scholarly journals A Case of Hypocomplementemic Urticarial Vasculitis Syndrome Successfully Treated With Omalizumab

2017 ◽  
Vol 27 (6) ◽  
pp. 382-384 ◽  
Author(s):  
E Nucera ◽  
F Basta ◽  
A Buonomo ◽  
S Mezzacappa ◽  
DP Margiotta ◽  
...  
Keyword(s):  
Urticarial Vasculitis ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Hypocomplementemic Urticarial Vasculitis

scholarly journals Kidney Involvement in Hypocomplementemic Urticarial Vasculitis Syndrome—A Case-Based Review

2020 ◽  
Vol 9 (7) ◽  
pp. 2131 ◽  
Author(s):  
Oana Ion ◽  
Bogdan Obrișcă ◽  
Gener Ismail ◽  
Bogdan Sorohan ◽  
Sonia Bălănică ◽  
...  
Keyword(s):  
Current Knowledge ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Small Vessel Vasculitis ◽  
Extensive Literature ◽  
Urticarial Vasculitis ◽  
Pattern Of Injury ◽  
Kidney Involvement ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Hypocomplementemic Urticarial Vasculitis

Hypocomplementemic urticarial vasculitis syndrome (HUVS), or McDuffie syndrome, is a rare small vessel vasculitis associated with urticaria, hypocomplementemia and positivity of anti-C1q antibodies. In rare cases, HUVS can manifest as an immune-complex mediated glomerulonephritis with a membranoproliferative pattern of injury. Due to the rarity of this disorder, little is known about the clinical manifestation, pathogenesis, treatment response and outcome of such patients. We describe here three cases of HUVS with severe renal involvement. These patients had a rapidly progressive form of glomerulonephritis with severe nephrotic syndrome against a background of a membranoproliferative pattern of glomerular injury with extensive crescent formation. Therefore, these patients required aggressive induction and maintenance immunosuppressive therapy, with a clinical and renal response in two patients, while the third patient progressed to end-stage renal disease. Because of the rarity of this condition, there are few data regarding the clinical presentation, pathology and outcome of such patients. Accordingly, we provide an extensive literature review of cases reported from 1976 until 2020 and place them in the context of the current knowledge of HUVS pathogenesis. We identified 60 patients with HUVS and renal involvement that had adequate clinical data reported, out of which 52 patients underwent a percutaneous kidney biopsy. The most frequent renal manifestation was hematuria associated with proteinuria (70% of patients), while one third had abnormal kidney function on presentation (estimated glomerular filtration (GFR) below 60 mL/min/1.73 m2). The most frequent glomerular pattern of injury was membranoproliferative (35%), followed by mesangioproliferative (21%) and membranous (19%). Similar to other systemic vasculitis, renal involvement carries a poorer prognosis, but the outcome can be improved by aggressive immunosuppressive treatment.

scholarly journals P1457 Septic and aseptic valvular involvement in hypocomplementemic urticarial vasculitis syndrome

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
V Scheggi ◽  
I Olivotto ◽  
B Alterini
Keyword(s):  
Rare Condition ◽  
Sudden Onset ◽  
Immunosuppressed Patient ◽  
Urticarial Vasculitis ◽  
Mitral Valves ◽  
Small Vessels ◽  
Classical Complement Pathway ◽  
Antibody Positivity ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Hypocomplementemic Urticarial Vasculitis

Abstract Background Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS) is a very rare disease involving small vessels and characterized by chronic urticarial vasculitis, arthralgia, arthritis, and activation of the classical complement pathway. To our knowledge, just other seven cases of HUVS with cardiac valvular involvement have been reported in the literature. Unlike previous cases, ours shows a broader antibody positivity, (but no anti-C1q antibodies) with an early and serious cardiovascular involvement. Case summary A 32 years old woman was brought to the emergency department because of sudden onset of right hemiplegia and global aphasia due to cerebral haemorrage. She suffered from a severe HUVS form with cardiac valvular involvement and she was cronically treated with immunosuppressant drugs. During previous years she had already undergone cardiac surgery twice for chronic aseptic inflammatory process involving aortic and mitral valves. Right hemiplegia and global aphasia were caused by septic embolism to left cerebral artery complicated by cerebral haemorrage. The patient underwent neurosurgical intervention followed by antibiotic therapy. This condition was secondary to Listeria monocytogenes aortic infective endocarditis in an immunosuppressed patient. Transesophageal echocardiogram showed periaortic abscess with internal colliquation. A further surgical intervention would have been necessary, but operative risk was judged to high and the patient died one year later. Discussion The association between HUVS, Jaccaud"s arthropathy and cardiac valvular disease is rarely described in the literature. The presence of valvular involvement is a negative prognostic factor. Unlike most cases, it is interesting to note that our patient was negative for anti-C1q antibodies and exhibited an extremely severe cardiac involvement. The case was complicated by L monocytogenes endocarditis, that is also a rare condition associated with a high mortality rate. Abstract P1457 Figure. Abscess

Renal Transplantation in a Patient With Hypocomplementemic Urticarial Vasculitis Syndrome

2001 ◽  
Vol 37 (1) ◽  
pp. 144-148 ◽  
Author(s):  
Philippe Grimbert ◽  
Klaus Schulte ◽  
Claude Buisson ◽  
Dominique Desvaux ◽  
Christophe Baron ◽  
...  
Keyword(s):  
Renal Transplantation ◽  
Urticarial Vasculitis ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Hypocomplementemic Urticarial Vasculitis

scholarly journals URTICARIAL VASCULITIS IN CHILDREN

2021 ◽  
Vol 2021 (3) ◽  
pp. 16-29
Author(s):  
O. M. Оkhotnikova ◽  
O. A. Oshlyanskaya
Keyword(s):  
Rare Disease ◽  
Chronic Urticaria ◽  
Immunoglobulin E ◽  
Combined Treatment ◽  
Urticarial Vasculitis ◽  
Complement Components ◽  
National Healthcare ◽  
Low Efficiency ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Hypocomplementemic Urticarial Vasculitis

URTICARIAL VASCULITIS IN CHILDREN O. M. Оkhotnikova, O. A. Oshlyanskaya Shupyk National Healthcare University of Ukraine, Kyiv, Ukraine Abstract. Different autoimmune diseases can present with organs malfunctioning and chronic urticaria symptoms in particular such urticarial vasculitis. This variant of vasculitis can be a separate nosological form, which includes a rare disease as hypocomplementemic urticarial vasculitis syndrome. In addition to chronic urticaria symptoms, hypocomplementemic urticarial vasculitis syndrome is characterized by severe systemic manifestations in different organs, decreased serum level of complement components, and appearance of specific markers, such as anti-C1q-antibodies. The diagnosis is confirmed by the results of skin biopsy, which is the «gold standard» of diagnosis. The condition often requires combined treatment with two immunosuppressive drugs. Hypocomplementemic urticarial vasculitis syndrome is a very rare disease, but, because of the multisystem manifestations, it can be encountered by any specialist (pediatrician, general practitioner, allergist, rheumatologist, ophthalmologist, dermatologist, nephrologist, etc.), therefore doctors should consider this condition when dealing with such patients in order to reduce the risk of hypocomplementemic urticarial vasculitis syndrome misdiagnosis as the variants of chronic urticaria or another immunopathogenetic disease. That is why doctors should be well aware of the features of the course of this disease, not only to reduce the likelihood of erroneous diagnosis of urticarial vasculitis as chronic urticaria or another disease of immunopathological genesis, but also because modern therapy of these conditions is radically different from each other: сhronic idiopathic urticaria requires adherence to a modern patient management algorithm with the initial use of H1-antihistamines with a possible increase in the dose (up to a 4-fold increase), and with their low efficiency — the transition to the use of cytostatics, monoclonal antibodies against immunoglobulin E –omalizumab, and is extremely limited and only for short-term use of glucocorticosteroids. Key words: urticarial vasculitis, its forms, hypocomplementemia, chronic urticaria, children. Olena Okhotnikova MD, PhD, professor, Head Department of Pediatrics #1, Shupyk National Healthcare University of Ukraine, 9, Dorogozhytska str., 04112, Kyiv, Ukraine e-mail: [email protected] Аsthma and Allergy, 2021, 3, P. 16–29.

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