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.
2 citations
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18 Nov 2016References
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TL;DR: A case of IgG4-related dacryoadenitis that is believed to have regressed due to the administration of anti-allergic agents is reported, and the hypotheses of spontaneous remission and/or the effect of epinastine hydrochloride administration can be proposed regarding the mechanism by which the lacrimal gland lesion decreased in size.
Abstract: There are no reports on the effect of anti-allergic agents against IgG4-related disease. We herein report a case of IgG4-related dacryoadenitis that is believed to have regressed due to the administration of anti-allergic agents. A 57-year-old woman consulted us because of bilateral temporal upper eyelid swelling and induration. She had also been suffering from allergic rhinitis and allergic conjunctivitis for 20 years. We performed an incisional biopsy of the lesion. With respect to the pathology, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue type was strongly suspected. On obtaining consent from the patient, follow-up alone was to be continued without radiation therapy. In addition to the observation of lacrimal gland lesions, the administration of epinastine hydrochloride at a dosage of 20 mg/day and 0.01% betamethasone eye drops twice a day to both eyes was commenced in order to treat both allergic rhinitis and allergic conjunctivitis. The lacrimal gland lesion decreased in size over time, becoming predominantly normal 7 years after the commencement of agent administration. We therefore re-examined the blood and pathology specimens. As a result, the serum IgG4 level was found to have increased to 540 mg/dl, while IgG4/IgG was 36.2%. The pathological diagnosis was revised to IgG4-related dacryoadenitis. The hypotheses of spontaneous remission and/or the effect of epinastine hydrochloride administration can be proposed regarding the mechanism by which the lacrimal gland lesion decreased in size. [J Clin Exp Hematop 53(1): 53-56, 2013].
22 citations
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...치료 IgG4 연관 질환 환자 중 일부는 치료 없이 저절로 좋아지 기도 한다[76]....
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TL;DR: Careful consideration should be given to the possible presence of IgG4-RD when forensic pathologists encounter cases of sudden death accompanied by coronary periarteritis, and further investigation of these conditions and their association with coronary lesions is needed.
Abstract: IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by marked infiltration of IgG4-positive (+) plasma cells into affected organs, but the concept of this disease has only recently been established. Coronary vasculitis is a rare disease that can cause sudden death, and it has recently been reported that IgG4-RD may be associated with vasculitis, including periarteritis and coronary disease. In this paper we report an autopsy case of sudden death of a man in his thirties, in which coronary periarteritis with features of IgG4-related periarteritis was detected. IgG4-RD was suspected from the presence of the following histopathological features: (1) markedly thickened adventitia and marked infiltration of the adventitia and periarterial fat by lymphocytes and plasma cells; and (2) infiltration of IgG4-positive plasma cells (ratio of IgG4+ cells to IgG4+ cells of >40 %, 50 IgG4+ plasma cells per high-power field) on immunostaining. The etiology and pathophysiology of IgG4-RD and IgG4-related periarteritis are still unclear, and further investigation of these conditions and their association with coronary lesions is needed. Careful consideration should be given to the possible presence of IgG4-RD when forensic pathologists encounter cases of sudden death accompanied by coronary periarteritis.
20 citations
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...물게 보고된 바 있다[61]....
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TL;DR: The case report supports the literature that there are similarities between IgG4-related Mikulicz's disease and Sjögren's syndrome, but the differences are significant.
Abstract: Objectives This paper aims to report a case of IgG4-related Mikulicz's disease with a systematic review. Methods The relevant English literature was searched using the keywords 'Mikulicz's disease' and 'IgG4'. Original and review articles were reviewed, and the clinical scenarios were exemplified with a case report. Results A 49-year-old Caucasian man presented with axillary lymphadenopathy and bilateral parotid/submandibular enlargement. A chest computerized tomography showed mediastinal lymphadenopathy, with low metabolic activity on the position emission tomography. A histopathological study showed an IgG4/IgG ratio of 75% in the plasma cells of the submandibular glands, associated with high levels of total serum IgG and IgG4. He had dry mouth, but minor salivary gland biopsy was negative without xerophthalmia. He had nasal obstruction and dyspnea, notably with supine position/cervical rotation, which substantially improved with glucocorticoid treatment. He had newly diagnosed diabetes mellitus with hyperlipasaemia and diffuse pancreatic swelling supportive of autoimmune pancreatitis. Conclusions Our case report supports the literature that there are similarities between IgG4-related Mikulicz's disease and Sjogren's syndrome, but the differences are significant. IgG4-related Mikulicz's disease is a multi-organ lymphoproliferative disease distinct from Sjogren's syndrome.
20 citations
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...이드로 치료하면 증상이 호전될 수 있다[40]....
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TL;DR: These results suggest an association between the pathogeneses ofKT-S (−) and IgG4-DS, but not KT-S (+), as indicated in recent studies.
Abstract: Objectives
Kuttner tumour (KT), so-called chronic sclerosing sialoadenitis, is characterised by concomitant swelling of the submandibular glands secondary to strong lymphocytic infiltration and fibrosis independent of sialolith formation. However, recent studies have indicated that some patients with KT develop high serum levels of IgG4 and infiltration of IgG4-positive plasma cells, namely IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease. The aim of this study was to clarify the clinical and pathological associations between KT and IgG4-DS.
Materials and Methods
Fifty-four patients pathologically diagnosed with KT or chronic sialoadenitis were divided into two groups according to the presence or absence of sialolith (KT-S (+) or KT-S (−), respectively).
Results
There were no significant differences in the clinical findings, including the mean age, sex and disease duration, between the two groups. All patients in the KT-S (+) group showed unilateral swelling without infiltration of IgG4-positive plasma cells or a history of other IgG4-related diseases (IgG4-RD), while those in the KT-S (−) group showed bilateral swelling (37.5%), strong infiltration of IgG4-positive plasma cells (87.5%) and a history of other IgG4-RD (12.5%).
Conclusions
These results suggest an association between the pathogeneses of KT-S (−) and IgG4-DS, but not KT-S (+).
18 citations
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...로 하고 Kuttner 종양으로도 알려진 만성 경화성타액선염 또한 IgG4 연관 질환으로 알려져 있다[41]....
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TL;DR: The first case of acquired thrombotic throm bocytopenic purpura (TTP) in a patient with IgG4-related lung disease with severe deficiency of ADAMTS-13 activity and IgG 4 autoantibody against ADAMts-13 is described.
Abstract: IgG4-related disease is a recently recognized group characterized by elevated serum IgG4 levels and prominent lymphoplasmacytic infiltration of IgG4-positive cells into multiple organs (1). The condition was first described in relation to the pancreas (i.e., autoimmune pancreatitis [AIP] [2]), but since then many other inflammatory conditions associated with IgG4-related disease have been reported, including sclerosing cholangitis, sialadenitis, lymphadenopathy, retroperitoneal fibrosis, interstitial pneumonia, and tubulointerstitial nephritis (1,3,4). Laboratory findings in IgG4-related disease are commonly characterized by hypergammaglobulinemia and high levels of serum IgG and IgG4, and in addition, a high serum IgE level, peripheral eosinophilia, and hypocomplementemia are often observed (3,4). Thrombocytopenia in IgG4-related disease is rare, but a few cases of autoimmune thrombocytopenia have been reported in patients with AIP (5,6). We describe here, to our knowledge, the first case of acquired thrombotic thrombocytopenic purpura (TTP) in a patient with IgG4-related lung disease with severe deficiency of ADAMTS-13 activity and IgG4 autoantibody against ADAMTS-13.
13 citations
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...통한 항호중구 세포질항체(antineutrophil cytoplasmic antibodies) 연관 혈관염 발생에 관여[27], 보통천포창 (pemphigus vulgaris)의 질병 활성도와 desmoglein3에 대 한 IgG4의 비례 관계[28], ADAMTS13에 대한 IgG4가 혈 전성 혈소판감소성 자반증(thrombotic thrombocytopenic purpura) 발병과 관련이 있는 점 등이다[29]....
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