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Rishi Bolia, Sze Yee Chong, Lee Coleman, Duncan MacGregor, Winita Hardikar, Mark R Oliver
We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children...
July 2016: ACG Case Reports Journal
Karla Lais Pêgas, Eduardo Cambruzzi, Gisele Lobato
IgG4-related disease (IgG4RD) is a recent inflammatory process of supposed autoimmune etiology, which is characterized by elevated serum IgG4 levels, dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells and storiform fibrosis. Tubulointerstitial nephritis is the most common renal manifestation, with different degrees of kidney dysfunction and variable clinical findings. Herein, the authors describe a new case of IgG4 tubulointerstitial nephritis (IgG4TN), and discuss clinic and pathologic criteria...
June 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
F Moosig, J H Schirmer, P Lamprecht, J U Holle
Rheumatologist should be familiar with the concept of IgG4-related disease (IgG4-RD). Due to the clinical spectrum IgG4-RD can fall directly within the scope of rheumatology and are often diagnosed primarily by rheumatologists. Furthermore, IgG4RD are relevant differential diagnoses for many other rheumatic conditions. Finally, there are an increasing amount of data suggesting an important role of immunological processes observed in IgG4-RD for other rheumatic diseases.
September 2016: Zeitschrift Für Rheumatologie
Abhijit Chougule, Amanjit Bal
OBJECTIVES: There is marked inconsistency in reporting the key features of IgG4-related inflammatory pseudotumor (IPT) cases. We aimed to analyze the various aspects of IgG4-related IPTs and to test the performance of the consensus criteria for their diagnosis. METHODS: PubMed database was searched for IgG4-related IPT cases. The data regarding patient demographics, clinical presentation, laboratory findings, histopathological features, and treatment response are extracted and are presented here in a descriptive manner...
July 14, 2016: Modern Rheumatology
Davide Gianfreda, Claudio Musetti, Maria Nicastro, Federica Maritati, Rocco Cobelli, Domenico Corradi, Augusto Vaglio
Immunoglobulin-G4 (IgG4)-related disease (IgG4RD) is a fibro-inflammatory disorder characterized by tissue-infiltrating IgG4 plasma cells, and, often, high serum IgG4. Several autoimmune, infectious, or proliferative conditions mimic IgG4RD. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by foamy histiocytic infiltration, fibrosis, and chronic inflammation. ECD and IgG4RD manifestations may overlap.A patient presented with huge fibrous retroperitoneal masses causing compression on neighboring structures; the case posed the challenge of the differential diagnosis between IgG4RD and ECD mainly because of a prominent serum and tissue IgG4 response...
May 2016: Medicine (Baltimore)
W Egner, K Swallow, R J Lock, D Patel
Immunoglobulin (Ig)G4 disease can have apparently 'normal' levels of IgG4 due to antigen excess conditions. IgG4 measurement therefore appears falsely low. UK National External Quality Assurance Scheme (UK NEQAS) data and other reports have suggested that this problem occurred despite pre-existing antigen excess detection steps. To determine the clinical relevance of the problem, we examined the prevalence and characteristics of prozoning in our laboratory and patient cohorts. We establish that the prevalence of raised IgG4 in routine IgG4 analysis is low (< 1%) using one of the two routine methods in use in the United Kingdom...
October 2016: Clinical and Experimental Immunology
Hong Ryeol Cheong, Bong Eun Lee, Geun Am Song, Gwang Ha Kim, Sung Gyu An, Won Lim
Immunoglobulin G4 (IgG4)-related disease (IgG4RD) is a relatively recently recognized entity that is histopathologically characterized by an extensive infiltration of lymphocytes and IgG4-positive plasma cells with dense fibrosis. IgG4RD is now known to affect any organ system, and a few cases of gastrointestinal lesions have also been reported. However, solitary IgG4RD of the stomach is still very rare. Furthermore, as it can mimic malignant conditions, it is important to recognize this disease to avoid unnecessary surgery...
March 2016: Clinical Endoscopy
Mitsuru Sugimoto, Hiroshi Watanabe, Tomoyuki Asano, Shuzo Sato, Tadayuki Takagi, Hiroko Kobayashi, Hiromasa Ohira
OBJECTIVE: To investigate which IgG subclasses contribute to the activation of the complement pathway in IgG4-related disease (IgG4RD) patients with hypocomplementemia. METHODS: Sera of IgG4RD patients were analyzed for the binding ability of IgG subclasses to complement component 1q (C1q). Polyethylene glycol (PEG) precipitates containing immune complexes (ICs) in sera of IgG4RD patients were analyzed for IgG subclass composition by Western blotting. PEG precipitates containing ICs (PEG-ICs) in sera of patients were also analyzed for their ability to consume complement in normal human serum (NHS) using a total complement hemolytic (CH50) assay and a commercial kit to measure the complement capacity of all three individual complement pathways...
2016: Modern Rheumatology
Abhijit Chougule, Amanjit Bal, Ashim Das, Gurpreet Singh
IgG4 related disease (IgG4RD) is a recently recognised condition characterised by mass forming lesions associated with storiform fibrosis, obliterative phlebitis, lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells and elevated serum IgG4 levels. Although rare, mammary involvement has been reported as IgG4 related sclerosing mastitis, the morphological counterpart of a growing family of IgG4 related diseases. A total of 17 cases belonging to mass forming benign inflammatory breast lesions such as plasma cell mastitis, granulomatous lobular mastitis, non-specific mastitis and inflammatory pseudotumour were investigated as a possible member of IgG4 related sclerosing mastitis...
January 2015: Pathology
Fang Fang, Yanming Li, Mingjun Sun, Songtao Hu, Zheng Wang, Dongge Liu, Chen Wang
OBJECTIVE: To evaluate the clinical and pathological features of IgG4-related disease (IgG4RD). METHODS: The clinical data, laboratory profiles, radiological, pathological and therapeutic features of eight cases of IgG4RD were analyzed. This cohort included two cases of common bile duct and partial hepatectomy specimens, two of submandibular gland excision specimens, one from lung biopsy specimen, one from open lung biopsy specimen, one from renal biopsy specimen, and one from renal excision specimen...
September 2014: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Clio P Mavragani, George E Fragoulis, Dimitra Rontogianni, Maria Kanariou, Haralampos M Moutsopoulos
OBJECTIVE: To determine IgG4 levels in a cohort of consecutive patients with primary Sjögren's syndrome (SS) and other autoimmune diseases and explore whether they associate with distinct clinical, serologic, and histopathologic features. METHODS: Serum IgG4 levels were measured in 133 primary SS patients and 49 healthy donors (HDs). Seventy-four lupus and 54 rheumatoid arthritis (RA) patients served as disease controls. Immunohistochemical IgG4 analysis was performed in paraffin-embedded minor salivary gland (MSG) tissues...
May 2014: Arthritis Care & Research
Naoko Ito, Kunimasa Yagi, Mitsuhiro Kawano, Yukiko Mori, Satoko Okazaki, Daisuke Chujo, Yoshiyu Takeda, Junji Kobayashi, Masakazu Yamagishi
IgG4-related disease (IgG4RD) is a newly recognized systemic disease characterized by the elevation of serum IgG4 levels and abundant IgG4-positive plasma cell infiltration into the involved organs. Few data exist regarding the relationship between diabetes or glucose intolerance and IgG4RD in the absence of obvious type 1 autoimmune pancreatitis (AIP). Therefore, we are characterizing pancreatic endocrine function in IgG4RD patients with no signs of type 1 AIP. 28 patients (12 men, mean age 62.1 years old) were diagnosed as having IgG4RD from serum IgG4 levels, histopathology and images...
2014: Endocrine Journal
Rachel B Cain, Thomas V Colby, Vijayan Balan, Naresh P Patel, Devyani Lal
OBJECTIVE: IgG4-related disease (IgG4RD) causing sinonasal and skull base pathology is uncommonly described. We present a series of suspected IgG4RD patients, with a pertinent review of the literature to highlight diagnostic challenges. STUDY DESIGN: Case series. SETTING: Academic tertiary care center. SUBJECTS AND METHODS: Case series of patients with IgG4RD or suspected IgG4RD involving the sinonasal cavity and skull base...
September 2014: Otolaryngology—Head and Neck Surgery
Andre Mattman, Sophia Wong, Nadine Urquhart, Patrick Wong
OBJECTIVE: IgG4 related disease (IgG4RD) is detected in part by measurement of serum IgG4 levels in patients suspected to have the condition. The immunonephelometric methods that are used to measure serum IgG4 are susceptible to hook effect errors. The quality control parameter IgGRDiff=100∗[Sum (IgG1, IgG2, IgG3, IgG4)-Total IgG]/Average [Sum (IgG1, IgG2, IgG3, IgG4), Total IgG] is theoretically of use in detecting large IgG4 hook effect. We evaluated the IgGRDiff parameter in this context...
August 2014: Clinical Biochemistry
Satoshi Yazawa, Rei Ohara, Takahiro Maeda, Kent Kanao, Seiya Hattori, Yosuke Nakajima, Mototsugu Oya
IgG4-related disease (IgG4RD) is a novel clinical entity characterized by tissue infiltration of IgG4-positive plasma cells. We report here 3 cases of IgG4RD associated with urinary tract obstruction. Patient 1 was a 59-year-old male who complained of difficulty on urination. A CT scan showed bilateral ureteral wall thickness, hydronephrosis, and an enlarged prostate. His serum IgG4 was 817 mg/dl. We made a diagnosis of IgG4RD and performed bilateral ureteral stenting and steroid therapy. A significant reduction in the size of the lesion was detected, and IgG4 was decreased to 272 mg/dl...
July 2013: Nihon Hinyōkika Gakkai Zasshi. the Japanese Journal of Urology
Takaaki Ogoshi, Takashi Kido, Kazuhiro Yatera, Keishi Oda, Toshinori Kawanami, Hiroshi Ishimoto, Noriho Sakamoto, Arisa Sano, Chiharu Yoshii, Shohei Shimajiri, Hiroshi Mukae
BACKGROUND: IgG4-related disease (IgG4RD) is a recently recognized disease entity. Differentiating IgG4RD from plasma cell type Castleman's disease (PCD) is important but also difficult using only pathological findings. In addition, little is known about the association between these two diseases with diffuse parenchymal lung involvement. METHODS: We analyzed the serum IgG4 levels and the ratio of IgG4/IgG-positive plasmacytes in the lung and lymph node specimens of eight patients previously pathologically diagnosed of PCD with diffuse parenchymal lung involvement (DL-PCD)...
December 2013: Lung
Wei Lin, Hua Chen, Qing-jun Wu, Wen-jie Zheng, Qun Shi, Xiao-mei Leng, Dong Xu, Min Shen, Wen Zhang, Yan Zhao, Xiao-feng Zeng, Feng-chun Zhang
OBJECTIVE: To explore the clinical features of immunoglobin (IgG4)-related Mikulicz's disease (MD). METHODS: Since December 2010, a prospective cohort study of IgG4 related disease (IgG4RD) was performed our hospital. A total of 42 IgG4RD patients were recruited along with 18 MD patients. Their clinical, laboratory and histopathological features and response to treatment were analyzed. RESULTS: The 18 MD patients accounted for 42.9% of IgG4RD...
April 2, 2013: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Tetsuya Makiishi, Tomoyuki Shirase, Nobuhiro Hieda, Sayako Maeda
A 56-year-old man was admitted to our hospital for renal dysfunction and symmetrical swelling of submandibular glands. Laboratory and imaging findings were consistent with immunoglobulin G4-related disease (IgG4RD). Histological findings of the submandibular gland and the kidney were also consistent with IgG4RD. However, the patient did not have elevated serum or tissue IgG4 levels. Oral prednisolone therapy, initially 50 mg/day and gradually tapered over 12 months, improved his laboratory abnormalities and the swelling of his affected organs...
2013: BMJ Case Reports
Minerva Lazos Ochoa, Belem Gabiño López, Raúl Romero Cabello, Raúl Romero Feregrino
IgG4-related disease (IgG4RD) is a chronic recurring fibro-inflammatory pathology that is considered to be of autoimmune origin. Histopathology is considered to be the gold standard method for diagnosis. IgG4RD affects multiple organs. IgG4RD was first identified in the pancreas and was called autoimmune pancreatitis (AIP). During the following years, the disease spectrum was expanded and it was realised that the extrapancreatic lesions can precede, coexist or appear after the diagnosis of AIP. At present, several illnesses such as Mikulicz disease, Küttner tumour, multifocal fibrosclerosis, etc, are considered to be part of the IgG4RD spectrum...
2013: BMJ Case Reports
Hirofumi Takei, Hayato Nagasawa, Ryota Sakai, Koji Nishimura, Takahiko Kurasawa, Ayumi Okuyama, Eiko Nishi, Yuichiro Shirai, Tsuneo Kondo, Hiroe Ogawa, Tatsuya Ito, Koichi Amano
IgG4-related disease (IgG4RD) is a unique systemic lymphoproliferative disorder characterized by elevated serum IgG4 levels and IgG4-producing plasma cell expansion in the affected tissues, which are accompanied by fibrotic or sclerotic changes. Vascular lesions may also be a part of IgG4RD as a number of case reports have discussed inflammatory abdominal aortic aneurysms associated with IgG4RD, but coronary artery lesions seem to be rare complications of IgG4RD. A 71-year-old man suffered from multiple giant coronary aneurysms and an abdominal aortic aneurysm with concurrent pancreatic, gall bladder, bile duct, and salivary gland lesions resulting from IgG4RD...
2012: Internal Medicine
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