Pauci-Immune Necrotizing and Crescentic Glomerulonephritis with Membranous Lupus Nephritis, Fifteen Years after Initial Diagnosis of Secondary Membranous Nephropathy

Burkhart, Ryan; Shah, Nina; Abel, Michael E.; Oliver, James D.; Lewin, Matthew R.

doi:10.1155/2015/120762

Cited by 3 publications

(3 citation statements)

References 20 publications

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“…In addition to these cases of overlap syndrome reported in the literature, there have also been cases of ANCA positive glomerulonephritis in the setting of SLE as well as ANCA negative glomerulonephritis outside the setting of SLE [1,4,7,[10][11][12]14]. There have also been cases of pauci-immune glomerulonephritis reported in the setting of concurrent lupus nephritis [4,8,15].…”

Section: A Rare Case Of Overlapping Syndrome Of Anca-associated Glomementioning

confidence: 98%

A Rare Case of Overlapping Syndrome of ANCAAssociated Glomerulonephritis and Systemic Lupus Erythematosus

Frey¹,

Shah²

2018

JRAD

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Introduction: Two very interesting rheumatologic diagnoses include Systemic Lupus Erythematosus (SLE) and Anti-Neutrophil Cytoplasmic Antibody (ANCA)-negative Vasculitis. It is rare to see these two conditions diagnosed in the same patient. This case analyzes the complex interactions between these two disease states. Case: This is the case of a 69 year old female who presented with shortness of breath, hematuria, and renal failure. Lab work was significant for elevated ANA (1:1280), positive double-stranded DNA antibody, normal complement levels and a negative Vasculitis panel. Renal biopsy was consistent with ANCA-associated Vasculitis (AAV). Thus the patient met diagnostic criteria for both SLE and AAV and was treated accordingly. Conclusion: A review of the literature reveals that these typically separate disease entities when diagnosed together may actually represent a completely new, overlapping syndrome. This case is unique in that there have not been any other cases of a simultaneous diagnosis of SLE and ANCA-negative Vasculitis without evidence of lupus nephritis reported in the literature. A deeper understanding of the mechanism driving these rare diseases can lead to improved prognostication and treatment of these conditions.

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Section: A Rare Case Of Overlapping Syndrome Of Anca-associated Glomementioning

confidence: 98%

A Rare Case of Overlapping Syndrome of ANCAAssociated Glomerulonephritis and Systemic Lupus Erythematosus

Frey¹,

Shah²

2018

JRAD

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“…In a significant number of patients, ANCAs are also detected [105]. Biopsy examination mainly reveals focal segmental glomerulosclerosis or mesangial proliferative glomerulonephritis lesions [106].…”

Section: T Cells Can Cause Disruption Of the Glomerular Basement Memb...mentioning

confidence: 99%

“…Biopsy examination mainly reveals focal segmental glomerulosclerosis or mesangial proliferative glomerulonephritis lesions [106]. In certain cases, ANCAs may be associated with membranous lupus nephritis [105]. ANCA-associated necrotizing and crescentic glomerulonephritis may present as superimposed nephropathy on lupus nephritis [107].…”

Section: T Cells Can Cause Disruption Of the Glomerular Basement Memb...mentioning

confidence: 99%

Three Diseases Mediated by Different Immunopathologic Mechanisms—ANCA-Associated Vasculitis, Anti-Glomerular Basement Membrane Disease, and Immune Complex-Mediated Glomerulonephritis—A Common Clinical and Histopathologic Picture: Rapidly Progressive Crescentic Glomerulonephritis

Gluhovschi,

Gadalean,

Velciov

et al. 2023

Biomedicines

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Immune mechanisms play an important role in the pathogenesis of glomerulonephritis (GN), with autoimmunity being the main underlying pathogenetic process of both primary and secondary GN. We present three autoimmune diseases mediated by different autoimmune mechanisms: glomerulonephritis in vasculitis mediated by anti-neutrophil cytoplasmic antibodies (ANCAs), glomerulonephritis mediated by anti-glomerular basement membrane antibodies (anti-GBM antibodies), and immune complex-mediated glomerulonephritis. Some of these diseases represent a common clinical and histopathologic scenario, namely rapidly progressive crescentic glomerulonephritis. This is a severe illness requiring complex therapy, with the main role being played by therapy aimed at targeting immune mechanisms. In the absence of immune therapy, the crescents, the characteristic histopathologic lesions of this common presentation, progress toward fibrosis, which is accompanied by end-stage renal disease (ESRD). The fact that three diseases mediated by different immunopathologic mechanisms have a common clinical and histopathologic picture reveals the complexity of the relationship between immunopathologic mechanisms and their clinical expression. Whereas most glomerular diseases progress by a slow process of sclerosis and fibrosis, the glomerular diseases accompanied by glomerular crescent formation can progress, if untreated, in a couple of months into whole-nephron glomerulosclerosis and fibrosis. The outcome of different immune processes in a common clinical and histopathologic phenotype reveals the complexity of the relationship of the kidney with the immune system. The aim of this review is to present different immune processes that lead to a common clinical and histopathologic phenotype, such as rapidly progressive crescentic glomerulonephritis.

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ANCA-Associated Necrotizing Glomerulonephritis Overlapping with Mesangial Proliferative Lupus Nephritis Refractory to Plasmapheresis, Steroid Pulse Therapy, and a Combination of Mycophenolate Mofetil and Rituximab

Sharmeen

Cassol

Katô

2018

Case Reports in Rheumatology

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Necrotizing glomerulonephritis (GN) associated with antineutrophil cytoplasmic antibody (ANCA) has been increasingly recognized in the context of class III or IV lupus nephritis (LN), hereafter designated as ANCA-associated necrotizing LN. While this subset of GN appears to portend an unfavorable renal outcome, it is not clear whether it represents a distinct entity and benefits from a more aggressive therapy. We report a 78-year-old woman who presented with rapidly progressive GN and was found to have a double-stranded DNA (dsDNA) antibody, hypocomplementemia, antiphospholipid antibody, and strongly positive myeloperoxidase antibody. Renal pathology showed necrotizing and crescentic GN on a background of mesangial proliferative GN. Her kidney disease did not improve despite the treatment with plasmapheresis, three doses of methylprednisolone pulse therapy followed by prednisone at 1 mg/kg/day, rituximab, and mycophenolate mofetil. This case not only reinforces the notion that ANCA-associated necrotizing LN is associated with high dsDNA antibody, hypocomplementemia, and worse renal outcome but also adds new insight into the full spectrum of this emerging disease entity by demonstrating that the concurrence of ANCA-associated vasculitis is not specific for class III or IV LN but can also be seen on a background of class II LN.

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Pauci-Immune Necrotizing and Crescentic Glomerulonephritis with Membranous Lupus Nephritis, Fifteen Years after Initial Diagnosis of Secondary Membranous Nephropathy

Cited by 3 publications

References 20 publications

A Rare Case of Overlapping Syndrome of ANCAAssociated Glomerulonephritis and Systemic Lupus Erythematosus

A Rare Case of Overlapping Syndrome of ANCAAssociated Glomerulonephritis and Systemic Lupus Erythematosus

Three Diseases Mediated by Different Immunopathologic Mechanisms—ANCA-Associated Vasculitis, Anti-Glomerular Basement Membrane Disease, and Immune Complex-Mediated Glomerulonephritis—A Common Clinical and Histopathologic Picture: Rapidly Progressive Crescentic Glomerulonephritis

ANCA-Associated Necrotizing Glomerulonephritis Overlapping with Mesangial Proliferative Lupus Nephritis Refractory to Plasmapheresis, Steroid Pulse Therapy, and a Combination of Mycophenolate Mofetil and Rituximab

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