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)
Citations
More filters
••
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.
2 citations
••
18 Nov 2016References
More filters
••
TL;DR: The distinctive histologic features of IgG4-related tubuloInterstitial nephritis can facilitate the differential diagnosis of tubulointerstitial nephitis, even without autoimmune pancreatitis or an abnormal computed tomography suggesting a renal tumor.
104 citations
••
TL;DR: IgG4-SD represents one, albeit rare, etiology of TAA, especially those originating in the aortic arch, and uniformly presents in the form of an aneurysm with distinct histologic and clinicopathologic features.
104 citations
Additional excerpts
...IgG4 연관 질환에서 흉대동맥이 침범되면 대동맥 확장, 동맥류, 대동맥 박리 등의 형태로 나타난다[60]....
[...]
••
TL;DR: It is demonstrated that eosinophils function as antigen-presenting cells for the induction of the primary and the expansion of the secondary Th2 immune responses to S. stercoralis in mice.
Abstract: The objective of the present study was to explore the ability of eosinophils to present Strongyloides stercoralis antigen in naive and immunized mice. Antigen-pulsed eosinophils were injected intraperitoneally into naive or immunized mice, and then mice were examined for antigen-specific immune responses. A single inoculation of antigen-pulsed eosinophils was sufficient to prime naive mice and to boost immunized mice for antigen-specific T helper cell type 2 (Th2) immune responses with increased interleukin (IL)-4 and IL-5 production. Mice inoculated 3 times with live eosinophils pulsed with antigen showed significant increases in parasite antigen-specific immunoglobulin (Ig) M and IgG levels in their serum. Antigen-pulsed eosinophils deficient in major histocompatibility complex class II molecules or antigen-pulsed dead eosinophils failed to induce immune responses, thereby demonstrating the requirement for direct interaction between eosinophils and T cells. These experiments demonstrate that eosinophils function as antigen-presenting cells for the induction of the primary and the expansion of the secondary Th2 immune responses to S. stercoralis in mice.
103 citations
Additional excerpts
...활성화된 호산구는 호산구량 이온 단백(eosinophil cationic protein)을 통한 콜라겐 수축, TGF-β, IL-1β 생성을 통한 섬유화 유도, T 세포로의 항 원 제시 증가를 통해 IgG 연관 질환 발병에 관여하는 것으 로 알려져 있다[18,19]....
[...]
••
TL;DR: The data suggest that EAF is part of the spectrum of IgG4-related systemic disease, whose longstanding disease was characterized principally by concentric perivascular fibrosis.
Abstract: BackgroundEosinophilic angiocentric fibrosis (EAF) is an uncommon tumefactive lesion of the orbit and upper respiratory tract of unknown etiology. The condition is characterized histologically by concentric layers of fibrosis around small-caliber arteries and a mixed inflammatory infiltrate dominate
103 citations
••
TL;DR: The serum IgG4 level was elevated in this 74-year-old female with pancreatic cancer as the result of an IgG 4-related systemic disease that had no clinical manifestations other than lymphadenopathy.
Abstract: Differentiation between autoimmune pancreatitis and pancreatic cancer is sometimes difficult. It has been reported that serum IgG4 concentrations are significantly elevated and particularly high (>135 mg/dL) in autoimmune pancreatitis. Measurement of serum IgG4 has become a useful tool for differentiating between autoimmune pancreatitis and pancreatic cancer. However, we present a 74-year-old female with a markedly elevated serum IgG4 (433 mg/dL) who underwent pancreaticoduodenectomy for pancreatic cancer. Elevated serum IgG4 levels continued after the resection. On histology, adenocarcinoma of the pancreas accompanied with moderate lymphoplasmacytic infiltration infiltrated the lower bile duct and duodenum, but there were no findings of autoimmune pancreatitis. Although a small metastasis was detected in one parapancreatic lymph node, regional lymph nodes were swollen. Abundant IgG4-positive plasma cells infiltrated the cancerous areas of the pancreas, but only a few IgG4-positive plasma cells were detected in the noncancerous areas. Pancreatic cancer cells were not immunoreactive for IgG4. An abundant infiltration of IgG4-positive plasma cells was detected in the swollen regional lymph nodes and in the duodenal mucosa. We believe that the serum IgG4 level was elevated in this patient with pancreatic cancer as the result of an IgG4-related systemic disease that had no clinical manifestations other than lymphadenopathy.
102 citations
Additional excerpts
...췌장암 환자의 4%∼10%에서도 혈액 내 IgG4가 상승되어 있다는 보고가 있어 해석에 주 의가 필요하다[35]....
[...]