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

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

scholarly journals PLA2R-Negative Membranous Nephropathy Presenting as a Prodrome of IgG4-Related Autoimmune Pancreatitis

2019 ◽  
Vol 9 (2) ◽  
pp. 85-91
Author(s):  
Magdalini Velegraki ◽  
Kostas G. Stylianou ◽  
Dimitrios Xydakis ◽  
Hariklia Gakiopoulou ◽  
Evangelos Voudoukis ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Autoimmune Pancreatitis ◽  
Membranous Nephropathy ◽  
Tubulointerstitial Nephritis ◽  
Pathological Feature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Phospholipase A2 Receptor ◽  
History Of

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.

Clinical case of membranous nephropathy in a patient with IgG4-related disease

2020 ◽  
Vol 24 (4) ◽  
pp. 110-121
Author(s):  
M. O. Pyatchenkov ◽  
O. A. Vorobyeva ◽  
A. N. Belskykh ◽  
M. V. Zakharov ◽  
M. Y. Dendrikova
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
The Body ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pathologic Features ◽  
Wide Range ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) currently is considered as a chronic fibroinflammatory immune-mediated multisystemic condition of unidentified etiology, which can imitate a wide range of malignant, infectious, rheumatologic, and other diseases. It can affect almost any organ system in the body synchronously or sequentially, but the most often affected are the pancreas, hepatobiliary tract, periorbital structures, salivary glands, kidneys, and lymph nodes. The most frequent renal manifestations of IgG4-RD is IgG4-related tubulointerstitial nephritis. Membranous nephropathy is the most common glomerular disease accompanied by IgG4-RD. Regardless of the organ localization, patients with IgG4-RD are characterized by elevated serum IgG4, but this laboratory abnormality is not specific and can be changed in other diseases. In all suspected cases of IgG4-RD the diagnosis should be confirmed by histological examination. Characteristic pathologic features include diffuse or focal lymphoplasmacytic infiltration with prominent IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. Patients with IgG4- RD usually have an excellent clinical response to glucocorticoids, but relapse rates after steroid withdrawal are high, which may require additional use of immunosuppressants or rituximab. Due to the low prevalence and multitude of clinical manifestations the disease often remains underdiagnosed on time. This case report describes middle-aged patients with a history of chronic recurrent pancreatitis complicated by the nephrotic syndrome. Kidney biopsy showed membranous nephropathy and diagnosis IgG4-RD with multiorgan involvement was made. Partial remission was achieved on corticosteroid therapy. The presented case clearly demonstrates the difficulties of diagnosis and treatment of IgG4-RD. IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with nephrotic syndrome accompanied by multiorgan dysfunction.

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

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
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.

Membranous glomerulonephritis

2018 ◽  
Author(s):  
Daniel C. Cattran ◽  
Heather N. Reich
Keyword(s):  
Membranous Nephropathy ◽  
Adult Life ◽  
Membranous Glomerulonephritis ◽  
Adult Onset ◽  
Common Cause ◽  
End Stage Renal Failure ◽  
Surface Molecules ◽  
Phospholipase A2 Receptor ◽  
Secondary Membranous Nephropathy ◽  
End Stage

Membranous glomerulonephritis (MGN) is the most common cause of adult-onset nephrotic syndrome, and a common glomerular cause of end-stage renal failure. It is caused by antibodies to podocyte surface molecules, usually autoantibodies. In most patients with primary membranous nephropathy the target is the phospholipase A2 receptor. It is hoped that robust assays for this antibody will help to guide therapy but it has not been possible to test this adequately yet. Primary MGN accounts for about 70% of cases with regional variations. MGN is more common in men than women (approximately 2:1) and its peak incidence is in middle adult life. Secondary membranous nephropathy occurs in lupus and some other immune or autoimmune disorders, in hepatitis B infection, after exposure to some drugs or toxins, and in some cancers.

scholarly journals Serum Antibody and Glomerular Antigen of Antiphospholipase A2 Receptor in Chinese Patients with Idiopathic Membranous Nephropathy

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Qiu-hua Zhang ◽  
Mian Wu ◽  
Zhi-gang Hu ◽  
Xiao-bin Liu ◽  
Biao Huang ◽  
...  
Keyword(s):  
Renal Function ◽  
Serum Creatinine ◽  
Serum Albumin ◽  
Membranous Nephropathy ◽  
Serum Antibody ◽  
Chinese Patients ◽  
Idiopathic Membranous Nephropathy ◽  
Phospholipase A2 Receptor ◽  
Secondary Membranous Nephropathy ◽  
Clinical Correlations

Background. M-type phospholipase A2 receptor (PLA2R) is the first autoantigen responsible for idiopathic membranous nephropathy (IMN). However, serum PLA2R antibody (PLA2R-Ab) can be inaccurate in distinguishing between IMN and secondary membranous nephropathy, while renal PLA2R antigen (PLA2R-Ag) emerges as an ancillary diagnostic. The present study is aimed at examining the associations between PLA2R-Ab in sera and PLA2R-Ag in kidneys in IMN patients. Methods. A total of 93 patients with IMN were retrospectively identified. Their serum PLA2R-Ab and renal PLA2R-Ag expression levels were determined, and the clinical correlations between these parameters and clinical features were examined. Results. The sensitivities of serum PLA2R-Ab and renal PLA2R-Ag for diagnosing IMN were 74.2% and 88.2%, respectively (P<0.001), with poor consistency. Higher serum PLA2R-Ab levels were correlated to stronger renal PLA2R-Ag expression (P=0.048). Patients with positive PLA2R-Ab significantly differed from those with negative levels, in terms of proteinuric levels over 24 hours (4.54 vs. 3.46 g/day, P=0.015) and serum albumin (23.28 vs. 27.95 g/L, P=0.038). Among patients with positive renal PLA2R-Ag, patients with positive PLA2R-Ab had significantly higher 24-hour proteinuria, when compared to patients with negative PLA2R-Ab (4.57 vs. 3.08 g/day, P=0.005). Among those with positive PLA2R-Ab in sera, their PLA2R-Ab levels were correlated with the estimated glomerular filtration and serum creatinine. Conclusion. Serum PLA2R-Ab exhibits a closer correlation with proteinuric severity and renal function, when compared to renal PLA2R-Ag.

scholarly journals Immunoglobulin G4 related disease: an overview

2021 ◽  
Vol 15 (2) ◽  
pp. 44-51
Author(s):  
Saika Farook ◽  
Abdullah Ahmed Solaiman ◽  
Md Shariful Alam Jilani
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Tubulointerstitial Nephritis ◽  
The Body ◽  
Histopathological Analysis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Serum Igg4 ◽  
First Time ◽  
Gland Enlargement

Immunoglobulin G4 related disease (IgG4-RD) is a recently perceived fibroinflammatory condition, identified as a systemic illness for the first time in the early 2000. It can involve virtually every organ of the body, commonly presenting as lymphadenopathy, retroperitoneal fibrosis, autoimmune pancreatitis, tubulointerstitial nephritis, parotid or lacrimal gland enlargement. The diagnosis is confirmed by histopathological analysis and is often, but not always accompanied by an increased level of serum IgG4 concentration. In fact, the name addressing this autoimmune fibroinflammatory condition may be considered a misnomer, as the role of the non-inflammatory immunoglobulin IgG4 in the immune mechanism of IgG4-RD remains to be elucidated. Ibrahim Med. Coll. J. 2021; 15(2): 44-51

scholarly journals A Refractory Case of Secondary Membranous Nephropathy Concurrent with IgG4-related Tubulointerstitial Nephritis

2018 ◽  
Vol 57 (19) ◽  
pp. 2873-2877 ◽  
Author(s):  
Hiroyuki Arai ◽  
Naohiro Toda ◽  
Ryo Kamimatsuse ◽  
Keisuke Nishioka ◽  
Shuichiro Endo ◽  
...  
Keyword(s):  
Membranous Nephropathy ◽  
Tubulointerstitial Nephritis ◽  
Secondary Membranous Nephropathy ◽  
Refractory Case

scholarly journals Phospholipase A2 receptor–associated membranous nephropathy in a patient with IgG4-related disease

Medicine ◽  
2019 ◽  
Vol 98 (20) ◽  
pp. e15616 ◽  
Author(s):  
Saif A. Muhsin ◽  
Ricard Masia ◽  
Rex N. Smith ◽  
Zachary S. Wallace ◽  
Cory A. Perugino ◽  
...  
Keyword(s):  
Phospholipase A2 ◽  
Membranous Nephropathy ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Phospholipase A2 Receptor

scholarly journals A245 IGG4- RELATED DISEASE AS A RARE CAUSE OF GASTRIC OUTLET OBSTRUCTION: A CASE REPORT AND LITERATURE REVIEW

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 303-304
Author(s):  
A Almudaires ◽  
M Alzahrani ◽  
L Chen ◽  
K Qumosani
Keyword(s):  
Abdominal Pain ◽  
Gastric Outlet Obstruction ◽  
Outlet Obstruction ◽  
Nausea And Vomiting ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Duodenal Stricture ◽  
Wide Range ◽  
Serum Igg4 ◽  
Inflammatory Changes

Abstract Background IgG4- related disease (IgG4-RD) is a newly recognized systemic fibroinflammatory condition that can affect a wide range of organs, including the pancreas, biliary system, retroperitoneum, lymph nodes and salivary glands. However, gastrointestinal luminal involvement is very rare, and the presentation with isolated gastrointestinal obstruction is extremely unusual. Aims We present a case of IgG4-RD presenting with gastric outlet obstruction secondary to severe duodenal stricture. Methods A 59-year-old female presented with 6 months history of abdominal pain, postprandial nausea and vomiting associated with significant weight loss. CT showed circumferential wall thickening of the 2ndand 3rd parts of the duodenum with gastroscopy showing severe ulcerated duodenal stricture that could not be passed through. Biopsies from the ulcerated area did not reveal a specific etiology, but malignancy could not be excluded, and the decision was made to pursue surgical management with Whipple’s procedure. Surgical pathology revealed IgG4 related disease in the form of an ulcerated gastric mass invading the duodenum and the pancreas with normal serum IgG4 level. Postoperatively, the patient developed anastomotic leak that was managed conservatively with antibiotics and drainage resulting in significant improvement in her symptoms. MRCP did not show any pancreatic or biliary abnormalities. Results A few weeks later, she represented with abdominal pain as well as nausea and vomiting. CT scan revealed severe inflammatory changes at the anastomosis site with mucosal thickening concerning for persistent leak. However, as she was optimally treated before, it was concluded that these changes are likely related to recurrent IgG4-RD. To induce remission, prednisone was started with remarkable improvement in her symptoms within two weeks, and complete resolution of the previous inflammatory changes around the anastomosis on repeat imaging. Subsequently, she was started on mycophenolate mofetil (MMF) with a slow prednisone taper. Conclusions IgG4-RD involving the gastrointestinal tract is rare with rare cases reported in the literature presenting in variable ways. Our case demonstrates the possibility of IgG4-RD presenting as gastric outlet obstruction; IgG4-RD should be considered in the differential diagnosis of unexplained duodenal stricture or gastric outlet obstruction. IgG4-RD usually responds to steroids but long-term response rates to steroid-sparing agents, especially in the subset of patients with luminal IgG4-RD, remains to be seen. Funding Agencies None

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