2018
DOI: 10.2169/internalmedicine.0836-18
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A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis

Abstract: A 58-year-old man with type 1 autoimmune pancreatitis was referred to nephrologists for severe proteinuria. Laboratory data revealed a high serum IgG4 level, hypoalbuminemia, and massive proteinuria, which were compatible with nephrotic syndrome. The renal pathological findings confirmed the diagnosis of secondary membranous nephropathy concurrent with IgG4-related tubulointerstitial nephritis. Despite the improvement of interstitial markers, the proteinuria was refractory to prednisolone, requiring cyclospori… Show more

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Cited by 6 publications
(4 citation statements)
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“…Idiopathic MN was previously described as PLA2R-positive in approximately 70% of MN cases ( 17 ), while MN associated with active sarcoidosis was reported to be positive in 56% (5/9 cases) ( 16 ). In contrast, PLA2R staining was negative in all 13 cases of MN with IgG4-RKD ( 15 , 18 ). Therefore, the negative PLA2R staining observed in the present case was not decisive for a diagnosis of secondary MN.…”
Section: Discussionmentioning
confidence: 88%
“…Idiopathic MN was previously described as PLA2R-positive in approximately 70% of MN cases ( 17 ), while MN associated with active sarcoidosis was reported to be positive in 56% (5/9 cases) ( 16 ). In contrast, PLA2R staining was negative in all 13 cases of MN with IgG4-RKD ( 15 , 18 ). Therefore, the negative PLA2R staining observed in the present case was not decisive for a diagnosis of secondary MN.…”
Section: Discussionmentioning
confidence: 88%
“…The predominant subclass of IgG deposited in the glomerular capillary wall is IgG4, although deposition of other IgG subclasses has also been reported. [15,[21][22][23][24][25][26] Several studies have proposed the involvement of galectin-3, [27] laminin-511, [28] and annexin-A11 [29] in the pathogenesis of IgG4-RD. IgG4 galectin-3 autoantibodies have been found in a subset of IgG4-RD patients and are correlated with plasma levels of galectin-3.…”
Section: Discussionmentioning
confidence: 99%
“…In addition, positive deposition of IgG4 has been observed in IgG4-MN, either predominantly or together with other IgG subclasses [24][25][26][27][28]. Thus, immunoprofile of IgG subclasses would likely not distinguish between primary MN and IgG4-MN [29]. It is worth noting that TBM immune complex deposits are less common in IgG4-MN than in IgG4-TIN.…”
Section: ) Tubulointerstitial Nephritis (Tin)mentioning
confidence: 99%
“…In contrast, glomerular lesions of IgG4-RKD are usually refractory to corticosteroid monotherapy [28,29]. Most cases required combination therapy with immunosuppressive agents to 9 achieve a complete or partial response.…”
Section: Treatment and Outcomementioning
confidence: 99%