C1Q nephropathy in children

Levart, Tanja Kersnik; Kenda, Rajko B.; Čavić, Mojca Avguštin; Ferluga, D; Hvala, Anastazija; Vizjak, Alenka

doi:10.1007/s00467-005-2040-4

Cited by 46 publications

(5 citation statements)

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“…We also found 3 more patients who could be diagnosed with IgMN according to IF criteria [ 16 ]. However, the inconsistency of diagnosis of IgMN and C1qN was not a problem in our study alone, since many other studies also used different criteria for the diagnosis of IgMN and C1qN [ 14 – 16 , 18 – 21 , 28 ]. We assume this reflects the uncertainty of IgMN and C1qN as distinct clinical disease entities [ 13 , 15 , 29 ].…”

Section: Discussionmentioning

confidence: 75%

Glomerular Immune Deposits Are Predictive of Poor Long-Term Outcome in Patients with Adult Biopsy-Proven Minimal Change Disease: A Cohort Study in Korea

Lee

Baek

et al. 2016

PLoS ONE

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Background and ObjectivesThere has been little published information on risk factors for poor long-term outcome in adult biopsy-proven minimal change disease (MCD).MethodsData from sixty-three adult, biopsy-proven primary MCD patients treated at a tertiary university hospital between 2003 and 2013 were analyzed. Baseline clinical and pathologic factors were assessed for the associations with composite outcome of creatinine doubling, end stage renal disease, or all-cause mortality.ResultsDuring a median (interquartile) 5.0 (2.8–5.0) years, the composite outcome occurred in 11.1% (7/63) of patients. The rate of glomerular immune deposits was 23.8% (15/63). Patients with glomerular immune deposits showed a significantly lower urine protein creatinine ratio than those without deposits (P = 0.033). The rate of non-responders was significantly higher in patients with glomerular immune deposits than in those without deposits (P = 0.033). In patients with deposits, 26.7% (4/15) developed the composite outcome, while only 6.3% (3/48) developed the composite outcome among those without deposits (P = 0.049). In multivariate Cox proportional hazards regression analysis, the presence of glomerular immune deposits was the only factor associated with development of the composite outcome (hazard ratio: 2.310, 95% confidence interval: 1.031–98.579, P = 0.047).ConclusionGlomerular immune deposits were associated with increased risk of a composite outcome in adult MCD patients. The higher rate of non-responders in patients with deposits might be related to the poor outcome. Future study is needed.

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Section: Discussionmentioning

confidence: 75%

Glomerular Immune Deposits Are Predictive of Poor Long-Term Outcome in Patients with Adult Biopsy-Proven Minimal Change Disease: A Cohort Study in Korea

Lee

Baek

et al. 2016

PLoS ONE

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“…Another possibility for why our eligible cases did not experience abnormal urinalysis is that immunosuppressive therapy against rejection might suppress the inflammation, although Kersnik Levart et al reported that cyclosporine A and/or mycophenorate mofetil were not effective for some cases of C1qN [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Predominant but silent C1q deposits in mesangium on transplanted kidneys - long-term observational study

et al. 2018

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BackgroundC1q nephropathy (C1qN) was first described as glomerular disease characterized by predominant meangial C1q deposits in patients with proteinuria and no evidence of systemic lupus erythematosus. Several studies, however, revealed the clinical heterogeneity of C1qN, showing some cases with normal urinalysis. To confirm the existence of cases with predominant mesangial C1q deposits and negative or mild proteinuria and/or hematuria, we investigated renal graft biopsy specimens showing negative to mild proteinuria (less than or equal to 1+ by dip stick test) and/or hematuria.MethodsEligible participants were kidney transplant cases who corresponded to the criteria for C1qN and were followed more than 10 years. Their medical records were reviewed to determine the age at detection of predominant mesangial C1q deposits, gender, original renal disease and reason for renal graft biopsy, blood pressure, degree of proteinuria and hematuria, and serum creatinine levels.ResultsFrom 414 cases in adults and children, five pediatric patients (the male to female ratio, 1:1.5) were eligible. At the time when predominant mesangial C1q deposits were detected, 2 cases presented with mild proteinuria without hematuria, but the other 3 cases showed normal urinalysis. Light microscopy revealed minor glomerular abnormality in all the cases. Immunofluorescent study showed predominant mesangial C1q deposits with IgG, IgM and C3 in all cases. All selected specimens presented electron dense-depos in the mesangium. Ten years later from the detection, 2 cases continued to be normal urinalysis and 3 cases had mild proteinuria without hematuria. During this follow-up period, no cases presented with persistent proteinuria and/or hematuria greater than or equal to 2+ by dip stick test. And no cases developed systemic lupus erythematosus. Follow-up renal graft biopsies were performed once in 2 cases 8 years later from the detection. They showed minor glomerular abnormalities. C1q deposit disappeared in one case. In another case, immunofluorescent study was not examined.ConclusionsThis long-term observational study on transplanted kidneys confirms the existence of cases with predominant but silent C1q deposits in the mesangium who have negative or mild proteinuria.Electronic supplementary materialThe online version of this article (10.1186/s12882-018-0874-9) contains supplementary material, which is available to authorized users.

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“…The histopathological findings described in patients with C1qN have usually varied with minimal change disease and FSGS being reported most commonly [ 2 , 4 , 6–8 , 10 ]. In a study by Markowitz et al , as many as 17 out of the 19 patients with C1qN had FSGS on renal histopathology.…”

Section: Discussionmentioning

confidence: 99%

C1q nephropathy: a true immune complex disease or an immunologic epiphenomenon?

Muorah

Sinha

Horsfield

et al. 2009

Clinical Kidney Journal

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We describe a 16-year-old Caucasian boy who presented with steroid-sensitive nephrotic syndrome aged 2 years. His clinical course was one of frequent relapses and severe steroid dependence. To manage this, he was sequentially treated with levamisole, then oral cyclophosphamide before being started on ciclosporin. A renal biopsy performed prior to commencement of ciclosporin confirmed minimal change disease on light microscopy. The immunohistochemistry and electron microscopy findings were in keeping with this. His complement levels were normal and his lupus serology negative. He remained on ciclosporin therapy for 8 years and had two further renal biopsies to detect ciclosporin-induced renal damage. Both biopsies showed evidence of increasing amounts of C1q deposition on immunohistochemistry and the presence of immune deposits on electron microscopy. As he had continued negative lupus serology, this was compatible with a diagnosis of C1q nephropathy. In addition both biopsies had changes compatible with chronic mild ciclosporin nephrotoxicity. This case is the first report describing in detail a paediatric patient with evolving C1q nephropathy who was treated successfully with rituximab. We discuss the role of C1q in this clinicopathological entity and question its significance.

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C1Q nephropathy in children

Cited by 46 publications

References 9 publications

Glomerular Immune Deposits Are Predictive of Poor Long-Term Outcome in Patients with Adult Biopsy-Proven Minimal Change Disease: A Cohort Study in Korea

Glomerular Immune Deposits Are Predictive of Poor Long-Term Outcome in Patients with Adult Biopsy-Proven Minimal Change Disease: A Cohort Study in Korea

Predominant but silent C1q deposits in mesangium on transplanted kidneys - long-term observational study

C1q nephropathy: a true immune complex disease or an immunologic epiphenomenon?

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