IgG4-related kidney disease (IgG4-RKD) with membranous nephropathy as its initial manifestation: report of one case and literature review

Abstract

Background IgG4-related disease (IgG4-RD) often affects multiple organs and tissues, especially the kidneys, and is characterized by interstitial nephritis, obstructive nephropathy, and in rare cases glomerulopathy (including membranous nephropathy). Case presentation In this article, we report a patient with nephrotic syndrome as the only initial manifestation. Membranous nephropathy was confirmed by renal biopsy, but without any renal interstitial lesions. The nephrotic syndrome completely resolved after treatment with immunosuppressants but recurred after drug withdrawal, which was accompanied by acute kidney injury. Ultimately, IgG4-related interstitial nephritis with membranous nephropathy was confirmed by a second renal biopsy. After routine administration of steroids and cyclophosphamide, renal function returned to normal after 2 months, and nephrotic syndrome was ameliorated after 5 months. Conclusion Special attention should be paid to this rare condition in the clinical setting. In patients with membranous nephropathy (MN) that is accompanied by multi-system damage, impaired renal function, elevated IgG4 levels (absolute or relative value), negative PLA2R, and/or renal interstitial plasma cell infiltration, the possibility of IgG4-related kidney disease (IgG4-RKD) should be carefully assessed.

Immune Dysregulation in IgG4-Related Disease

“…Autoimmune pancreatitis (AIP) was the first IgG 4 -RD to be described in 2001 by Japanese scientists Hamano (5)(6)(7), kidneys (8)(9)(10)(11), and lungs (12)(13)(14). With the deepening of research on IgG 4 -RD, an article in Autoimmunity Reviews (15) officially confirmed the existence of this disease in 2010.…”

“…( 4 ) observed infiltration of IgG 4 -secreting plasma cells in extra-pancreatic organs and proposed a new clinicopathological entity that linked AIP and systemic IgG 4 -RD. Since then, an increasing number of IgG 4 -RDs have been discovered in different organs, such as the liver ( 5 – 7 ), kidneys ( 8 – 11 ), and lungs ( 12 – 14 ). With the deepening of research on IgG 4 -RD, an article in Autoimmunity Reviews ( 15 ) officially confirmed the existence of this disease in 2010.…”

A Case of M-Type Phospholipase A2 Receptor-Associated Membranous Nephropathy With IgG4-Positive Cells Infiltration in the Interstitium

“…7 An interesting case of IgG4-RKD in which MN preceded TIN was reported. 15 Therefore, the current case might be diagnosed with MN prior to developing TIN as IgG4-RKD. Recently, a new classification criterion for IgG4-RD was published from ACCR/EULAR.…”

IgG4-related nephritis and interstitial pulmonary disease complicated by invasive pulmonary fungal infection: a case report

“…Since kidney involvement was firstly reported in a patient with IgG4-RD in 2004 [22], many similar cases have been described [23][24][25]. A cross-sectional study reported in 2010 revealed all kidney lesions were associated with extrarenal disease among 114 patients with IgG4-related disease [26].…”