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IgG4- related diseases

Alexandros Stamatopoulos, Davide Patrini, Efstratios Koletsis, Elaine Borg, Reena Khiroja, Martin Hayward, David Lawrence, Nikolaos Panagiotopoulos
IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect practically every organ. Although it was first identified in pancreas and salivary glands, major organs like liver, biliary tree, kidney, thyroid glands and lungs are commonly involved, sometimes resulting in organ failure. We describe a case of an 41-year-old man presented with back pain after a rotator cuff injury. A Computed Tomography (CT) revealed incidentally a right lower lobe paravertebral lesion extending across the T5 and T6 vertebral levels and invading into the adjacent pleural surface...
2016: Respiratory Medicine Case Reports
Zhanli Fu, Meng Liu, Jin Zhang, Ziao Li, Qian Li
Multiple renal lesions, which showed clearly demarcated low densities on contrast-enhanced CT, were revealed in a 56-year-old woman. The F-FDG avidity of the renal lesions was equal to or mildly higher than that of the renal cortex on the routine PET/CT scan, but significantly higher on the delayed scan. IgG4-related disease with renal involvement was confirmed subsequently by the measurement of serous IgG4 and the pathology of renal lesion.
October 18, 2016: Clinical Nuclear Medicine
Nicole C Droz, Stephanie D Mathew
After presenting with nonspecific symptoms, a 52-year-old female was found to have a 3.4 × 2.6 cm retroperitoneal mass resulting in hydronephrosis on abdominal computed tomography scan. After extensive workup including surgical debulking, excisional biopsy, and autoimmune panel, she was found to have idiopathic retroperitoneal fibrosis (IRF) with negative staining for immunoglogulin G4 (IgG4). Two years after presentation, the patient developed dyspnea, cough, and xerostomia. Repeat anti-Sjögren Syndrome A and anti-Sjögren Syndrome B titers and labial gland biopsy confirmed the diagnosis of concomitant Sjögren's syndrome (SS)...
October 2016: Military Medicine
Masafumi Moriyama, Seiji Nakamura
IgG4-related disease (IgG4-RD) is a systemic disease characterized by elevated serum IgG4 levels and a strong infiltration of IgG4-positive plasma cells in various organs. IgG4-RD patients also frequently suffer from allergic diseases, including asthma and atopic dermatitis. It is well known that T helper type 2 (Th2) cells have an important role in the initiation of allergic diseases, and Th2 cytokines such as interleukin (IL)-4 and IL-13 promote class switching to IgG4. Therefore, IgG4-RD is considered to be a Th2-predominant disease...
October 16, 2016: Current Topics in Microbiology and Immunology
Tomohiro Watanabe, Kouhei Yamashita, Masatoshi Kudo
An increased number of clinicopathological studies on autoimmune pancreatitis, cholangitis, and sialoadenitis have led to the recognition of immunoglobulin G4-related disease (IgG4-RD) as a novel disorder, characterized by elevated levels of serum IgG4 and infiltration of IgG4-expressing plasma cells in the affected organs. Although the immunological background associated with the development of IgG4-RD remains poorly understood, recent studies have suggested involvement of the innate immune response in its pathogenesis...
October 16, 2016: Current Topics in Microbiology and Immunology
John Wei-Liang Tan, Carolina Valerio, Elizabeth H Barnes, Paul J Turner, Peter A Van Asperen, Alyson M Kakakios, Dianne E Campbell
BACKGROUND: Epidemiological evidence suggests delayed introduction of egg may not protect against egg allergy in infants at risk of allergic disease. OBJECTIVE: To assess whether dietary introduction of egg between 4-6 months in infants at risk of allergy would reduce sensitization to egg. METHODS: We conducted a randomized controlled trial in infants with at least one first-degree relative with allergic disease. Infants with a skin prick test to egg white (EW-SPT) <2mm were randomized at age 4 months to receive whole-egg powder or placebo (rice powder) until 8-months of age, with all other dietary egg excluded...
October 11, 2016: Journal of Allergy and Clinical Immunology
Manish Soneja, S Achintya, Neeraj Nischal, Piyush Ranjan, Surabhi Vyas, Sudheer Arava, S K Sharma
A large proportion of patients with retroperitoneal fibrosis (RPF) have been labelled as having idiopathic RPF historically. Recent reports of a subset of these patients having a fibro-inflammatory condition with characteristic histology and good response to therapy has helped in renewed understanding of an old disease. A 40 year old man presented with vague abdominal discomfort of 5 months duration. Ultrasound examination revealed soft tissue thickening with calcification over lower abdominal aorta. CT scan characterized the lesion as a retroperitoneal mass encasing the aorta with an associated lesion in upper pole of left kidney...
June 2016: Journal of the Association of Physicians of India
Mercedes Acevedo Ribó, Francisco Javier Ahijado Hormigos, Francisco Díaz, Marta Romero Molina, Maria Angeles Fernandez Rojo, Maria Antonia Garcia Rubiales, Eugenio García Diaz
This case report describes a patient with a previous history of autoimmune pancreatitis secondary to IgG4-related disease, who developed an overt nephrotic syndrome due to membranous nephropathy, surprisingly idiopathic. In all the previously described cases with both concurrent diseases, membranous nephropathy was considered to be secondary to the IgG4-related disease based on the absence of anti-PLA2R1 autoantibodies, and nephrotic syndrome usually remitted after treatment with steroids alone. However, in our patient positivity of serum anti-PLA2R1 autoantibodies together with a normal serum IgG4 level, and the absence of the other most commonly associated diseases were compatible with an idiopathic membranous nephropathy...
October 14, 2016: Clinical Nephrology
Rafael Coimbra Ferreira Beltrame, Maurício Friderichs, Bárbara Rayanne Fior, Pedro Guilherme Schaefer, Gustavo Gomes Thomé, Dirceu Reis da Silva, Elvino José Guardão Barros, Renato Seligman, Francisco Veríssimo Veronese
The IgG4-related disease has a wide clinical spectrum where multiple organs can be affected, and the diagnosis depends on typical histopathological findings and an elevated IgG4 expression in plasma cells in the affected tissue. We describe the clinical presentation and evolution of a patient with acute tubulointerstitial nephritis, severe kidney failure and systemic manifestations such as lymphadenomegaly and chronic pancreatitis. The diagnosis was confirmed by the clinical picture and kidney and lymph node histopathology, in which immunohistochemistry of the lymphoid tissue showed policlonality and increased expression of IgG4, with a IgG4/total IgG ratio > 80%...
July 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Lilun Li, Bryan Ward, Margaret Cocks, Amir Kheradmand, Howard W Francis
OBJECTIVE: IgG4-related disease (IgG4-RD) is an idiopathic inflammatory condition that causes pseudotumor formation in single or multiple organs, including those of the head and neck. Temporal bone involvement is rare, with only 3 cases of unilateral temporal bone IgG4-RD described in the literature. We report the first known case of IgG4-RD of bilateral temporal bones and describe its clinical presentation, diagnosis, and treatment. METHODS: The patient was a 52-year-old man with latent tuberculosis (TB) who presented with a 10-year history of bilateral profound hearing loss and vestibular dysfunction...
October 10, 2016: Annals of Otology, Rhinology, and Laryngology
Shigeyuki Kawa
High serum immunoglobulin (Ig) G4 concentration and abundant IgG4-bearing plasma cell infiltration are characteristic features in autoimmune pancreatitis (AIP). AIP is also complicated with a variety of other organ involvements that commonly share marked IgG4-bearing plasma cell infiltration, suggesting the existence of a systemic disease associated with IgG4 currently recognized as IgG4-related disease (IgG4-RD). However, it is controversial whether IgG4 plays a role in the pathogenesis of AIP or IgG4-RD through such characteristic attributes as Fab-arm exchange and rheumatoid factor (RF)-like activity...
September 21, 2016: Current Topics in Microbiology and Immunology
J Rosendahl
No abstract text is available yet for this article.
October 2016: Zeitschrift Für Gastroenterologie
Robert W Hoffman, Joan T Merrill, Marta M E Alarcón-Riquelme, Michelle Petri, Ernst R Dow, Eric Nantz, Laura K Nisenbaum, Krista M Schroeder, Wendy J Komocsar, Narayanan B Perumal, Matthew D Linnik, David C Airey, Yushi Liu, Guilherme V Rocha, Richard E Higgs
OBJECTIVE: Systemic lupus erythematosus (SLE) has substantial unmet medical need and its pathogenesis is incompletely understood. This study characterized baseline gene expression and pharmacodynamic (PD)-induced changes in whole blood gene expression from two phase III, 52-week (W), randomized, placebo-controlled, double-blind studies of 1,760 SLE patients treated with the B cell activating factor (BAFF)-blocking IgG4 monoclonal antibody, tabalumab. METHODS: Patient samples were obtained from ILLUMINATE-1 and -2 while control samples were from healthy donors...
October 9, 2016: Arthritis & Rheumatology
Melanie C Bois, Hiroshi Sekiguchi, Jay H Ryu, Eunhee S Yi
Pulmonary nodular lymphoid hyperplasia (PNLH) may show heavy lymphoplasmacytic infiltrates with increased Immunoglobulin G4 (IgG4)-positive plasma cells as in IgG4-related disease (IgG4-RD). However, it is unclear whether PNLH could be a manifestation of IgG4-RD. A recent study reported the presence of Epstein-Barr virus (EBV) in IgG4-related lymphadenopathy. We postulated that a subset of PNLH might represent IgG4-related lung disease (IgG4-RLD) with EBV-positive lymphocytes as has been reported in IgG4-related lymphadenopathy...
October 5, 2016: Human Pathology
Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders, reported both in Crohn's disease (CD) and ulcerative colitis (UC). They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions. Fatty liver is considered as the most common hepatobiliary complication in IBD whilst primary sclerosing cholangitis (PSC) the most specific one. Less frequently, IBD-associated hepatobiliary disorders include: autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis (PBC), hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, and liver abscess...
October 6, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Dipankar Das, Panna Deka, Geeta Verma, Ganesh Chandra Kuri, Harsha Bhattacharjee, Gayatri Bharali, Divya Pandey, Akanksha Koul, Bidisha Das, Apurba Deka
Immunoglobulin G4 (IgG4-related diseases) affects various tissues and organs of the human body. Orbital, adnexal, and scleral inflammations were already reported in the medical literature. To the best of our knowledge, we report the first case of intraocular IgG4-associated inflammatory mass in the ciliary body mimicking as a melanoma in a 23-year-old female from Northeast India. Characteristic histopathology, immunohistochemistry in the tissue, protein chemistry, and raised serum IgG4 were supportive for the diagnosis...
August 2016: Indian Journal of Ophthalmology
Kenji Nishida, Yuka Sogabe, Ayako Makihara, Akemi Senoo, Hisanori Morimoto, Mai Takeuchi, Yuka Gion, Tadashi Yoshino, Yasuharu Sato
A 41-year-old man was diagnosed with immunoglobulin G4-related disease (IgG4-RD) in both eyelids 4 years ago and exhibited good response to steroid therapy. However, rapid swelling of the right eyelid lesion was recently observed. As IgG4-RD progression was suspected, biopsy was performed. Although the histology was consistent with IgG4-RD, the infiltrating large atypical lymphoid cells showed immunoglobulin light-chain restriction and IgH gene rearrangement. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells...
August 11, 2016: Modern Rheumatology
Miroslav Průcha, Petr Czinner, Petra Prokopová
Currently, Ormond's disease is classified among IgG4-associated diseases. Its clinical manifestation varies and is characterized by the presence of fibrous retroperitoneal tissue that often affects the ureters or abdominal aorta and iliac arteries. We present a unique case of the polycystic form of Ormond's disease, imitating tumour in the retroperitoneal space. At the time of diagnosis, the disease was not metabolically active and did not require immunosuppressive therapy. The polycystic mass was removed surgically...
2016: Prague Medical Report
Emanuel Della-Torre, John H Stone
IgG4-related disease (IgG4-RD) is an immune-mediated disease driven by interactions on several levels between cells of the B and T lineages. A key driver of IgG4-RD is believed to be a novel CD4+ cytotoxic T lymphocyte that bears SLAM-F7 on its surface. This cell is presumed to be sustained by continuous antigen presentation by cells of the B cell lineage, particularly plasmablasts. Because IgG4-RD has been recognized for fewer than 15 years as a unified diagnostic entity, no controlled clinical trials or few prospective studies of any sort have been performed...
September 26, 2016: Journal of Clinical Immunology
W J Shi, Y N Zhang, P Lin, X Peng, S C Zhang
No abstract text is available yet for this article.
September 7, 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
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