_unstemmed | Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report |
title_short | Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report |
title_sort | hypocomplementemic urticarial vasculitis syndrome masquerading as systemic lupus erythematosus: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9936762/ https://www.ncbi.nlm.nih.gov/pubmed/36817292 http://dx.doi.org/10.1159/000525942 |
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