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"IgG4-related kidney disease"

Ichiro Mizushima, Motohisa Yamamoto, Dai Inoue, Shinichi Nishi, Yoshinori Taniguchi, Yoshifumi Ubara, Shoko Matsui, Tetsuhiko Yasuno, Hitoshi Nakashima, Hiroki Takahashi, Kazunori Yamada, Hideki Nomura, Masakazu Yamagishi, Takao Saito, Mitsuhiro Kawano
BACKGROUND: In immunoglobulin G4-related kidney disease (IgG4-RKD), focal or diffuse renal cortical atrophy is often observed in the clinical course after glucocorticoid therapy. This study aimed to clarify the factors related to renal atrophy after glucocorticoid therapy in IgG4-RKD. METHODS: We retrospectively evaluated clinical features including laboratory data and computed tomography (CT) findings before and after glucocorticoid therapy in 23 patients diagnosed with IgG4-RKD, all of whom were followed up for more than 24 months...
November 25, 2016: Arthritis Research & Therapy
Shigeto Horita, Hiroshi Fujii, Ichiro Mizushima, Yuhei Fujisawa, Satoshi Hara, Kazunori Yamada, Dai Inoue, Kenichi Nakajima, Kenichi Harada, Mitsuhiro Kawano
We describe an 81-year-old man with immunoglobulin G4-related disease (IgG4-RD) presenting with submandibular gland, lymph node, lung, kidney, aortic wall, and prostate lesions with concomitant gastric cancer. After curative surgical treatment of the gastric cancer, corticosteroid therapy for progressively decreasing renal function was started. Before starting steroid therapy, fluorodeoxyglucose positron emission tomography-computed tomography revealed multiple lesions of IgG4-RD but no metastasis of the cancer...
October 27, 2016: Modern Rheumatology
Zeyuan Lu, Jianyong Yin, Hongda Bao, Qiong Jiao, Huijuan Wu, Rui Wu, Qin Xue, Niansong Wang, Zhigang Zhang, Feng Wang
INTRODUCTION: IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that may involve almost each organ or system. IgG4-related kidney disease (IgG4-RKD) refers to renal lesions associated with IgG4-RD. The most frequent morphological type of renal lesions is IgG4-related tubulointerstitial nephritis (IgG4-TIN) which is associated with increased IgG4-positive plasma cell infiltration and interstitial fibrosis. CASE REPORT: Herein, we present a rare case with coexisting IgG4-RKD and acute crescent glomerulonephritis with concomitant severe tubulointerstitial lesions instead of classic IgG4-TIN...
May 2016: Case Reports in Nephrology and Dialysis
Mitsuhiro Kawano, Kazunori Yamada
Immunoglobulin G4-related kidney disease (IgG4-RKD) is the collective name encompassing renal parenchymal and renal pelvic lesions. The hallmark of renal parenchymal lesions of IgG4-related disease is plasma cell-rich tubulointerstitial nephritis with numerous IgG4-positive plasma cells and characteristic fibrosis. In addition, glomerular lesions are sometimes present, with membranous glomerulonephritis being the most common. Although IgG4-RKD shows good responsiveness to corticosteroid therapy, follow-up imaging studies have revealed that partial cortical scars persist when the start of therapy is delayed...
August 2016: Seminars in Liver Disease
Shawna Mann, Michael A Seidman, Sean J Barbour, Adeera Levin, Mollie Carruthers, Luke Y C Chen
PURPOSE OF THE REVIEW: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review. OBJECTIVE: The study aims to describe when IgG4-TIN should be suspected and to summarize the diagnosis, treatment, and natural history of the disease. SOURCES OF INFORMATION: Ovid MEDLINE, Google Scholar, and PubMed were searched for full-text English language articles up to January 2016...
2016: Canadian Journal of Kidney Health and Disease
Satoshi Hara, Mitsuhiro Kawano, Ichiro Mizushima, Kenichi Harada, Takuma Takata, Takako Saeki, Yoshifumi Ubara, Yasuharu Sato, Michio Nagata
IgG4-related kidney disease (IgG4-RKD) occasionally progresses to chronic renal failure and is pathologically characterized by IgG4-positive lymphoplasmacyte-rich tubulointerstitial nephritis with storiform fibrosis (bird's-eye pattern fibrosis). Although radiology reveals a heterogeneous distribution of affected areas in this disease, their true distribution within the whole kidney is still unknown because of difficulty in estimating this from needle biopsy samples. Using 5 autopsy specimens, the present study histologically characterized the distribution and components of interstitial inflammation and fibrosis in IgG4-RKD...
September 2016: Human Pathology
Simone Krebs, Serena Monti, Surya Seshan, Josef Fox, Lorenzo Mannelli
A 64-year-old African American woman with history of stage III breast carcinoma, 3 years after complete response to therapy, presents with progressive fatigue, increasing arthralgia, and unintentional weight loss of 15 lb in 3 months. An F-FDG PET demonstrated new diffuse FDG avidity of the renal parenchyma, new FDG-avid foci in pancreas and lungs, and new FDG-avid lymph nodes above and below the diaphragm. While a retroperitoneal lymph node biopsy was inconclusive, a kidney biopsy resulted in diagnosis of diffuse, severe, IgG4-related tubulointerstitial nephritis...
August 2016: Clinical Nuclear Medicine
Hitoshi Nakashima, Mitsuhiro Kawano, Takako Saeki, Yoshifumi Ubara, Satoshi Hisano, Michio Nagata, Yoh Zen, Motoko Yanagita, Yutaka Yamaguchi, Shinichi Nishi, Takao Saito
BACKGROUND: More than 2 years have passed since the proposal of the diagnostic criteria for IgG4-related kidney disease (IgG4-RKD). The aim of this study was to estimate the number of histological diagnosis for IgG4-RKD throughout Japan and to clarify the regional distribution of the development of this disease. METHODS: A questionnaire was supplied to 140 research facilities registered in the Japan Renal Biopsy Registry (J-RBR). The items of the questionnaire were the total number of renal biopsies performed and the number of cases diagnosed as IgG4-RKD in 2012 and 2013 at each facility...
February 2017: Clinical and Experimental Nephrology
Dario Galeano, Luca Zanoli, Viviana Rosalia Scarfia, Vincenzo L'Imperio, Lorenzo Malatino, Pasquale Fatuzzo, Antonio Granata
IgG4 related disease is a systemic fibro-inflammatory disorder characterized by multiple organ and multiple tissue lesions. The real pathogenesis is currentlyactually unknown. For these reasons many authors compare IgG4 related disease to sarcoidosis. Lesions are often localized in the pancreas, salivary and lacrimal glands, biliary ducts, retroperitoneum and in many other organs. The diagnosisis difficult because of mild symptoms and the possibility of mimicking other severe diseases. Therefore, histopathology together with clinical and radiological typical findings are mandatory tools for diagnosis...
January 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
Wen-Hui Lei, Jun Xin, Chu-Xiao Shao, Ming-Feng Mao, Chao-Yong Zhu, Chui-Fen Wu, Lie Jin
Immunoglobulin G4-related disease is a recently recognized systemic disease that can affect any organ or tissue in the body, including the kidneys. IgG4-related kidney disease (IgG4-RKD) is an important part of immunoglobulin G4-related disease. The most common renal manifestation of IgG4-RKD is tubulointerstitial nephritis and glomerular lesions. There, however, is few case of IgG4-RKD mimicking malignant ureter tumor leading to severe hydronephrosis. We herein report an unusual case of IgG4-RKD mimicking malignancy...
January 2016: Medicine (Baltimore)
N Nithyashree, S Rajagopalan, R A Annigeri, R Mani
Tubulointerstitial nephritis is an uncommon manifestation of IgG4 related disease. A case of tubulointerstitial nephritis with special features including isolated renal involvement in this multisystem disorder and the absence of response to steroid therapy in a young male is reported here. There was no nephromegaly, eosinophilia or other organ involvement. The importance of early detection and treatment for preservation of kidney function is highlighted.
September 2015: Indian Journal of Nephrology
Takeshi Zoshima, Kazunori Yamada, Satoshi Hara, Ichiro Mizushima, Masakazu Yamagishi, Kenichi Harada, Yasuharu Sato, Mitsuhiro Kawano
Multicentric Castleman disease is a benign lymphoproliferative disorder with heterogenous clinical symptoms and involves systemic organs in addition to lymph nodes. Elevated serum IgG4 levels and IgG4-positive plasma cell (IgG4+PC) infiltrates have been reported in lymph nodes, lung and skin in some multicentric Castleman disease cases, resembling IgG4-related disease (IgG4-RD) histologically. However, no report has been available regarding IgG4+PC infiltration in the kidneys of multicentric Castleman disease...
April 2016: American Journal of Surgical Pathology
Kenji Ueki, Yuta Matsukuma, Kosuke Masutani, Akihiro Tsuchimoto, Kiichiro Fujisaki, Kumiko Torisu, Shigeru Tanaka, Tamotsu Kiyoshima, Satoshi Hisano, Takanari Kitazono, Kazuhiko Tsuruya
BACKGROUND: IgG4-related disease is a novel disease entity characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. Typical renal pathology is tubulointerstitial nephritis with storiform fibrosis, although the co-existence of various glomerular lesions has been described. Here, we present the first report of a case of IgG4-related kidney disease and membranoproliferative glomerulonephritis showing the discrepancy in IgG subclasses between the kidney interstitium and glomeruli...
October 26, 2015: BMC Nephrology
Lilian Vasaitis
IgG4-related disease (IgG4-RD) is a recently recognized chronic fibrotic inflammation, which can affect almost every organ, and may come to clinical attention first due to visible organ swelling or organ dysfunction, or is identified incidentally by imaging and specific biopsy. The disorder has an allergic background and is immune-mediated. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in disease progression. About 30-50% of patients are atopic or have mild eosinophilia...
January 2016: European Journal of Internal Medicine
Anxo Martínez-de-Alegría, Sandra Baleato-González, Roberto García-Figueiras, Anaberta Bermúdez-Naveira, Ihab Abdulkader-Nallib, José A Díaz-Peromingo, Carmen Villalba-Martín
Immunoglobulin G4 (IgG4)-related disease is a relatively recently proposed clinical-pathologic entity that is characterized by fibro-inflammatory lesions rich in IgG4-positive plasma cells and, often but not always, elevated serum IgG4 concentrations. IgG4-related disease was recognized as a systemic disease in 2003, when extrapancreatic manifestations were identified in patients with autoimmune pancreatitis. Since then, the disease has been reported as affecting virtually every organ system and has been identified in the biliary tree, salivary and lacrimal glands, periorbital tissues, lungs, lymph nodes, thyroid gland, kidneys, prostate gland, testicles, breasts, and pituitary gland...
November 2015: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Diane Kottler, Stéphane Barète, Gaelle Quéreux, Saskia Ingen-Housz-Oro, Sylvie Fraitag, Nicolas Ortonne, Lydia Deschamps, Michel Rybojad, Béatrice Flageul, Béatrice Crickx, Anne Janin, Martine Bagot, Maxime Battistella
BACKGROUND: Kimura disease (KD) is a rare lymphoproliferative inflammatory disease of unknown etiology. Data regarding therapeutic modalities and pathophysiology are scarce. OBJECTIVES: Analyze therapeutic and follow-up data and compare KD with cutaneous IgG4-related disease (IgG4-RD). METHODS: Multicentric retrospective study of 25 KD patients with analysis of treatment, follow-up and IgG4 immunostaining. Comparison with published cases of cutaneous IgG4-RD...
2015: Dermatology: International Journal for Clinical and Investigative Dermatology
Vijaya B P Ramasamy, Rhiannon Trefor, Kaushik Rajamani, Divya Santosh, David Griffiths, Kieron Donovan
Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition that may involve any organ in the body, including the kidneys. However, renal parenchymal lesions are not seen frequently and the treatment strategy remains unclear. We describe a case of IgG4-related renal mass, which resolved spontaneously. The patient presented with right loin pain, constitutional symptoms and raised inflammatory markers. Magnetic resonance imaging (MRI) showed a large infiltrative mass centered on the right renal hilum and biopsy demonstrated histological changes in keeping with IgG4-RD...
August 2015: Clinical Medicine: Journal of the Royal College of Physicians of London
Koichi Inoue, Takehiko Okubo, Takashi Kato, Kazuo Shimamura, Teruji Sugita, Mitsuhiro Kubota, Kohji Kanaya, Daisuke Yamachika, Makoto Sato, Dai Inoue, Kenichi Harada, Mitsuhiro Kawano
We describe a 74-year-old man with immunoglobulin G4-related disease (IgG4-RD) presenting with gastric cancer, stomach and kidney lesions. For 15 years, the patient had been treated under a diagnosis of sclerosing cholangitis, which was revealed to be IgG4-RD only when the cancer was found. Histology revealed the gastric cancer and IgG4-related lesion in the muscularis propria to be separate. This case suggests that the stomach muscle can also be a site of involvement of IgG4-RD.
September 18, 2015: Modern Rheumatology
Nieun Seo, Jin Hee Kim, Jae Ho Byun, Seung Soo Lee, Hyoung Jung Kim, Moon-Gyu Lee
Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review...
September 2015: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
Ritambhra Nada, Raja Ramachandran, Ashwani Kumar, Manish Rathi, Amit Rawat, Kusum Joshi, Harbir Singh Kohli, Krishan Lal Gupta
AIMS: Immunoglobulin-G4 (IgG4)-related tubulo-interstitial nephritis (IgG4TIN) could be the first presentation of IgG4-related systemic disease. Most of the data is from the West or Japan and retrospective, with good patient outcome. METHODS: This study was carried out from April 2011 to July 2013. We report a prospective follow-up of 11 patients who presented with renal dysfunction and had histological diagnosis of IgG4TIN followed for a minimum period of 1 year or until end-stage renal disease...
July 2016: International Journal of Rheumatic Diseases
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