Increased Lifetime Risk of ESRD in Familial IgA Nephropathy

Abstract

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Positive renal familial history in IgA nephropathy is associated with worse renal outcomes: a single-center longitudinal study

“…Our observation indicates that the prevalence of positive-FH was 11.6% among histologically proven, primary IgAN cases. The incidence of family clustering is almost comparable to that in previous studies: 6.7% in China [ 8 ] and 17.2–34% in Italy [ 5 ] (Additional file 2 ). Other studies with Caucasians showed a lower frequency of familial aggregation in total sporadic cases: 1.8% in Ireland [ 6 ] and 1.4% in the US [ 15 ].…”

“…A majority of IgAN cases are sporadic, while some show a familial cluster of renal diseases. Familial aggregation of IgAN is observed among IgAN patients of various ethnic backgrounds [ 1 , 3 – 8 ], suggesting the existence of a genetic component to the disease worldwide. In most cases, the clustering pattern is autosomal dominant with variable penetrance; however, some pedigrees may fit with a more complex genetic mode [ 3 ].…”

“…In most cases, the clustering pattern is autosomal dominant with variable penetrance; however, some pedigrees may fit with a more complex genetic mode [ 3 ]. The overall prevalence of positive-FH in all patients with IgAN is reported as 2 to 17% [ 1 , 3 – 8 ], though it might be underestimated in some populations [ 9 ]. There are some controversial results regarding renal outcomes among familial IgAN patients.…”

“…There are some controversial results regarding renal outcomes among familial IgAN patients. Studies on IgAN patients in China and Italy showed that the positive-FH subtype accounts for up to 7 and 17% of all IgAN patients, respectively, and positive-FH was consistently associated with a worse renal prognosis [ 5 , 8 ]. Studies on Irish patients with IgAN reported that the positive-FH subgroup accounts for 1.8% of the total IgAN prevalence and had unfavorable outcomes [ 4 ].…”

Familial Aggregation of CKD: Gene or Environment?

“…For example, a recent study suggested that familial forms of IgA nephropathy are associated with increased lifetime risk of kidney failure. 12 Future studies will need to assess whether a positive family history of CKD also predicts complications of CKD such as progression or risk of cardiovascular disease. Moreover, it would be interesting to determine whether the risk imparted varies across different CKD categories such as glomerular diseases or diabetic nephropathy.…”