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2018 ; 19
(1
): 82
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Predominant but silent C1q deposits in mesangium on transplanted kidneys -
long-term observational study
#MMPMID29625558
Kanai T
; Akioka Y
; Miura K
; Hisano M
; Koike J
; Yamaguchi Y
; Hattori M
BMC Nephrol
2018[Apr]; 19
(1
): 82
PMID29625558
show ga
BACKGROUND: C1q 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. METHODS: Eligible 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. RESULTS: From 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. CONCLUSIONS: This 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.