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IgG4 related kidney disease

Jie Chang, Wen Zhang
IgG4-related disease (IgG4-RD) is a chronic and systemic disease that can involve multiple organs. The most commonly involved organs include the salivary glands, orbital adnexal structures, paranasal sinus, thyroid, lungs, breasts, aorta, pancreas, biliary ducts, kidneys, retroperitoneum, lymph nodes, prostate, pituitary, and endocranium. Due to increased disease research, several new site-specific nuances of IgG4-RD have been described. The authors have reviewed the recent literature and briefly summarize the infrequent organ involvement of IgG4-RD...
March 3, 2018: Clinical Rheumatology
Toshio Doi, Tatsumi Moriya, Yui Fujita, Naoto Minagawa, Masaru Usami, Tomoko Sasaki, Hideharu Abe, Seiji Kishi, Taichi Murakami, Motoshi Ouchi, Go Ichien, Keiichi Yamamoto, Hiroki Ikeda, Yasuhiko Koezuka, Norimichi Takamatsu, Kenji Shima, Michael Mauer, Kojiro Nagai, Tatsuya Tominaga
Diabetic nephropathy (DN) is the major cause of end-stage kidney disease but early biomarkers of DN risk are limited. Herein we examine urinary immunoglobulin G4 (IgG4) and Smad1 as additional early DN biomarkers. 815 patients with type 2 diabetes mellitus were recruited; 554 patients fulfilled the criteria of more than 60 ml/min of estimated glomerular filtration rate (eGFR) and no macroalbuminuria at baseline with followed-up for 5 years. Patients without macroalbuminuria were also recruited for renal biopsies...
February 28, 2018: Diabetes
Jianchun Xiao, Peiran Xu, Binglu Li, Tao Hong, Wei Liu, Xiaodong He, Chaoji Zheng, Yupei Zhao
Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC...
February 2018: Medicine (Baltimore)
Atsuko Masunaga, Fumihiro Ishibashi, Eitetsu Koh, Takashi Oide, Yasuo Sekine, Kenzo Hiroshima
BACKGROUND: IgG4-related disease often forms a mass and the affected lesion is clinically removed because the mass cannot be differentiated from a neoplasm. Affected lesions commonly occur in the pancreas, hepatobiliary tract, kidney, and retroperitoneum. However, the lesion rarely occurs in the thymus. A histological worldwide consensus of IgG4-related disease proposed that pathological diagnosis of IgG4-related disease should meet more than two of three major features: 1) dense lymphoplasmacytic infiltration with greater than 40% IgG4+/IgG+ plasma cells, 2) storiform fibrosis; and 3) obliterative phlebitis...
January 17, 2018: Diagnostic Pathology
Taku Iguchi, Koji Takaori, Akiko Mii, Yuki Sato, Yasunori Suzuki, Hajime Yoshifuji, Hiroshi Seno, Osamu Ogawa, Koichi Omori, Kazuhisa Bessho, Satoru Kondo, Tomokazu Yoshizaki, Hitoshi Nakashima, Takao Saito, Tsuneyo Mimori, Hironori Haga, Mitsuhiro Kawano, Motoko Yanagita
Immunoglobulin G4-related disease is a rare immune-mediated disease characterized by the infiltration of IgG4-positive plasma cells and unique storiform fibrosis of multiple organs. The majority of IgG4-related disease patients respond to glucocorticoids, yet the precise mechanism of their action remains unclear. Pathological sections of the submaxillary gland, kidney, and retroperitoneum from 20 patients in total diagnosed with IgG4-related disease were analyzed for glucocorticoid receptor expression and the cell types expressing glucocorticoid receptor...
February 12, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Wei Zhang, Feng Xue, Cui Wang, Leping Shao
IgG4-related retroperitoneal fibrosis (IgG4-RPF) is a newly recognized entity which often mimics cancer. We aimed to investigate the clinical features and the causes of renal failure, as well as to explore the factors affecting the prognosis of renal function by analysis of the clinical data of patients with IgG4-RPF. We reviewed clinical features of 10 patients with renal failure caused by IgG4-RPF, which was confirmed by pathology review and clinic-pathologic correlations. All patients were male, and the mean age at onset was 64...
January 5, 2018: Oncotarget
Wei Zhang, Jeffrey H Glaze, David Wynne
IgG4-related disease (IgG4-RD) is a newly recognized immune-mediated multisystemic disease characterized by a fibro-inflammatory condition with tissue infiltration of IgG4-positive plasma cells and often associated with elevated serum IgG4 levels. Typical renal involvement of IgG4-RD presents as tubulointerstitial nephritis (TIN), membranous or membranoproliferative nephropathy. We are presenting a case with combined IgG4 membranous nephropathy and TIN, as well as a literature review on pathophysiology, diagnosis and treatment of IgG4-RD...
February 1, 2018: CEN Case Reports
Maurizio Salvadori, Aris Tsalouchos
This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases (IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis...
January 6, 2018: World Journal of Nephrology
Smita Mary Matthai, Anjali Mohapatra, Raiyani Palak, Gopal Basu
Immunoglobulin G4-related tubulointerstitial nephritis (IgG4-TIN) is a newly recognized clinicopathological entity characterized by a dense interstitial infiltrate of IgG4-positive plasma cells accompanied by fibrosis and obliterative phlebitis causing acute or chronic renal dysfunction amenable to corticosteroid therapy. IgG4-TIN is the dominant manifestation of renal involvement in IgG4-related disease (IgG4-RD) which is a novel, immune-mediated, fibroinflammatory and multiorgan disorder. We describe a case of IgG4-TIN with isolated renal involvement in an elderly male patient with poor response to corticosteroid therapy...
October 2017: Indian Journal of Pathology & Microbiology
Luis Del Carpio-Orantes
The case of a patient affected by transient diabetes insipidus associated with pregnancy, in the context of eclampsia, which was presented during seizures and identified by polyuria important, as well as changes in the urinary density occurs, and improving after nasal administration of desmopressin, which confirmed the diagnosis and treatment served completely by sending the picture without any sequel.
September 2017: Revista Médica del Instituto Mexicano del Seguro Social
Kazunori Yamada, Motohisa Yamamoto, Takako Saeki, Ichiro Mizushima, Shoko Matsui, Yuhei Fujisawa, Satoshi Hara, Hiroki Takahashi, Hideki Nomura, Shigeyuki Kawa, Mitsuhiro Kawano
BACKGROUND: The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. METHODS: Various specialists retrospectively evaluated IgG4-RD patients diagnosed between 1996 and 2015 in five hospitals by analyzing their baseline clinical features, laboratory, imaging, and pathological test findings, and treatment...
December 1, 2017: Arthritis Research & Therapy
Luis Francisco Pineda-Galindo, Olga Lidia Vera-Lastra, Lucía Carrazco-Ibarra, Alejandra Carrasco-Gómez, Martín Becerril-Ángeles, Patricia Ramos-Salazar, Jaime Martínez-Solano
IgG4-related disease is an inflammatory condition characterized by high levels of IgG4. It affects salivary and lacrimal glands, pancreas, lymph nodes, lungs or kidney. The diagnosis is based on identifying a histological pattern with a dense lymphocyte and plasmacyte infiltration, focal fibrosis or phlebitis, finding more than 10 IgG4 positive cells per high power field and/or IgG4/IgG ratio in plasma higher than 40%. We present a patient with Mikulicz's disease who meets histological findings required for the diagnosis of IgG4 related disease...
November 2017: Revista Médica del Instituto Mexicano del Seguro Social
Michael Girsberger, Jörg P Halter, Helmut Hopfer, Michael Dickenmann, Thomas Menter
Hematopoietic cell transplantation (HCT) is an increasingly used treatment for hematologic malignancies as well as for nonmalignant diseases. Kidney impairment remains an important early and late post-transplantation complication. Although numerous histopathological changes have been reported, the pathophysiology remains incompletely understood. Furthermore, correlations between clinical findings and morphological changes have not been well studied. Between 2000 and 2016, 17 recipients of allogeneic (n = 12) or autologous (n = 5) HCT underwent kidney biopsy for either proteinuria or deterioration of kidney function at our center...
March 2018: Biology of Blood and Marrow Transplantation
Ke Zheng, Fei Teng, Xue-Mei Li
Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized clinical entity that often involves multiple organs; it is characterized by high levels of serum immunoglobulin G4 (IgG4), dense infiltration of IgG4+ cells, and storiform fibrosis. Cellular immunity, particularly T cell-mediated immunity, has been implicated in the pathogenesis of IgG4-RD. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulonephropathy (MGN), and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis, prostatitis, or ureter inflammation...
September 2017: Chronic diseases and translational medicine
Miroslav Průcha, Lenka Sedláčková
IgG4-related diseases represent a heterogeneous group of conditions characterised by elevated serum IgG4 levels and fibrotic or sclerosing changes in the affected organs or systems accompanied by IgG4-positive plasma cells. A disease associated with IgG4 may affect virtually any organ - salivary glands, periorbital tissue, kidneys, lungs, meninges, aorta, prostate, pericardium or skin. Histopathological findings are uniform, characterised by a major lymphoplasmocytic infiltrate and the presence of IgG4-producing plasma cells, irrespective of the affected site...
2017: Prague Medical Report
Inbar Raber, Asiri Ediriwickrema, John Higgins, Neeraja Kambham, Alan C Pao
Immunoglobulin G4 (IgG4)-related disease is an uncommon autoimmune disease that affects multiple organ systems. Renal involvement typically presents as tubulointerstitial nephritis and less commonly as membranous glomerulonephritis. In this case report, we discuss a 68-year-old patient who presented with rapidly progressive glomerulonephritis. His renal biopsy revealed a membranoproliferative pattern of injury with fibrocellular crescents and extensive infiltration of the tubulointerstitium with IgG4-positive plasma cells...
September 2017: American Journal of the Medical Sciences
Daniele Bianchi, Luca Topazio, Gabriele Gaziev, Valerio Iacovelli, Pierluigi Bove, Alessandro Mauriello, Enrico Finazzi Agrò
IgG4-related disease (IgG4-RD) is a nosological entity defined as a chronic immune-mediated fibro-inflammatory condition characterized by a tendency to form tumefactive, tissue-destructive lesions or by organ failure. Urologic involvement in IgG4-RD has been described in some short series of patients and in isolated case reports, most often involving the kidneys in so-called IgG4-related kidney disease (IgG4-RKD). The disease can occasionally mimic malignancies and is at risk of being misdiagnosed due to its rarity...
2017: Case Reports in Surgery
P P Zhang, J Z Zhao, M Wang, R E Feng, X W Liu, X M Lai, X J Li, J G Zeng, H J Shi, H D Zhu, W Xue, H Zhang, Y Y Chen, L Y Fei, X F Peng, F C Zeng, Y M Zhang, W Zhang
Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Victoria Y Y Xu, Mary Bell, Alireza Zahirieh, Janey Hsiao, Kevin Higgins, Zeina Ghorab, Arthur Bookman, Pak Cheung Chan
A 76-year-old man was incidentally found on a CT scan to have lymphadenopathy and bilateral kidney enlargement suggestive of infiltrative renal disease. He was largely asymptomatic but had bilateral salivary and lacrimal gland enlargement. A grossly elevated serum IgG (>70 g/L) with concomitant suppression of other immunoglobulins, a small IgG restriction, and a parotid biopsy revealing lymphoplasmacytic infiltrate with slight kappa light chain excess all suggested a lymphoproliferative disorder (LPD)...
2017: Case Reports in Medicine
Hiroyuki Ono, Taichi Murakami, Akira Mima, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Sayo Ueda, Fumi Kishi, Seiji Kishi, Takashi Kawanaka, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Masashi Harada, Toshio Doi
BACKGROUND: Immunoglobulin G4-related kidney disease characterized by immunoglobulin G4-positive plasma cell-rich tubulointerstitial nephritis has distinctive serological and radiological findings. Renal prognosis is good because of a good response to glucocorticoids. Here we report a case of successful treatment of highly advanced immunoglobulin G4-related kidney disease presenting renal mass-like regions with end-stage kidney failure. CASE PRESENTATION: A 59-year-old Japanese man was referred to our hospital because of uremia with a creatinine level of 12...
August 3, 2017: BMC Nephrology
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