Hypocomplementaemic urticarial vasculitis syndrome: a mimicker of systemic lupus erythematosus

Roy, Kaustuv; Talukdar, Arunansu; Kumar, B N Yathindra; Sarkar, Sumanta

doi:10.1136/bcr-2013-009082

Cited by 10 publications

(5 citation statements)

References 6 publications

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“…The CSU definition was based on international consensus [9] . Diseases related to urticaria/angioedema were excluded, particularly urticaria vasculitis and hypocomplementemic urticaria vasculitis syndrome [21][22][23] , since these abnormalities are associated with localized, nonblanching, painful lesions [23] which persist for >24 h and leave brownish residues when healed [22] .…”

Section: Discussionmentioning

confidence: 99%

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Ferriani

Silva

Pereira

et al. 2015

Int Arch Allergy Immunol

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Background: Data regarding the prevalence of chronic spontaneous urticaria (CSU) in childhood-onset systemic lupus erythematosus (cSLE) patients and possible associated factors are limited to a few case reports. The objectives of this study were to assess CSU in a large cSLE population, in order to evaluate the demographic data, clinical manifestations, disease activity/damage, laboratory abnormalities and treatment. Methods: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services and included 852 cSLE patients. CSU was diagnosed according to the guidelines of the European Academy of Allergy and Clinical Immunology, the Global Allergy and Asthma European Network, the European Dermatology Forum and the World Allergy Organization. Patients with CSU (evaluated at urticaria diagnosis) and patients without CSU (evaluated at the last visit) were assessed for lupus clinical/laboratory features and treatment. Results: CSU was observed in 10/852 cSLE patients (1.17%). The median of cSLE duration at urticaria diagnosis was 0 (-3 to 5) years. Comparison of cSLE patients with and without CSU revealed a greater frequency of constitutional symptoms (40 vs. 8%, p = 0.006), reticuloendothelial system involvement (30 vs. 3%, p = 0.003), mucocutaneous (90 vs. 28%, p < 0.0001) and musculoskeletal manifestations (50 vs. 6%, p < 0.0001) and methylprednisolone pulse therapy use (60 vs. 9%, p < 0.0001) in the former group. The frequency of immunosuppressive treatment was lower in patients with CSU (p = 0.018). The median SLE Disease Activity Index 2000 (12 vs. 2, p < 0.0001) and erythrocyte sedimentation rate (40 vs. 19 mm/1st hour, p = 0.024), was higher in patients with CSU. Conclusions: To our knowledge, this is the first study with evidence that CSU may be linked to cSLE. We also demonstrated that this particular skin manifestation occurs predominantly at disease onset and is associated with lupus moderate/high disease activity without major organ involvement.

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Section: Discussionmentioning

confidence: 99%

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Ferriani

Silva

Pereira

et al. 2015

Int Arch Allergy Immunol

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“…This case meets both major diagnostic criteria of HUVS, chronic urticaria over 6 months and hypocomplementaemia, and minor criteria including arthralgia, uveitis and C1q antibodies. Other important differential diagnoses excluded were Churg‐Strauss syndrome, Schnitzler syndrome and granulomatosis with polyangiitis.…”

Section: Select Investigation Results For This Patient With Hypocomplmentioning

confidence: 72%

“…Thirty percent of patients with HUVS have ocular inflammation in the form of uveitis, episcleritis and conjunctivitis . Other ocular findings include oedematous eyelids, anterior cervical lymphadenopathy, subconjunctival haemorrhage, bilateral vitreous haemorrhage and subretinal fluid in a case, and bilateral recurrent iridocyclitis …”

Section: Select Investigation Results For This Patient With Hypocomplmentioning

confidence: 98%

Hypocomplementaemic urticarial vasculitis syndrome presenting as unilateral dacryoadenitis

Liong

Salonga-Reyes

Morris

2019

Clinical Exper Ophthalmology

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“…Misdiagnosis of HUVS occurs because of similarities to SLE. Arthritis and arthralgia are common to both, urticarial vasculitis occurs in 5-10% of patient with systemic lupus and may be a presenting manifestation, and 28% of 47% of patient with SLE have IgG antibody to C1q [6,15,16]. The complement abnormalities are identical in both diseases as well.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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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.

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Hypocomplementaemic urticarial vasculitis syndrome: a mimicker of systemic lupus erythematosus

Cited by 10 publications

References 6 publications

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Hypocomplementaemic urticarial vasculitis syndrome presenting as unilateral dacryoadenitis

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

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