IgG4-related disease.

Published on Apr 26, 2012in The New England Journal of Medicine74.699
· DOI :10.1056/NEJMC1202768
John H. Stone76
Estimated H-index: 76
(Harvard University),
Markus Hufnagel17
Estimated H-index: 17
+ 0 AuthorsAnnette Schmitt-Graeff38
Estimated H-index: 38
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Objective T-helper type 2 responses are crucial in Churg–Strauss syndrome (CSS) and may enhance the production of IgG4 antibodies. The authors assessed the IgG4 immune response in CSS patients. Methods The authors included 46 consecutive patients with CSS (24 with active and 22 with quiescent disease), 26 with granulomatosis with polyangiitis (GPA, Wegener9s), 25 with atopic asthma and 20 healthy controls and determined serum IgG, IgM, IgA, IgE and IgG subclass levels. Tissue infiltration by IgG...
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Background IgG4-related systemic fibrosclerosis is a recently defined disorder characterised by a diffuse or tumefactive inflammatory reaction rich in IgG4-positive plasma cells associated with sclerosis and obliterative phlebitis. Although characteristic histopathological features are essential for the diagnosis of these disorders, to date there exists no consensus regarding the cut-off values used to define a ‘significant IgG4-positive plasma cell count,’ and data regarding the distribution of...
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Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. ...
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Abstract IgG4-related disease is a distinct clinical entity, whose characteristic features are the following; Serum IgG4 is prominently elevated, IgG4-positive plasma cells infiltrate in involved tissues, various mass-forming lesions with fibrosis develop in a timely and spatial manner and the response to corticosteroids is prompt and good. IgG4-related diseases mainly target two organs. One is the pancreas (autoimmune pancreatitis; AIP), and the other comprises the lacrimal and salivary glands,...
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Background: Mikulicz’s disease (MD) has been considered as one manifestation of Sjogren’s syndrome (SS). Recently, it has also been considered as an IgG 4 -related disorder. Objective: To determine the differences between IgG 4 -related disorders including MD and SS. Methods: A study was undertaken to investigate patients with MD and IgG 4 -related disorders registered in Japan and to set up provisional criteria for the new clinical entity IgG 4 -positive multiorgan lymphoproliferative syndrome ...
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This article will review the structure and function of IgG4, methods of measuring serum IgG4 concentrations, clinical conditions associated with increased and decreased serum IgG4, and the test characteristics of serum IgG4 in the diagnosis and management of Immunoglobulin G4-Related Disease (IgG4-RD). The four subclasses of IgG were discovered in 1964 through experiments on monoclonal IgG in patients with myeloma. Since 2001, interest in measuring serum IgG subclasses has increased dramatically...
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PURPOSE Specific IgG4 (sIgG4) increases with allergen specific immunotherapy and may reflect a state of immune tolerance in food allergy. While ImmunoCAP® has been widely used to measure sIgG4 to a single allergen, PROTIA™ Specific IgG4® has been designed as a multiplex assay for measuring sIgG4. This study sought to validate this assay in comparison to ImmunoCAP®. MATERIALS AND METHODS Measurements of sIgG4 were compared between PROTIA™ Specific IgG4® and ImmunoCAP® using sera from 519 allergy ...
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OBJECTIVES: Serum IgG4 may often increases in allergic diseases. Eosinophilic granulomatosis with polyangiitis (EGPA) has an allergic phase. For this reason, it was questionable whether IgG4-RD and EGPA may share some clinical and laboratory features. In this study, we investigated whether definite IgG4-RD might share those features with EGPA. METHODS: We retrospectively reviewed the medical records of 42 Korean immunosuppressive drug-naive patients with definite IgG4-RD. The American College of...
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#2Gabrielle E Thottam (UMN: University of Minnesota)
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IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing disease which causes tumor-like swelling of organs and commonly mimics symptoms of malignancy. It has been increasing in prevalence in the last decade, but esophageal involvement remains rare. IgG4-RD was first known to involve certain organs, such as the pancreas. It has, since, been described as a systemic disease process. IgG4-RD should be considered in patients presenting with dysphagia. Initiation of appropriate treatment with ...