Treatment approaches to IgG4-related systemic disease

Published on Jan 1, 2011in Current Opinion in Rheumatology4.006
· DOI :10.1097/BOR.0B013E328341A240
Arezou Khosroshahi30
Estimated H-index: 30
(Harvard University),
John H. Stone76
Estimated H-index: 76
Purpose of reviewIgG4-related systemic disease (IgG4-RSD) is a systemic fibroinflammatory condition that can affect any organ system. Prompt recognition and management of this disease process are necessary to prevent sclerosis and permanent organ damage. Here, we review the advances in treatment app
📖 Papers frequently viewed together
2,772 Citations
346 Citations
1,433 Citations
#1Mollie Dahlgren (Harvard University)H-Index: 2
#2Arezou Khosroshahi (Harvard University)H-Index: 30
Last. John H. Stone (Harvard University)H-Index: 76
view all 5 authors...
Objective Riedel's thyroiditis is a chronic fibrosing disorder of unknown etiology often associated with “multifocal fibrosclerosis.” IgG4-related systemic disease is characterized by IgG4+ plasma cell infiltration and fibrosis throughout many organs. We hypothesized that Riedel's thyroiditis is part of the IgG4-related systemic disease spectrum. Methods We searched our institution's pathology database using the terms “Riedel's,” “struma,” “thyroid,” and “fibrosis,” and identified 3 cases of Rie...
244 CitationsSource
#1Hee Man Kim (Kwandong University)H-Index: 21
#2Moon Jae Chung (Yonsei University)H-Index: 18
Last. Jae Bock ChungH-Index: 31
view all 3 authors...
Abstract Autoimmune pancreatitis (AIP) is a type of chronic pancreatitis characterized by swelling of the pancreas, narrowing of the main pancreatic duct, elevation of serum immunoglobulin G or G4 level or presence of several autoantibodies, or lymphoplasmacytic infiltration and fibrosis in the pancreas. However, the pathogenesis of AIP remains unclear, and the natural history and long-term prognosis of AIP are little known. Oral corticosteroid therapy for AIP is recommended. The absolute indica...
26 CitationsSource
#1Arezou Khosroshahi (Harvard University)H-Index: 30
#2Donald B. Bloch (Harvard University)H-Index: 41
Last. John H. Stone (Harvard University)H-Index: 76
view all 4 authors...
Objective. Patients with IgG4-related systemic disease (IgG4-RSD) frequently show an incomplete response to treatment with glucocorticoids and traditional disease-modifying antirheumatic drugs (DMARDs). B lymphocyte depletion is a therapeutic strategy known to be effective for pemphigus vulgaris, an autoimmune condition mediated by IgG4 autoantibodies. This study was performed to assess the clinical and serologic responses to B lymphocyte depletion therapy with rituximab in patients with IgG4-RS...
544 CitationsSource
#1Meaghan L. Khan (Mayo Clinic)H-Index: 5
#2Thomas V. Colby (Mayo Clinic)H-Index: 105
Last. Rafael Fonseca (Mayo Clinic)H-Index: 98
view all 4 authors...
Abstract Hyper IgG4 disease is a recently described inflammatory disease characterized by lymphoplasmacytic infiltration leading to fibrosis and tissue destruction. Whereas most cases have been successfully treated with corticosteroids, recurrent or refractory cases may benefit from alternative therapies. Bortezomib has proven to be successful in the treatment of multiple myeloma, and its mechanism indicates that it may have merit in autoimmune or other plasmacytic disorders. We report a patient...
70 CitationsSource
#1Shigeru B.H. Ko (Nagoya University)H-Index: 29
#2Nobumasa MizunoH-Index: 42
Last. Hidemi Goto (Nagoya University)H-Index: 57
view all 11 authors...
Background & Aims Corticosteroids are now widely accepted as a treatment for autoimmune pancreatitis (AIP). However, the molecular mechanism by which steroid treatment improves AIP remains largely unknown. The aim of this study was to elucidate cellular mechanisms by which corticosteroids improve both pancreatic exocrine function and histopathology in AIP. Methods Pancreatic exocrine function was evaluated by the secretin-stimulated function test and pancreatic biopsy specimens were processed fo...
115 CitationsSource
#1Terumi Kamisawa (Tokyo Metropolitan Komagome Hospital)H-Index: 60
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 68
Last. Masao Tanaka (Kyushu University)H-Index: 78
view all 5 authors...
Steroid therapy appeared to be a standard treatment for autoimmune pancreatitis (AIP), although some AIP patients improve spontaneously. The indications for steroid therapy in AIP patients are symptoms such as obstructive jaundice, abdominal pain, and back pain, and the presence of symptomatic extrapancreatic lesions. Before steroid therapy, jaundice should be managed by biliary drainage in patients with obstructive jaundice, and blood glucose levels should be controlled in patients with diabete...
286 CitationsSource
#1John H. Stone (Harvard University)H-Index: 76
#2Arezou Khosroshahi (Harvard University)H-Index: 30
Last. James R. Stone (Harvard University)H-Index: 52
view all 4 authors...
Objective IgG4-related systemic disease, a disorder recognized only recently, can cause lymphoplasmacytic inflammation in the thoracic aorta. The percentage of cases caused by IgG4-related systemic disease is not known. We aimed to determine the percentage of noninfectious thoracic aortitis cases that are associated with IgG4-related systemic disease and to establish pathologic criteria for identifying involvement of the thoracic aorta by this disorder. Methods We searched our Pathology Service ...
164 CitationsSource
#1Yoh Zen (University of Cambridge)H-Index: 69
#2Manabu OnoderaH-Index: 10
Last. Yasuni NakanumaH-Index: 87
view all 16 authors...
The possible involvement of immunoglobulin G4 (IgG4) in the pathogenesis of idiopathic sclerosing lesions has been suggested. In this study, a clinicopathologic analysis was performed to reveal characteristics of retroperitoneal fibrosis relating to IgG4. The study involved 17 patients with retroperitoneal fibrosis. Immunohistochemistry revealed numerous IgG4-positive plasma cell infiltrates in 10 cases (IgG4-related), but only a few positive cells in 7 cases (non-IgG4-related). All patients wit...
314 CitationsSource
#1Terumi Kamisawa (Tokyo Metropolitan Komagome Hospital)H-Index: 60
#2Tooru Shimosegawa (Tohoku University)H-Index: 69
Last. Masao Omata (UTokyo: University of Tokyo)H-Index: 105
view all 19 authors...
Objective: To establish an appropriate steroid treatment regimen for autoimmune pancreatitis (AIP). Methods: A retrospective survey of AIP treatment was conducted in 17 centres in Japan. The main outcome measures were rate of remission and relapse. Results: Of 563 patients with AIP, 459 (82%) received steroid treatment. The remission rate of steroid-treated AIP was 98%, which was significantly higher than that of patients without steroid treatment (74%, 77/104; p Conclusions: The major indicatio...
698 CitationsSource
#1Rahul Pannala (Mayo Clinic)H-Index: 21
#2Suresh T. ChariH-Index: 83
Autoimmune pancreatitis (AIP) is the only pancreatic disorder responsive to steroid treatment. AIP has recently been subclassified into type 1 and type 2 AIP.1 Type 1 AIP is the pancreatic manifestation of immunoglobulin G4 (IgG4)-associated systemic disease (ISD), a fibroinflammatory autoimmune disorder that involves multiple organ systems and is characteristically associated with elevation of serum levels of IgG4.2 Commonly used diagnostic criteria for AIP include the Asian Consensus diagnosti...
69 CitationsSource
Cited By183
Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Respo...
#1Samuel J. Cler (WashU: Washington University in St. Louis)
#2Nima Sharifai (WashU: Washington University in St. Louis)H-Index: 3
Last. Michael R. Chicoine (WashU: Washington University in St. Louis)H-Index: 35
view all 9 authors...
Abstract Objective IgG4-Related Disease (IgG4-RD) is an inflammatory process that uncommonly can present in the skull base and calvarium and mimic a tumor but the nature of this condition is not well summarized in the neurosurgical literature. Methods A review of 2 cases of IgG4-RD in the skull base highlighting the diagnostic challenges with assessment of these skull base lesions was performed, and a systematic review of relevant literature summarized. Results A systematic review of literature ...
#1Snigdha Nutalapati (UK: University of Kentucky)
#2Richard O'Neal (UK: University of Kentucky)H-Index: 2
Last. Gerhard C. Hildebrandt (UK: University of Kentucky)H-Index: 11
view all 5 authors...
Eosinophilic angiocentric fibrosis (EAF) is an exceeding rare clinical entity and is considered a part of the spectrum of IgG4-related disease (IgG4RD). We hereby present such an unusual case of a 60-year-old female who presented to us with recurrent sinonasal mass, after a decade long haul of multiple clinical evaluations, biopsies, and debulking surgery without a definitive diagnosis. Over this period, the mass eroded through the ethmoid cells along with nasal septal destruction leading to sad...
#1Fardad Behzadi (Brigham and Women's Hospital)
#2Chong Hyun Suh (UOU: University of Ulsan)H-Index: 30
Last. Jeffrey P. Guenette (Brigham and Women's Hospital)H-Index: 9
view all 6 authors...
Abstract This systematic review aims to clarify and comprehensively detail the sometimes variable published imaging features as well as the pathogenesis, clinical diagnostic criteria, and treatment options of IgG4-Related Diseases (IgG4-RD) in the head and neck to aid the radiologist in diagnosing relapse and new sites of disease. A literature search in PubMed and EMBASE for reported cases of IgG4-RD was performed in December 2019. Case reports or series of IgG4-RD in the head and neck in adults...
#1Su Jin Kim (PNU: Pusan National University)H-Index: 2
#2Seung Uk Lee (Kosin University)H-Index: 5
Last. Ji Eun Lee (PNU: Pusan National University)H-Index: 4
view all 7 authors...
BACKGROUND We report a case of atypical presentation of IgG4-related disease (IgG4-RD) with recurrent scleritis and optic nerve involvement. CASE PRESENTATION A 61-year-old male presented with ocular pain and injection in his left eye for 2 months. Ocular examination together with ancillary testing led to the diagnosis of scleritis, which relapsed in spite of several courses of steroid treatment. After cessation of steroid, the patient complained of severe retro-orbital pain and blurred vision. ...
#1Giacomo Quattrocchio (UNITO: University of Turin)H-Index: 14
#2Antonella Barreca (UNITO: University of Turin)H-Index: 12
Last. Dario Roccatello (UNITO: University of Turin)H-Index: 39
view all 10 authors...
IgG4-related disease (IgG4-RD) is an immune-mediated disorder often showing elevated serum IgG4 concentrations, dense T and B lymphocyte infiltration, and IgG4-positive plasma cells and storiform fibrosis. We prospectively evaluated for 4 years 5 patients with histologically proven IgG4-RD of whom 3 had tubulointerstitial nephritis (TIN) and 2 had retroperitoneal fibrosis (RPF). They received an intensive B depletion therapy with rituximab. The estimated glomerular filtration rate of TIN patient...
2 CitationsSource