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IgG4- related diseases ( IgG4-RD )

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
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
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
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
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
Rajwant Kaur, Suvradeep Mitra, Arvind Rajwanshi, Ashim Das, Uma Nahar Saikia, Pranab Dey
BACKGROUND: IgG4-related disease (IgG4-RD) is a tumefactive fibro-inflammatory lesion that can affect any organ system in the body. Till date, no cytological data on IgG4-RD are available and this is the first study depicting the cytopathology features of IgG4-RD. AIM AND OBJECTIVE: To describe the cytopathological features and potential diagnostic errors of IgG4-RD. MATERIALS AND METHODS: The cytological features of 10 histopathology proven IgG4-RD cases (11 samples) were retrospectively reviewed along with corresponding histopathology...
September 26, 2016: Diagnostic Cytopathology
Marius N Stan, Vikram Sonawane, Thomas J Sebo, Prabin Thapa, Rebecca S Bahn
CONTEXT: IgG4-positive (+) plasma cells have been reported in both Riedel's thyroiditis (RT) and Hashimoto's thyroiditis (HT). These cells are the hallmark of IgG4-related disease (IgG4-RD). OBJECTIVE: We sought to determine whether RT is part of IgG4-RD spectrum. DESIGN, SETTING AND PATIENTS: This was a case-control study performed at a tertiary medical centre. We included RT cases from the period 1958 to 2008 that had sufficient paraffin-embedded tissue for IgG4 immunostaining...
September 20, 2016: Clinical Endocrinology
Marco Lanzillotta, Corrado Campochiaro, Matteo Trimarchi, Gianluigi Arrigoni, Simonetta Gerevini, Raffaella Milani, Enrica Bozzolo, Matteo Biafora, Elena Venturini, Maria Pia Cicalese, John H Stone, Maria Grazia Sabbadini, Emanuel Della-Torre
OBJECTIVE: A series of destructive and tumefactive lesions of the midline structures have been recently added to the spectrum of IgG4-related disease (IgG4-RD). We examined the clinical, serological, endoscopic, radiological, and histological features that might be of utility in distinguishing IgG4-RD from other forms of inflammatory conditions with the potential to involve the sinonasal area and the oral cavity. METHODS: We studied 11 consecutive patients with erosive and/or tumefactive lesions of the midline structures referred to our tertiary care center...
September 13, 2016: Modern Rheumatology
Cameron C Wick, Joseph Zachariah, Sunil Manjila, William C Brown, Prerna Malla, Bashar Katirji, Mark Cohen, Cliff A Megerian
IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her resected ear mass suggested a benign inflammatory process but no specific diagnosis...
August 18, 2016: American Journal of Otolaryngology
Wei-Yu Chiang, Ting-Ting Liu, Wan-Ting Huang, Ming-Tse Kuo
Herein, we elucidate that ligneous conjunctivitis (LC) was proved as an IgG4-related disease (IgG4-RD) by a series of pathologic studies from primary and recurrent episodes of an LC patient. LC was diagnosed based on clinical presentation and pathological appearance; furthermore, combined with serological examination and immunohistochemical study, the case also conformed to the diagnosis of IgG4-RD. The IgG4-RD, broadly discussed in recent times, is an idiopathic disease entity with tissue fibrosis possibly involving multiple organs...
July 2016: Indian Journal of Ophthalmology
I A Kazantseva, S V Lishchuk, Yu P Gribunov, I N Shestakova, K A Pavlov
The paper presents the data available in the literature on IgG4-related disease (IgG4-RD) concurrent with malignancies at different sites, as well as possible common pathogenetic mechanisms of their development and morphological diagnostic criteria for IgG4-RD. The authors give their own observation of gastric signet ring cell carcinoma concurrent with morphologically verified IgG4-RD.
July 2016: Arkhiv Patologii
John H Stone
IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect essentially any organ. The disease shows similar histopathology findings across organ systems, consisting of a lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. IgG4 itself appears to be a reactive phenomenon rather than the primary disease driver. Recent investigations have focused on the interactions between cells of the B cell lineage and a novel CD4+ SLAMF7+ cytotoxic T cells capable of promoting fibrosis...
July 2016: Clinical and Experimental Rheumatology
Yosuke Kunishita, Ryusuke Yoshimi, Mitsuhiro Takeno, Shoji Yamanaka, Yumiko Sugiyama, Naomi Tsuchida, Daiga Kishimoto, Reikou Kamiyama, Kaoru Minegishi, Maasa Hama, Yohei Kirino, Yoshiaki Ishigatsubo, Atsuhisa Ueda, Hideaki Nakajima
Multifocal fibrosclerosis (MFS), which causes systemic and chronic connective tissue inflammation, has been associated with IgG4 and regarded as an identical entity with "IgG4-related disease (IgG4-RD)". Although a few cases of MFS mimicking IgG4-RD histopathologically, despite the absence of a serum IgG4 elevation and IgG4-positive plasma cell infiltration, have been reported, there is, so far, little information regarding such exceptional cases. We herein demonstrate a case of non-IgG4-related MFS presenting with periaortitis and parotiditis, whose histological findings were consistent with IgG4-RD despite the absence of elevated serum and tissue IgG4 levels...
2016: Internal Medicine
Jonathan Dillon, Andrea Dart, Tom Sutherland
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that was first recognised as a systemic disease in 2003, when patients with autoimmune pancreatitis were found to have extrapancreatic manifestations. Since 2003, IgG4-RD has been described in a diverse range of other organs including the biliary tree, orbits, lacrimal glands, salivary glands, lungs, kidneys, aorta, retroperitoneum, lymph nodes, pachymeninges, prostate and pituitary gland. The disease frequently occurs in the absence of pancreatic involvement...
August 26, 2016: Journal of Medical Imaging and Radiation Oncology
Emanuel Della-Torre, Marco Lanzillotta, Corrado Campochiaro, Emanuele Bozzalla, Enrica Bozzolo, Alessandro Bandiera, Elena Bazzigaluppi, Carla Canevari, Giulio Modorati, John H Stone, Angelo Manfredi, Claudio Doglioni
BACKGROUND: IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by serum IgG4 elevation and tissue infiltration of IgG4-positive plasma cells. Substantial overlap between IgG4-RD and antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) exists in terms of organ involvement and histopathological features. A positive ANCA assay is regarded as a highly specific finding in favor of an AAV, and generally influences away from a diagnosis of IgG4-RD...
August 2016: Medicine (Baltimore)
Wen-Long Xu, Ying-Chun Ling, Zhi-Kai Wang, Fang Deng
An elevated serum IgG4 level is one of the most useful factors in the diagnosis of IgG4-related disease (IgG4-RD). In this study, we performed a meta-analysis of the published articles assessing the diagnostic accuracy of serum IgG4 concentrations for IgG4-RD. The databases of MEDLINE/PubMed, EMBASE and Web of Science were systematically searched for relevant studies. Sensitivities and specificities of serum IgG4 in each study were calculated, and the hierarchical summary receiver operating characteristic (HSROC) model with a random effects model were employed to obtain the individual and pooled estimates of sensitivities and specificities...
2016: Scientific Reports
Akifumi Ichiki, Norisato Hashimoto, Tomoki Ueda, Shinichiro Hiraiwa, Takuma Tajiri, Naoya Nakamura, Kiyoshi Ando, Kenji Yokoyama
A 61-year-old Japanese woman with anemia, submandibular gland swelling, and enlarged lymph nodes was diagnosed with IgG4-related disease (IgG4-RD) by lymph node biopsy. Bone marrow involvement of IgG4-RD was detected by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) and immunohistochemically proven by bone marrow biopsy in this patient. Anemia progressed gradually, and prednisolone treatment was initiated. Anemia and submandibular gland swelling improved soon after prednisolone treatment was initiated...
2016: Internal Medicine
Michelle M Williams, Hazem Mashaly, Vinay K Puduvalli, Ming Jin, Ehud Mendel
The authors report a case of immunoglobulin G4-related disease (IgG4-RD) presenting as a paraspinal, epidural mass. This disease encompasses a host of autoimmune conditions that were previously thought to be separate entities. It is characterized by fibrosis, mediated by the aberrant proliferation and tissue invasion of IgG4-positive plasma cells, which can occur in any organ. As with other autoimmune conditions, it tends to be responsive to steroids and other immunosuppressants. It can rarely present as a tumefactive lesion of the central nervous system, creating the potential for misdiagnosis (given its similar radiological appearance to malignancy) and mistreatment...
August 12, 2016: Journal of Neurosurgery. Spine
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