2020
DOI: 10.1590/2175-8239-jbn-2019-0077 View full text |Buy / Rent full text
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Abstract: Idiopathic membranous nephropathy (IMN) is a frequent cause of nephrotic syndrome in adults. In terms of etiology, the condition may be categorized as primary/idiopathic or secondary. Literature on the pathophysiology of IMN has indicated the presence of autoantibodies (PLA2R and THSD7A) directed against podocyte antigens. The detection of antibodies against a domain favors IMN. The presence of autoantibodies against one of the domains would in theory exclude the possibility of there being autoantibodies again… Show more

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“…The immunohistochemical PLA2R1 positivity was not expected. These histological findings support that PLA2R1 positivity alone is specific for primary MN, as it is also observed, besides syphilis, in chronic Schistosoma mansoni [ 2 ] infection, membranous lupus nephritis [ 3 ] and cancer [ 4 ]. In this regard, PLA2R1 expression can be increased in podocytes by non-specific inflammatory mediators such as tumour necrosis factor-like weak inducer of apoptosis (TWEAK) [ 5 ].…”
supporting
“…The reported frequencies of occurrence of anti-PLA2R and anti-THSD7A antibodies in MN are 50–80% and 5–10% ( 13 ), respectively; the target antigen of MN is uncertain in 10–20% of cases. The presence of one type of antibody does not exclude the presence of other antibody types, as shown by a reported case of anti-PLA2R- and anti-THSD7A-positive MN ( 34 ). It has been suggested that MGN is secondary to an increase in serum IgG4 concentration, which may be due to the deposition of the circulating immune complexes or other unknown mechanisms.…”
Section: Discussionmentioning
“… 7 Although autoantibodies against PLA 2 R and THSD7A are mutually exclusive in most cases of MN, 2 , 8 some reports have demonstrated that these autoantibodies may be concomitantly produced through unknown pathophysiologic mechanisms. 9 , 10 , 11 EXT1/EXT2 were also thought to be exclusive to autoimmune-type secondary MN. 7 In this report, we describe 2 patients with primary MN accompanied by enhanced granular expression of EXT1/EXT2 in glomeruli.…”
Section: Introductionmentioning