Combined membranous nephropathy and tubulointerstitial nephritis as a rare renal manifestation of IgG4-related disease: a case-based literature review

Membranous nephropathy (MN) with tubulointerstitial nephritis (TIN) is an established manifestation of immunoglobulin G4 (IgG4)-related disease (IgG4-RD). A pathological feature aiming to distinguish between primary and secondary MN is the presence or absence of glomerular staining for phospholipase A2 receptor (PLA2R), respectively. Isolated MN without TIN has been rarely reported in the context of IgG4-RD. This case report describes a patient with a history of MN successfully treated with steroids and cyclophosphamide, who, 3 years later, presented with unexplained exacerbation of diabetes mellitus due to IgG4-related autoimmune pancreatitis. Pancreatitis was treated, and diabetes improved after treatment with steroids. Based on the presence of isolated IgG4 glomerular capillary deposits along with negative staining for PLA2R and the metachronous appearance of autoimmune pancreatitis, MN was retrospectively classified as secondary to IgG4-RD. Isolated IgG4-positive/PLA2R-negative MN without TIN can be a prodrome of IgG4-RD, reminiscent of MN secondary to neoplasms.

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IgG4-Related Disease Is Not Associated with Antibody to the Phospholipase A2 Receptor

International Journal of Rheumatology ◽

10.1155/2012/139409 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 32

Author(s):

Arezou Khosroshahi ◽

Rivka Ayalon ◽

Laurence H. Beck ◽

David J. Salant ◽

Donald B. Bloch ◽

...

Keyword(s):

Phospholipase A2 ◽

Membranous Nephropathy ◽

Plasma Cells ◽

Tubulointerstitial Nephritis ◽

Renal Involvement ◽

Common Antigen ◽

Related Disease ◽

Definite Relationship ◽

Igg4 Related Disease ◽

Phospholipase A2 Receptor

Patients with IgG4-related disease (IgG4-RD) share histopathological characteristics that are similar across affected organs. The finding of infiltration with IgG4+ plasma cells in the proper clinical and histopathological contexts connects a large number of clinical entities that were viewed previously as separate conditions. The renal involvement in IgG4-RD is usually characterized by tubulointerstitial nephritis, but membranous nephropathy has also been reported to be one of the renal complications of IgG4-RD. The recent discovery that a high proportion of patients with idiopathic membranous nephropathy (IMN) have IgG4 autoantibodies to the M-type phospholipase A2 receptor (PLA2R) in the circulation and glomerular immune deposits, together with the profound IgG4 hypergammaglobulinemia and occasional reports of membranous nephropathy in IgG4-RD, raised the question of a common antigen. To assess the presence of anti-PLA2R antibody in patients with IgG4-RD, we screened sera from 28 IgG4-RD patients by immunoblot. None of the patients in this cohort had detectable circulating anti-PLA2R antibodies. This study suggests that despite some clinical and serological overlaps between IgG4-RD and IMN,anti-PLA2R antibodies do not play a role in the pathogenesis of IgG4-RD. Additional studies of IgG4-RD with evidence of membranous nephropathy are important to exclude any definite relationship.

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Membranous nephropathy as a rare renal manifestation of IgG4-related disease

Indian Journal of Nephrology ◽

10.4103/0971-4065.143300 ◽

2015 ◽

Vol 25 (3) ◽

pp. 164 ◽

Cited By ~ 3

Author(s):

AA Kurien ◽

A Raychaudhury ◽

PD Walker

Keyword(s):

Membranous Nephropathy ◽

Related Disease ◽

Igg4 Related Disease ◽

Renal Manifestation

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Membranous nephropathy associated with IgG4-related disease successfully treated with obinutuzumab

Clinical Kidney Journal ◽

10.1093/ckj/sfab250 ◽

2021 ◽

Author(s):

Noemi E Ginthör ◽

Katharina Artinger ◽

Marion J Pollheimer ◽

Martin H Stradner ◽

Kathrin Eller

Keyword(s):

Complete Remission ◽

Anaphylactic Reaction ◽

Interstitial Nephritis ◽

Membranous Nephropathy ◽

Idiopathic Membranous Nephropathy ◽

Normal Range ◽

Related Disease ◽

Igg4 Related Disease ◽

Renal Manifestation

Abstract IgG4-releated disease is typically associated with interstitial nephritis, but rare cases of idiopathic membranous nephropathy as renal manifestation have been described. Obinutuzumab was successfully used in refractory membranous nephropathy, but evidence for the treatment of IgG4-related disease with obinutuzumab is lacking so far. We report one patient's case with membranous nephropathy associated with IgG4-related disease, who was treated with obinutuzumab following anaphylactic reaction to rituximab. Obinutuzumab treatment resulted in a sustained complete remission of membranous nephropathy and decrease of IgG4 to the normal range. This case demonstrates that membranous nephropathy associated with IgG4-related disease can successfully be treated with obinutuzumab.

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Case Review of lgG4-Related Disease Patient with Combined Tubulointerstitial Nephritis and Membranous Nephropathy Coexisting with Cholangiocarcinoma: Case Report

Journal of the Medical Association of Thailand ◽

10.35755/jmedassocthai.2020.11.10685 ◽

2020 ◽

Vol 103 (11) ◽

pp. 1230-1235

Keyword(s):

Renal Failure ◽

Membranous Nephropathy ◽

Tubulointerstitial Nephritis ◽

Disease Patient ◽

Case Review ◽

Related Disease ◽

Phospholipase A2 Receptor ◽

Wide Range ◽

Serum Igg4 ◽

Secondary Membranous Nephropathy

Immunoglobulin G4-related disease (IgG4-RD) has recently been recognized as an autoimmune disorder involving multiple organs. The kidney is a represented organ with a wide range of renal manifestations. The authors report a case of an 83-year-old Thai male with combined IgG4 tubulointerstitial nephritis and membranous nephropathy coexisting with cholangiocarcinoma. The patient presented with proteinuria, acute renal failure, eosinophilia, hypocomplementemia, and high serum IgG4 concentration. The diagnosis was IgG 4-related tubulointerstitial nephritis and membranous nephropathy on renal biopsy, with negative immunohistochemistry for anti-phospholipase A2 receptor antibodies. Magnetic resonance imaging (MRI) abdomen showed two wedge shaped arterial enhancing lesions of liver. Liver biopsy revealed adenocarcinoma, compatible with cholangiocarcinoma. Proteinuria and renal failure were resolved with initial steroid treatment. Meanwhile, IgG4-related membranous nephropathy should be considered in the differential diagnosis for patients with proteinuria. Potentially, IgG4-RD may be rarely associated with carcinoma development. However, further studies are recommended to ratify and confirm the association between IgG4-RD and incidence of malignancies. Keywords: IgG4-related disease, Membranous nephropathy, Secondary membranous nephropathy, Tubulointerstitial nephritis, Cholangiocarcinoma

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