Clinicopathologic Correlation of C1q Nephropathy in Children

Fukuma, Yuko; Hisano, Satoshi; Segawa, Yoshie; Niimi, Kazuhiko; Tsuru, Noboru; Kaku, Yoshitsugu; Hatae, Ken; Kiyoshi, Yasuhiro; Mitsudome, Akihisa; Iwasaki, Hiroshi

doi:10.1053/j.ajkd.2005.11.013

Cited by 47 publications

(44 citation statements)

References 13 publications

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“…The prevalence of C1q nephropathy among native kidney biopsies ranges from 0.2 to 1.9%. 2,4,6,10 The prevalence of intense C1q-dominant or codominant mesangial deposits among our renal allograft biopsies that underwent full IF analysis was 0.4%. This prevalence suggests that de novo 'C1q nephropathy' likely represents the third most common de novo morphological glomerular pattern after de novo focal segmental glomerulosclerosis (seen in 10-20% of allografts) and de novo membranous glomerulopathy (seen in 2-9% of allograft biopsies).…”

Section: Discussionmentioning

confidence: 90%

“…[2][3][4][5] In more recent reports, patients presenting with asymptomatic proteinuria or hematuria outnumbered those presenting with nephrotic syndrome. 6,7,10 In a recent study by Hisano et al, from Japan, 59% of C1q nephropathy cases were detected by urine screening tests, most commonly for school children. 7 A third of all their patients had mild proteinuria (r50 mg per 100 ml), but only one had no proteinuria.…”

Section: Discussionmentioning

confidence: 98%

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C1q deposition in the renal allograft: a report of 24 cases

et al. 2010

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C1q nephropathy is an uncommon glomerular disease characterized by dominant or codominant mesangial staining for C1q in the absence of systemic lupus erythematosus. There are no series in the literature addressing the significance of C1q deposition in the renal allograft. We retrospectively analyzed 24 patients, most of whom were white (83%) and male (63%), with a mean age at transplant of 31 years. None of the patients were diagnosed with C1q nephropathy in the native kidney or had any features of systemic lupus erythematosus. The mean time from transplant to detection of mesangial C1q deposits was 37 months (412 months in 71% of cases). Half of the patients had a preceding infection. The indication for biopsy was surveillance (63%) or graft dysfunction (37%). At biopsy, 52% had proteinuria (41g/day in only 17%). The mean creatinine was 1.8 mg per 100 ml. Only 9% developed hematuria and none had hypoalbuminemia. The glomerular pattern on light microscopy was mesangial hypercellularity (46%), focal segmental glomerulosclerosis (21%), or no lesions (33%). All cases showed intense (Z2 þ ) dominant (67%) or codominant (33%) mesangial staining for C1q on immunofluorescence. Mesangial electron-dense deposits were seen in 82% of cases. On follow-up (mean 1 year) of the 10 patients without rejection, most had stable creatinine with no or stable proteinuria, and none lost their graft. We conclude that C1q-dominant mesangial deposition in the renal allograft is a morphological pattern with no apparent clinical significance in the majority of patients. It is usually detected after the first year. The rate of preceding infection and the prevalence of proteinuria seem to be similar to the renal transplant recipients in general. Most cases show mesangial hypercellularity or no glomerular changes on light microscopy. Modern Pathology (2010) 23, 1080-1088; doi:10.1038/modpathol.2010.92; published online 14 May 2010 Keywords: C1q nephropathy; transplant; de novo glomerular disease; FSGS; proteinuria; mesangial hypercellularityIn 1982, Jones and Magil 1 reported five patients with proteinuria and nonsystemic mesangiopathic glomerulonephritis, one of whom had C1q codominant mesangial deposits. In 1985, Jennette and Hipp 2 introduced the term 'C1q nephropathy' and defined it as dominant or codominant mesangial staining for C1q in patients lacking clinical or serological evidence of systemic lupus erythematosus. In their report, which included 15 patients who presented in their second or third decade of life with nephrotic-range proteinuria, 100% of patients had proteinuria (range 1-24 g/day), 40% hematuria, 0% hypocomplementemia, and 0% positive antinuclear antibodies. The light microscopic findings varied from normal-appearing glomeruli to mesangial hypercellularity to endocapillary hypercellularity. On immunofluorescence (IF), all cases showed intense segmental or global mesangial staining for C1q, which was accompanied by equal or weaker staining for C3 and immunoglobulins. On electron microscopy, all cases showed mesangial ele...

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Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 98%

C1q deposition in the renal allograft: a report of 24 cases

et al. 2010

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“…3,4,6 -9 Patients with nephrotic syndrome, particularly those with FSGS, showed a poor response to corticosteroid therapy, whereas patients presenting with asymptomatic urinary abnormalities maintained normal renal function. 3,4,8,9 Additional studies are needed to elucidate this heterogeneous glomerular disease.…”

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confidence: 99%

Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy

Vizjak

Ferluga

Rožič

et al. 2008

Journal of the American Society of Nephrology

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C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions

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“…However, according to another study, 30% of patients who had nephrotic-range proteinuria had no evidence of nephrotic syndrome [4]. On the other hand, Fukuma et al reported 60% of C1q nephropathy patients who presented with non-nephrotic-range proteinuria [10]. One case was reported to have rapidly progressive glomerulonephritis [11].…”

Section: Discussionmentioning

confidence: 98%

Atypical Presentation of C1q Nephropathy in an Adolescent with Initial Hypertension

Çonkar¹,

S²,

Bulut³

et al. 2016

JCR

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Abstract:Background: Hypertension is the leading cause of mortality worldwide. Besides its already high disease burden, also the prevalence in children is increasing. Major presentation of hypertension among young patients is as essential or primary hypertension, while only a minority of patients present as secondary hypertension. Characterized by mesangial immune deposits, C1q nephropathy is a relatively rare glomerular disease with a dominant or co-dominant staining for C1q, presenting often with proteinuria in the nephrotic range. Case Report: We present an 18-year-old Turkish boy who presented with hypertension five years ago in the absence of proteinuria followed by a renal biopsy five years later demonstrating dominant mesangial deposits of C1q. Conclusion: We suggest that C1q nephropathy may present with hypertension before the manifestation of proteinuria and thus, C1q nephropathy should be kept in mind in patients presenting with hypertension. More specialized tests should be performed when diagnosing primary hypertension. We suggest that every patient with hypertension should be carefully followed up for C1q nephropathy.

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Clinicopathologic Correlation of C1q Nephropathy in Children

Cited by 47 publications

References 13 publications

C1q deposition in the renal allograft: a report of 24 cases

C1q deposition in the renal allograft: a report of 24 cases

Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy

Atypical Presentation of C1q Nephropathy in an Adolescent with Initial Hypertension

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