Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

Her, Minyoung; Song, Jooyeon; Kim, Dong Yook

doi:10.3346/jkms.2009.24.1.184

Cited by 21 publications

(19 citation statements)

References 8 publications

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“…According to a retrospective study by Davis et al, 24 out of 132 patients with urticarial vasculitis had hypocomplementemia, 54% of whom developed SLE [20]. Many case reports since have presented patients with both diagnoses of SLE and HUVS [5,7,20,21]. Although understanding of the genetics and pathophysiology of HUVS and SLE is far from complete, providers should be aware of the dual diagnoses to provide appropriate treatment for both conditions.…”

Section: Discussionmentioning

confidence: 99%

“…Hypocomplementemic urticarial vasculitic syndrome (HUVS) is a rare disorder characterized by chronic urticarial vasculitis and hypocomplementemia [1]. First described in 1973 by McDuf ie et al [2], the etiology of HUVS remains controversial [3][4][5][6][7][8]. HUVS is triggered by an unknown event that leads to immune complex deposition in small blood vessels and activation of the complement pathway.…”

Section: Introductionmentioning

confidence: 99%

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A case report of Hypocomplementemic urticarial vasculitic syndrome presenting with Renal failure

Hopkins¹,

Gibbs²,

Griffiths³

et al. 2018

J Clini Nephrol

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We present a case of hypocomplementemic urticarial vasculitic syndrome (HUVS) who developed severe renal failure requiring ICU-level care. Our patient is a 66-year-old man who presented with abdominal pain, rash, confusion, oliguria, and shortness of breath. He was found to be in acute renal failure with leukocytosis and elevated lactate. Work-up for infectious, autoimmune, and hematologic malignant diseases was negative. The presence of chronic urticaria, abdominal pain, hypocomplementemia, and leukocytoclastic vasculitis on skin biopsy confi rmed the diagnosis of HUVS. He required hemodialysis for renal failure as well as gastrostomy tube placement for nutritional support secondary to the development of mucosal ulcers, a rare fi nding in HUVS. He recovered with several months of high-dose steroids and hemodialysis. This case highlights the effectiveness of steroids for initial treatment of HUVS, and the relapsing and remitting nature of the disease. Providers should also be aware of the broad range of presenting symptoms such as mucosal lesions that may require nutritional support. Interestingly, unlike many previously reported cases of HUVS, our patient had not yet developed signs and symptoms of systemic lupus erythematosus, which often overlaps with HUVS.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A case report of Hypocomplementemic urticarial vasculitic syndrome presenting with Renal failure

Hopkins¹,

Gibbs²,

Griffiths³

et al. 2018

J Clini Nephrol

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“…Some patients with HUVS have characteristic features of connective tissue disease, particularly SLE or Sjögren syndrome [2,30]. Clinical and serologic fi ndings distinguish these two entities (Table 1).…”

Section: Clinical Serologic and Histologic Featuresmentioning

confidence: 95%

Hypocomplementemic urticarial vasculitis syndrome

Jara

Navarro²,

Medina³

et al. 2009

Curr Rheumatol Rep

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Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon immune complex-mediated entity characterized by urticaria with persistent acquired hypocomplementemia. First described in 1973, HUVS is associated with several systemic findings including leukocytoclastic vasculitis, severe angioedema, laryngeal edema, pulmonary involvement, arthritis, arthralgia, glomerulonephritis, and uveitis. These manifestations should be present for at least 6 months. Laboratory findings include low complement levels of classical pathway, namely C1q, C2, C3, and C4. The disease marker is the serum presence of anti-C1q antibodies. Treatment, based on disease severity, involves corticosteroids and other immunosuppressive agents that have demonstrated some success. Patients may have significant morbidity and mortality, most commonly caused by chronic obstructive pulmonary disease and acute laryngeal edema.

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“…A dose of 20-30 mg/day is necessary, 273,282 and steroid pulse therapy was reportedly necessary for conditions complicated by SLE. 283,284 For patients who show serious systemic symptoms or GC resistance, the concomitant use of CyA, AZA or CY can be an option 285-287 (recommendation class: IIb, evidence level: C). In addition, plasma exchange, 288 IVIG, 289 and RTX, an anti-CD20 antibody preparation, 287 have also been reported (recommendation class: IIb, evidence level: C).…”

Section: ▋ 52 Seroimmunological Examinationsmentioning

confidence: 99%

JCS 2017 Guideline on Management of Vasculitis Syndrome　― Digest Version ―

Isobe

Amano

Arimura

et al. 2020

Circ J

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AAV ANCA-associated vasculitis ACR American College of Rheumatology ADA adalimumab ANCA anti-neutrophil cytoplasmic antibody AZA azathioprine bDMARDs biologic disease-modifying anti-rheumatic drugs c-ANCA cytoplasmic ANCA CG cryoglobulin CHCC Chapel Hill Consensus Conference Cr creatinine CT computed tomography CRP C-reactive protein CyA cyclosporine CV cryoglobulinemic vasculitis CY cyclophosphamide DMARDs disease-modifying anti-rheumatic drugs * a The aorta and its primary branches corresond to the aorta (ascending, arch, thoracic descending, abdominal descending), primary branches of the aorta (including the coronary artery), and pulmonary artery. * b Multiple lesions are defined as those that involve two or more of the above arteries or sites or two or more segments of the aorta. * c Hypertrophic lesions are detected by ultrasonography (macaroni sign of the common carotid artery), contrast-enhanced CT, contrastenhanced MRI (circumferential contrast enhancement of the arterial wall), and PET-CT (circumferential FDG uptake of the arterial wall). * d Stenotic lesions and dilated lesions are detected by chest radiography (wave-like deformation of the descending aorta), CT angiography, MR angiography, echocardiography (aortic insufficiency), and angiography. They are accompanied by dilatation of the ascending aorta and frequently also by aortic insufficiency. In the chronic stage, circumferential calcification of the arterial wall is visualized by CT, and the development of collateral circulation is detected by CT angiography and MR angiography Points of attention in imaging diagnosis: Contrast-enhanced CT is performed in the late phase of contrast enhancement. CT angiography is performed in the early phase of contrast enhancement with 3-dimensional image processing. Angiography is usually performed when other procedures such as endovascular treatment and coronary artery angiography or left ventriculography are simultaneously intended. C. Conditions to be included in the differential diagnoses of Takayasu arteritis Arteriosclerosis, congenital vascular anomaly, inflammatory abdominal aortic aneurysm, infectious aneurysm, syphilitic mesaortitis, giant cell arteritis (temporal arteritis), vascular Behçet's disease, IgG4-related diseases. Definite: At least 1 of the items in A + any of the conditions in B are observed, and conditions in C can be excluded.

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Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

Cited by 21 publications

References 8 publications

A case report of Hypocomplementemic urticarial vasculitic syndrome presenting with Renal failure

A case report of Hypocomplementemic urticarial vasculitic syndrome presenting with Renal failure

Hypocomplementemic urticarial vasculitis syndrome

JCS 2017 Guideline on Management of Vasculitis Syndrome　― Digest Version ―

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Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

Cited by 21 publications

References 8 publications

A case report of Hypocomplementemic urticarial vasculitic syndrome presenting with Renal failure

A case report of Hypocomplementemic urticarial vasculitic syndrome presenting with Renal failure

Hypocomplementemic urticarial vasculitis syndrome

JCS 2017 Guideline on Management of Vasculitis Syndrome ― Digest Version ―

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JCS 2017 Guideline on Management of Vasculitis Syndrome　― Digest Version ―