Immunoglobulin G4-Related Disease
01 Aug 2015-Journal of Reproduction and Development (Korean College of Rheumatology)-Vol. 22, Iss: 4, pp 213-222
TL;DR: Rituximab is reported to be an effective therapy for treatment of IgG4-RD, even without concomitant glucocorticoid therapy, which is the first-line therapy for patients with multiple organ dysfunction and clinical symptoms.
Abstract: Immunoglobulin G4-related disease (IgG4-RD) is an emerging immune-mediated fibro-inflammatory disorder which can involve any organ. The main characteristics of IgG4-RD are increased serum IgG4 concentration, abundant IgG4+ plasma cells in affected tissues, and painless swollen organs often without general symptoms. Typical pathology features of IgG4-RD are lymphoplasmacytic infiltration, dense storiform fibrosis, and obliterative pheblitis. The pathogenesis of IgG4-RD remains elusive, but involvement of excess production of type 2 T helper cells, regulatory T-cell cytokines, and B-cell activating factor in the development of IgG4-RD has been suggested. Diagnosis of IgG4-RD can be made on the basis of serological, imaging, particularly histopathological findings. Glucocorticoid is the first-line therapy for patients with multiple organ dysfunction and clinical symptoms. Drugs such as azathioprine, mycophenolate mofetil, methotrexate, and cyclophosphamide can be used as steroid-sparing agents. Rituximab is reported to be an effective therapy for treatment of IgG4-RD, even without concomitant glucocorticoid therapy. This review summarizes current concepts on pathophysiology, clinical manifestations, and treatment of IgG4-RD. (J Rheum Dis 2015;22:213-222)
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TL;DR: Using alternative preoperative diagnostic methods such as endoscopic ultrasound-guided fine-needle biopsy or the biopsy unroofing technique could spare the patient from unnecessary surgical treatment.
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18 Nov 2016References
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TL;DR: The results of these studies suggest that abnormal innate immune responses against microbial antigens may underlie the immunopathogenesis of IgG4-related disease.
Abstract: Objective
IgG4-related disease is a recently recognized entity affecting multiple organs, including the pancreas, biliary tracts, and salivary glands. Although IgG4-related disease is characterized by systemic IgG4 antibody responses and by infiltration of IgG4-expressing plasma cells, the innate immune responses leading to adaptive IgG4 antibody responses are poorly understood. The aim of this study was to clarify the innate immune responses leading to IgG4 antibody production.
Methods
IgG4 and cytokine responses to various nucleotide-binding oligomerization domain (NOD)–like receptor (NLR) and Toll-like receptor (TLR) ligands were examined using peripheral blood mononuclear cells (PBMCs) from healthy control subjects and patients with IgG4-related autoimmune pancreatitis.
Results
Activation of NOD-2 in monocytes from healthy control subjects induced IgG4 production by B cells in a BAFF-dependent and T cell–independent manner. In addition, PBMCs from patients with IgG4-related disease produced a large amount of IgG4 upon stimulation with NLR and TLR ligands; this enhanced IgG4 production was associated with the induction of BAFF by NLR and TLR ligands. Monocytes from patients with IgG4-related disease induced IgG4 production by B cells from healthy control subjects upon stimulation with NLR and TLR ligands.
Conclusion
The results of these studies suggest that abnormal innate immune responses against microbial antigens may underlie the immunopathogenesis of IgG4-related disease.
117 citations
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TL;DR: The current thought and understanding of this disease with regard to nomenclature, organ system involvement, and approaches to therapy are reviewed.
115 citations
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...6 mg/kg/d)가 사용되며 신체 검사, 혈액 검사, 방사선 검사 소견 등을 종합하여 서서히 감량한다[77]....
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TL;DR: The results of recent studies are discussed and an overview of MD as an IgG4-related disease is provided, thought to be singular systemic IgG 4-related plasmacytic diseases.
112 citations
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...대칭적인 눈물샘염과 귀밑 침샘 또 는 턱밑 침샘 종대를 특징으로 하는 Mikulicz 병은 쇼그렌 증후군의 아형으로 알려져 왔으나 최근에 IgG4 연관 질환 임이 밝혀졌다[39]....
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TL;DR: Results indicate that IL-10 and CCL18 secreted by preferential M2 macrophages possibly play a key role in the development of severe fibrosis in IgG4-DS.
110 citations
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...Furukawa 등[16]은 MIkulicz 병 환자의 턱밑 타액선의 M2 대식세포수가 일차성 쇼그...
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TL;DR: The data suggest that the prevalence of IgG4-related hypophysitis has been underestimated and the possibility of the development of hypopituitarism and/or diabetes insipidus caused by IgG3-relatedhypophysitis during the clinical course of other IgG 4-RDs is considered.
Abstract: Objective: The prevalence and clinical characteristics of IgG4-related hypophysitis remain unclear due to the limited number of case reports. Therefore, in this study, we screened consecutive outpatients with hypopituitarism and/or diabetes insipidus (DI) to estimate its prevalence. Methods: A total of 170 consecutive outpatients with hypopituitarism and/or central DI were screened at Kobe University Hospital for detecting IgG4-related hypophysitis by pituitary magnetic resonance imaging, measuring serum IgG4 concentrations, assessing the involvement of other organs, and carrying out an immunohistochemical analysis to detect IgG4-positive cell infiltration. Results: Among the screened cases, 116 cases were excluded due to diagnosis of other causes such as tumors and congenital abnormalities. Additionally, 22 cases with isolated ACTH deficiency were analyzed and were found not to meet the criteria of IgG4-related hypophysitis. The remaining 32 cases were screened and seven were diagnosed with IgG4-related hypophysitis, of which three cases were diagnosed by analyzing pituitary specimens. IgG4-related hypophysitis was detected in 30% (seven of 23 patients) of hypophysitis cases and 4% of all hypopituitarism/DI cases. The mean age at the onset of IgG4-related hypophysitis was 61.8G8.8 years, and the serum IgG4 concentration was 191.1G78.3 mg/dl (normal values 5‐105 mg/dl and values in IgG4-related disease (RD) R135 mg/dl). Pituitary gland and/or stalk swelling was observed in six patients, and an empty sella was observed in one patient. Multiple co-existing organ involvement was observed in four of the seven patients prior to the onset of IgG4-related hypophysitis. Conclusion: These data suggest that the prevalence of IgG4-related hypophysitis has been underestimated. We should also consider the possibility of the development of hypopituitarism/DI caused by IgG4-related hypophysitis during the clinical course of other IgG4-RDs.
109 citations
Additional excerpts
...드물게 뇌하수체에 IgG4 연관 질환이 발생하여 뇌하 수체 저하증 또는 요붕증이 발병할 수 있다[70]....
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