Diagnosis and treatment of IgA nephropathy and IgA vasculitis nephritis in Chinese children

Abstract

IgA nephropathy (IgAN) and IgA vasculitis-associated nephritis (IgAVN) are among the most frequent childhood glomerular diseases and are characterized by significant variability in clinical manifestations, pathological presentation and long-term outcomes. IgAVN, alternatively called purpura nephritis, is pathologically indistinguishable from kidney-limited IgAN. In Chinese children, the clinical presentations and pathological manifestations of IgAN and IgAVN are variable. The severity of proteinuria and abnormalities in kidney function and blood pressure of children in China are comparable to those of children in Europe, the USA, and Japan. Compared to Caucasian children and Japanese children, crescents were more common in Chinese children with IgAN or IgAVN. Approximately 10–20% of childhood IgAN or IgAVN progresses to impaired kidney function in China. Since 2007, a series of guidelines on the diagnosis and treatment of pediatric kidney diseases has been published following the principles of evidence-based medicine. However, a large difference exists between the Chinese evidence-based guidelines and the guidelines developed by Kidney Disease: Improving Global Outcomes (KDIGO) in 2021. Chinese children with IgAN or IgAVN were more likely to be treated with steroids or immunosuppressive agents. Further studies exploring the optimal treatment regimen for childhood IgAN or IgAVN are needed in the future.

Immunoglobulin A Vasculitis With Intussusception in Children

“…4 Louie et al suggested that the diagnosis of IgA vasculitis (HSP) should be considered when the pathological biopsy sequence shows isolated severe acute duodenitis, and biopsy should be performed before commencing steroids, because they may mask the underlying pathology and thus, further delay the diagnosis. 20 These pathological manifestations are atypical for purpura, which may affect the diagnosis, but we can see that their experience suggests that, in patients with IgA vasculitis complicated by intussusception, the duodenum may be more prone to the development of severe lesions. We believe that the pathology results are helpful for the diagnosis of the disease.…”

Renal disease in pediatric rheumatology

“…However, there are no randomized trials to determine the best treatment strategies. Retrospective, observational studies show that outcomes are for the most part favorable, regardless of the type of immunosuppression used, which includes MMF, corticosteroids, azathioprine amongst others [39 ▪ ]. Immunosuppression was more commonly used with severe IgAVN, which may have improved both follow-up and outcomes [39 ▪ ].…”

“…Retrospective, observational studies show that outcomes are for the most part favorable, regardless of the type of immunosuppression used, which includes MMF, corticosteroids, azathioprine amongst others [39 ▪ ]. Immunosuppression was more commonly used with severe IgAVN, which may have improved both follow-up and outcomes [39 ▪ ]. More recently the benefits of combination therapy for severe IgAVN were explored, defined as kidney pathology showing crescents/segmental lesions (graded using the International Study of Kidney Disease in children), persistence of urine protein >0.2 g/g creatinine for 3 months or urine protein >1 g/g creatinine for 1 month post kidney biopsy.…”

Validation of the children international IgA nephropathy prediction tool based on data in Southwest China

“…Our medical center standardizes the treatment of patients according to the guidelines for the diagnosis and treatment of primary IgA nephropathy published by the Chinese Society of Pediatric Nephrology Medical in 2017 (16). Steroids combined with immunosuppressive therapy were given to children with nephrotic proteinuria, moderate to severe mesangial proliferation, or crescent formation.…”