scholarly journals A Case of Acute Renal Failure Associated with IgG4-Related Disease Presenting Both Tubulointerstitial Nephritis and Retroperitoneal Fibrosis

2016 ◽  
Vol 06 (03) ◽  
pp. 86-92
Author(s):  
Hirohito Sugawara ◽  
Hideki Takizawa ◽  
Norihito Moniwa ◽  
Naoki Takamatsu ◽  
Yusuke Ohashi ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Retroperitoneal Fibrosis ◽  
Tubulointerstitial Nephritis ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals IgG4-related kidney disease: a rare cause of tubulointerstitial nephritis

2017 ◽  
Vol 20 (1) ◽  
Author(s):  
Thadathilankal J. John ◽  
Adriano Pellizon ◽  
Wiiliam D. Bates
Keyword(s):  
Renal Failure ◽  
Kidney Biopsy ◽  
Retroperitoneal Fibrosis ◽  
Tubulointerstitial Nephritis ◽  
Obstructive Uropathy ◽  
Immunosuppressive Treatment ◽  
Initial Response ◽  
Response To Therapy ◽  
Inflammatory Mass ◽  
Igg4 Related Disease

A 42-year-old black female was referred with unexplained renal failure. Initial radiological examination revealed a right adnexal inflammatory mass and bilateral hydronephrosis. The renal failure persisted despite the insertion of double J stents. A kidney biopsy showed a lymphoplasmacytic interstitial infiltrate that stained positive for IgG4. A diagnosis of IgG4-related tubulointerstitial nephritis (TIN) with pelvic-limited retroperitoneal fibrosis (RPF) was made and immunosuppressive treatment was initiated, with a good initial response to therapy. This report highlights the importance of considering RPF in cases of unexplained obstructive uropathy and of considering the diagnosis of IgG4-related disease when TIN is found in combination with other organ involvement.

scholarly journals Renal Involvement in IgG4-Related Disease: From Sunlight to Twilight

2021 ◽  
Vol 8 ◽  
Author(s):  
Riccardo Capecchi ◽  
Domenico Giannese ◽  
Diego Moriconi ◽  
Angelo G. Bonadio ◽  
Federico Pratesi ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Plasma Cells ◽  
Retroperitoneal Fibrosis ◽  
Tubulointerstitial Nephritis ◽  
Renal Involvement ◽  
Organ Damage ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Storiform Fibrosis

IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.

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 A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110169
Author(s):  
Kim Rouven Liedtke ◽  
Christoph Käding ◽  
Paula Döring ◽  
Sander Bekeschus ◽  
Anne Susann Glitsch
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Inflammatory Diseases ◽  
Histological Change ◽  
Iliac Vessel ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Cystic Tumors ◽  
Retroperitoneal Cyst ◽  
Retroperitoneal Lymphangioma ◽  
Severe Fibrosis

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.

scholarly journals Erdheim-Chester Disease: The Importance of Information Integration

2017 ◽  
Vol 10 (2) ◽  
pp. 613-619 ◽  
Author(s):  
Anna Nikonova ◽  
Khashayar Esfahani ◽  
Guillaume Chausse ◽  
Stephan Probst ◽  
Tina Petrogiannis-Haliotis ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Braf V600e Mutation ◽  
Braf V600e ◽  
High Dose ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Erdheim Chester Disease ◽  
History Of ◽  
Erdheim Chester ◽  
Chester Disease

Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. Case Presentation: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease. However, the patient was refractory to high-dose steroids and progressed further, developing an epicardial soft tissue mass and recurrent neurological symptoms. Integration of the above findings with new information at another hospital about a radiological history of symmetrical lower extremities long bone lesions raised the differential diagnosis of ECD. Molecular analysis of formalin-fixed paraffin-embedded tissue of both of the patient’s retroperitoneal biopsies (the second one of which had shown a small focus of foamy histiocytes, CD68+/CD1a–) was positive for BRAF mutation, confirming the diagnosis of ECD. The patient demonstrated a dramatic and sustained metabolic response to vemurafenib on follow-up positron emission tomography scans. Conclusion: This case highlights the need for developing a high index of suspicion for presentations of retroperitoneal fibrosis that could represent IgG4-related disease but fail to respond to steroids. When unusual multisystem involvement occurs, one should consider a diagnosis of a rare histiocytosis. Vemurafenib appears to be an effective treatment for even advanced cases of both ECD and Langerhans histiocytosis bearing the BRAF V600E mutation.

scholarly journals Retroperitoneal Fibrosis Diagnosed as IgG4-related Disease after 35 Years

2019 ◽  
Vol 58 (4) ◽  
pp. 609-613 ◽  
Author(s):  
Satoshi Konno ◽  
Yoshihiro Matsuno ◽  
Shingo Ichimiya ◽  
Masaharu Nishimura ◽  
Yoshikazu Kawakami
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Related Disease ◽  
Igg4 Related Disease

Severe acute renal failure due to tubulointerstitial nephritis, pancreatitis, and hyperthyroidism in a patient during rifampicin therapy

2005 ◽  
Vol 22 (3) ◽  
pp. 241-243 ◽  
Author(s):  
Saime Paydas ◽  
Mustafa Balal ◽  
Ibrahim Karayaylali ◽  
Neslihan Seyrek
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Tubulointerstitial Nephritis
Sign in / Sign up

Export Citation Format

Share Document