Abstract：
:Since the earliest reports in 2001, immunoglobulin G4 (IgG4)-related disease has been defined as an autoimmune systemic disease characterized by the lymphoplasmacytic infiltration of affected tissues leading to fibrosis and obliterative phlebitis along with elevated serum IgG4 levels. Prior to this unifying hypothesis, a plethora of clinical manifestations were considered as separate entities despite the similar laboratory profile. The pathology can be observed in virtually all organs and may thus be a challenging diagnosis, especially when the adequate clinical suspicion is not present or when obtaining a tissue biopsy is not feasible. Nonetheless, the most frequently involved organs are the pancreas and exocrine glands but these may be spared. Immunosuppressants lead to a prompt clinical response in virtually all cases and prevent histological sequelae and, as a consequence, an early differential diagnosis from other conditions, particularly infections and cancer, as well as an early treatment should be pursued. We describe herein two cases in which atypical disease manifestations were observed, i.e., one with recurrent neck lymph node enlargement and proptosis, and one with jaundice. Our understanding of the pathogenesis of IgG4-related disease is largely incomplete but data support a significant role for Th2 cytokines with the contribution of innate immunity factors such as Toll-like receptors, macrophages and basophils. Further, macrophages activated by IL4 overexpress B cell activating factors and contribute to chronic inflammation and the development of fibrosis. We cannot rule out the possibility that the largely variable disease phenotypes reflect different pathogenetic mechanisms and the tissue microenvironment may then contribute to the organ involvement.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Islam AD,Selmi C,Datta-Mitra A,Sonu R,Chen M,Gershwin ME,Raychaudhuri SPdoi
10.1016/j.autrev.2015.06.003subject
Has Abstractpub_date
2015-10-01 00:00:00pages
914-22issue
10eissn
1568-9972issn
1873-0183pii
S1568-9972(15)00134-2journal_volume
14pub_type
杂志文章,评审abstract：:Immune thrombocytopenia (ITP) is a rare autoimmune disease due to an abnormal T cell response, notably supported by splenic T follicular helper cells, that stimulates the proliferation and differentiation of autoreactive B cells. The antiplatelet autoantibodies they produce facilitate platelet phagocytosis by macropha...
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pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2007.10.002
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pub_type： 杂志文章,评审
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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pub_type： 杂志文章,评审
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pub_type： 杂志文章
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pub_type： 杂志文章
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pub_type： 杂志文章,评审
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pub_type： 杂志文章,评审
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pub_type： 杂志文章,评审
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pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2004.04.002
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pub_type： 杂志文章
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journal_title：Autoimmunity reviews
pub_type： 临床试验,杂志文章
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
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journal_title：Autoimmunity reviews
pub_type： 杂志文章,评审
doi：10.1016/j.autrev.2011.11.005
更新日期：2012-07-01 00:00:00
