Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression

Häffner, Karsten; Michelfelder, Stefan; Pöhl, Martin

doi:10.1007/s00467-015-3111-9

Cited by 26 publications

(20 citation statements)

References 41 publications

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“…Owing to the severity of his disease presentation, the massive complement consumption, and the lack of other treatment options in C3G, we decided to start TPE to remove autoantibodies. Plasma exchange treatment in patients with C3G has been reported otherwise . He had a complete recovery after TPE, resulting in normal renal function and no proteinuria.…”

Section: Resultsmentioning

confidence: 99%

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Therapeutic plasma exchange in children: One center's experience

Cortina

Ojinaga

Giner

et al. 2017

J of Clinical Apheresis

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Section: Resultsmentioning

confidence: 99%

“…Plasma exchange treatment in patients with C3G has been reported otherwise. 13 He had a complete recovery after TPE, resulting in normal renal function and no proteinuria.…”

Section: Glomerulopathiesmentioning

confidence: 94%

Therapeutic plasma exchange in children: One center's experience

Cortina

Ojinaga

Giner

et al. 2017

J of Clinical Apheresis

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“…C3‐nephritic factor is a kind of auto‐antibody which stabilizes convertases of the alternative complement pathway, resulting in consumption of C3 (hypocomplementaemia) and amplification of downstream events in the complement cascade. C3‐nephritic factor is found in the majority of patients with dense deposit disease and in half of the patients with C3 glomerulonephritis . Studies also demonstrate that deficiency and dysfunction of CFH are important in some types of C3G.…”

Section: Complement‐associated Kidney Diseasesmentioning

confidence: 99%

“…C3-nephritic factor is found in the majority of patients with dense deposit disease and in half of the patients with C3 glomerulonephritis. 15 Studies also demonstrate that deficiency and dysfunction of CFH are important in some types of C3G. Meanwhile, structurally abnormal of factor H-related (CFHR) proteins is currently thought to antagonize CFH, a process termed 'CFH de-regulation.'…”

Section: C3 Glomerulopathymentioning

confidence: 99%

Complement in glomerular diseases

Tan

Zhao

2018

Nephrology

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Complement activation has been identified to play a vital role in the pathogenesis of many glomerulonephritis, either as direct complement activation‐driven factor in thrombotic microangiopathy and C3 glomerulopathy, and/or as an important contributor in lupus nephritis and anti‐neutrophil cytoplasmic antibody‐associated vasculitis. Recent studies indicated that complement activation may also play roles in the pathogenesis of immunoglobulin A nephropathy and focal segmental glomerulosclerosis. Interestingly, monoclonal immunoglobulins/light chains from patients with monoclonal gammopathy may interfere with complement activation and thus indirectly result in complement‐mediated glomerulonephritis. Understanding of the pathogenic roles of complement activation in various glomerulonephritis will facilitate the identification of potential novel therapeutic targets in complement system.

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“…The rates of graft loss due to recurrence ranges between 34 and 66% [167,168]. A few patients may respond to plasma exchange [169]. Good results have been reported with eculizumab in DDD patients [170,171].…”

Section: Mpgnmentioning

confidence: 99%

Histopathological findings in transplanted kidneys

et al. 2017

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Improvements in immunosuppression have reduced acute kidney allograft rejection and clinicians are now seeking ways to prolong allograft survival to 20 years and beyond. The primary cause of kidney allograft loss is still chronic rejection, followed by death with a functioning allograft and primary kidney disease recurrence. Thus, overcoming kidney allograft rejection remains the most important issue. Kidney allograft rejection can be classified into two types: T cell-and antibody-mediated rejection. Both are diagnosed pathologically based on the Banff 2013 classification. Other important pathological features in addition to rejection include calcineurin inhibitor toxicity, polyomavirus nephropathy, and recurrence of the primary kidney disease. Here, we review the diagnosis and representative features of histopathological findings in transplanted kidneys.

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Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression

Abstract: As this multimodal therapeutic approach was effective in all four treated patients with suspected autoimmune etiology of C3G, it offers a treatment option for severely affected patients with this rare disease until more specific regimens are available.

Cited by 26 publications

References 41 publications

Therapeutic plasma exchange in children: One center's experience

Therapeutic plasma exchange in children: One center's experience

Complement in glomerular diseases

Histopathological findings in transplanted kidneys

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