Clinicopathologic Correlation and Outcome of C1q Nephropathy

Hisano, Satoshi; Fukuma, Yuko; Segawa, Yoshie; Niimi, Kazuhiko; Kaku, Yoshitsugu; Hatae, Ken; Saitoh, Takao; Takeshita, Morishige; Iwasaki, Hiroshi

doi:10.2215/cjn.00830208

Cited by 36 publications

(32 citation statements)

References 17 publications

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“…[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%

“…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. Most cases with C1q deposition in the renal allograft in our cohort were diagnosed as an incidental finding on surveillance biopsy.…”

Section: Discussionmentioning

confidence: 98%

“…In the study by Hisano et al, 7 of 61 cases of C1q nephropathy in the native kidney, 8 patients underwent repeat biopsies. C1q deposits disappeared in three patients including two who had a minimal change disease pattern in the first biopsy and a focal segmental glomerulosclerosis pattern on the second biopsy and persisted in five patients.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…In another large study of 61 patients from Japan, the light microscopy pattern varied from no lesions (75%) to mesangial proliferative glomerulonephritis (12%), to focal segmental glomerulosclerosis (13%). 7 Clinically, 36 patients had asymptomatic proteinuria (r50 mg per 100 ml in most cases) and/or hematuria detected incidentally at urine screening tests, whereas the remaining 25 patients presented with nephrotic syndrome. 7 There has been only a single case report of de novo 'C1q nephropathy' in the renal transplant.…”

mentioning

confidence: 99%

“…7 Clinically, 36 patients had asymptomatic proteinuria (r50 mg per 100 ml in most cases) and/or hematuria detected incidentally at urine screening tests, whereas the remaining 25 patients presented with nephrotic syndrome. 7 There has been only a single case report of de novo 'C1q nephropathy' in the renal transplant. 8 The patient presented with renal allograft dysfunction without proteinuria.…”

mentioning

confidence: 99%

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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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