Clinical And Pathological Features Of Membranous Nephropathy In Children

BackgroundMembranous nephropathy(MN)is a group of chronic glomerular disease with characteristic pathological changes.MN is typically classified as idiopathic or secondary according to the etiology.The incidence,etiology,clinical and pathological features of MN in children are quite different from those in adults.ObjectiveTo analyze the clinical and pathological features,treatment and prognosis of children’s MN to guide clinical diagnosis and treatment.MethodThis study jointly collected cases from multiple centers,including Nanfang Hospital of Southern Medical University,the First Affiliated Hospital of Jinan University and Sun Yat-sen Memorial Hospital of Sun Yat-sen University;MN patients diagnosed with MN from 2008 to 2021 were followed up until February 2022.General information,vaccination history,exposure history,laboratory tests and examinations,renal biopsy pathology,treatment plan,follow-up renal function and other datas were collected and analyzed by statistical analysis.Result(1)22 patients in total,including 4 cases of IMN(18.2%),18 cases(81.1%)of secondary MN,including 6 patients with HBV-MN(27.3%),9 patients with Lupus nephritis(40.9%),and scleroderma,Hypereosinophilia and autoimmune thyroiditis each had 1 case(4.5%).(2)12 females and 10 males among the 22patients,the radio of male:female of IMN is 1:1,and secondary MN is 5:4.The average age of onset was 10.39±3.98 years old.(3)Nephrotic syndrome is common type in both IMN and secondary membranous nephropathy,most of the patients accompany hematuria.(4)Both IMN and secondary MN are most common in stage Ⅱ MN.Typical pathological features of MN such as diffuse immune complex deposition under GBM epithelium and diffuse thickening of GBM can be observed,mainly IgG and C3 deposition,manifestations such as mesangial cell and matrix hyperplasia,interstitial changes and deposition of electron dense material at multiple sites can be observed in secondary MN.(5)Compared with SLE-MN patients with type IV+V LN,the levels of SCr,complement IgG and C4,positive rate of anticardiolipin antibody and positive rate of crescent were statistically different(P<0.05).Conclusion1.the initial levels of Scr,IgG and C4 in children’s SLE-MN were lower than those of type IV+V LN,and type IV+V LN was more likely to combine with crescent formation.2.The early stage of SLE in children may be atypical and easily misdiagnosed.Positive anti-PLA2R antibody and/or positive PLA2R and IgG4 in renal tissue cannot completely rule out secondary MN such as SLE-MN.Renal histological changes and disease progression may occur in the process from early atypical to typical SLE manifestations,and repeat renal biopsy may be performed if necessary.It is possible that two glomerular diseases with different pathogenesis,SLE-MN and IMN,co-occur in the same patient.3.The clinical manifestations of HBV-MN in children are not typical,and a few have inconsistency between renal tissue and serum HBV markers.In children with MN,even if serum HBV markers are negative,kidney HB V markers should be checked,and the clinical manifestations and pathological findings should be integrated to avoid missed diagnosis and misdiagnosis.Some patients had a history of vaccination,family history of hepatitis B and contact history.4.MN can coexist with other types of glomerulonephritis.Patients with MN and anti-GBM disease are rare and most of them are adults,but it can also occur in children.Treatment regimens should be individualized.