scholarly journals From membranoproliferative glomerulonephritis to a final diagnosis: Hypocomplementemic urticarial vasculitis syndrome

2022 ◽  
Vol 35 (4) ◽  
Author(s):  
Joana Marques ◽  
◽  
Tiago Pereira ◽  
Dulce Carvalho ◽  
Mário Góis ◽  
...  
Keyword(s):  
Membranoproliferative Glomerulonephritis ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
Final Diagnosis ◽  
Skin Lesions ◽  
Glomerular Injury ◽  
Urticarial Vasculitis ◽  
Proper Diagnosis ◽  
Hypocomplementemic Urticarial Vasculitis Syndrome ◽  
Hypocomplementemic Urticarial Vasculitis

Membranoproliferative glomerulonephritis describes a glomerular-injury pattern common to a heterogeneous group of diseases. Evaluation based on clinical and laboratory presentation and immunofluorescence staining on kidney biopsy allows identification of underlying pathophysiological processes and may facilitate proper diagnosis and treatment. Hypocomplementemic urticarial vasculitis syndrome is a rare autoimmune disease of multi-organ involvement. The diagnosis is based on well-defined clinical and laboratory criteria. The pathophysiology is not completely understood but the presence of anti-C1q antibody seems to be involved. Renal involvement occurs in up to 50% of cases. It can be heterogeneous and can be indistinguishable from lupus nephritis. Serological findings and skin involvement distinguish these two entities. We report the case of a 40-year-old female who presented with urticarial skin lesions, hypocomplementemia and nephrotic syndrome. Kidney biopsy showed membranoproliferative glomerulonephritis with full house immune complex deposits. The diagnosis of hypocomplementemic urticarial vasculitis syndrome was made and the patient was successfully treated with prednisolone and mycophenolate mofetil.

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 Biopsy-Proven Kidney Involvement in Hypocomplementemic Urticarial Vasculitis

2021 ◽  
Author(s):  
Alice Corthier ◽  
Marie Jachiet ◽  
Daniel Bertin ◽  
Aude Servais ◽  
Christelle Barbet ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Kidney Biopsy ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Kidney Injury ◽  
Urticarial Vasculitis ◽  
Kidney Involvement ◽  
National Cohort ◽  
Hypocomplementemic Urticarial Vasculitis

Abstract Background. Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis. We aimed to describe the kidney involvement of HUV in a multicenter national cohort with an extended follow-up. Methods. All patients with HUV (international Schwartz criteria) with a biopsy-proven kidney involvement, identified through a survey of the French Vasculitis Study Group, were included. A systematic literature review on kidney involvement of HUV was performed. Results. Twelve patients were included, among whom 8 had positive anti-C1q antibodies. All presented with proteinuria, from mild to nephrotic, and 8 displayed acute kidney injury (AKI), requiring temporary haemodialysis in 2. Kidney biopsy showed membrano-proliferative glomerulonephritis (GN) in 8 patients, pauci-immune crescentic GN or necrotizing vasculitis in 3 patients (with a mild to severe interstitial inflammation), and an isolated interstitial nephritis in 1 patient. C1q deposits were observed in the glomeruli (n=6), tubules (n=4) or renal arterioles (n=3) of 8 patients. All patients received corticosteroids, and 9 were also treated with immunosuppressants or apheresis. After a mean follow-up of 8.9 years, 6 patients had a preserved renal function, but 2 patients had developed stage 3-4 chronic kidney disease (CKD) and 4 patients had reached end-stage kidney disease (ESKD), among whom 1 had received a kidney transplant. Conclusion. Renal involvement of HUV can be responsible for severe AKI, CKD and ESKD. It is not always associated with circulating anti-C1q antibodies. Kidney biopsy shows mostly membrano-proliferative GN or crescentic GN, with frequent C1q deposits in the glomeruli, tubules or arterioles.

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

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