scholarly journals Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Teresa Carbone ◽  
Ricardo Azêdo Montes ◽  
Beatriz Andrade ◽  
Pedro Lanzieri ◽  
Luis Mocarzel
Keyword(s):  
Elderly Patient ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Lymphocytic Infiltration ◽  
Serum Levels ◽  
High Serum ◽  
Orbital Pseudotumor ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.

scholarly journals Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Kazuichi Okazaki ◽  
Hisanori Umehara
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Levels ◽  
Mikulicz’S Disease ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Biopsy Specimens ◽  
Organ Specific ◽  
Low Sensitivity

Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.

scholarly journals Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Caroline G. Olson ◽  
Nancy Y. Olson
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Pelvic Mass ◽  
Lymphocytic Infiltration ◽  
Orbital Mass ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
New Development ◽  
Initial Improvement ◽  
Functional Antibodies

We report a case of IgG4-RD in a patient with high IgG4 levels, low functional antibodies, and low IgM levels. He presented with bilateral orbital pseudotumors and, after initial improvement on corticosteroids, relapsed with recurrent pleural effusion and pelvic pseudotumor. He had a grossly elevated serum IgG (1905 mg/dl) with elevations in all IgG subclasses but marked elevation in IgG4 (412 mg/dl), low IgM, and low pneumococcal antibodies. Orbital mass biopsy showed polyclonal lymphocytic infiltration and increased IgG4 plasma cells. The patient was started on prednisone and tried several immunosuppressive medications including mycophenolate mofetil, methotrexate, hydroxychloroquine, and azathioprine with decrease in size of the orbital pseudotumor. During a period when the patient stopped his medications, the pseudotumor enlarged with new development of recurrent pleural effusions. He was also found to have a pelvic mass that was biopsy positive for IgG4 proliferation. This case with progression to multiorgan involvement highlights the importance of identifying patients with IgG4-related disease. In contrast to previous cases with normal-to-high IgM, the IgM was low with impaired functional antibodies.

scholarly journals Urologic manifestations of IgG4-related disease

2020 ◽  
Vol 80 (5) ◽  
pp. 1-10
Author(s):  
Benjamin Enrique Montaño Roca ◽  
Davide Vanacore ◽  
Gustavo Gallegos Sánchez ◽  
César Eduardo Rosales Velázquez ◽  
Guillermo Enrique Ruvalcaba Oceguera ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Aged Patients ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Urinary Tract Symptoms ◽  
Serum Igg4 ◽  
Inflammatory Pseudotumors ◽  
Clinically Significant

IgG4-related disease (IgG4-RD) is a clinical entity characterized by elevated serum IgG4 and tumor-like inflammation, with tissue infiltration by IgG4 and plasma cells. IgG4-RD is rare, but clinically significant, and its urologic manifestations have been reported in the literature. The present review covers a broad spectrum, describing the pathologies related to the area of urology. In 2003, Terumi Kamisawa was the first to recognize IgG4-RD, characterized by multiorgan lesions in patients with autoimmune pancreatitis and classified as an inflammatory and fibrotic entity with a dense lymphoplasmacytic infiltrate, positive for  IgG4.(1–3) It presents in middle-aged patients, between 59-68 years of age, with no clear distribution by sex, (4–6) and has different clinical presentations. The main urologic manifestations are inflammatory pseudotumors and lower urinary tract symptoms. The present article offers a clear, general overview of the disease, encompassing its pathophysiology, diagnosis, and treatment, from the perspective of urology. 

scholarly journals IgG4-Related Disease: A Multispecialty Condition

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Iuri Usêda Santana ◽  
Emanuela Pimenta da Fonseca ◽  
Mittermayer Barreto Santiago
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Diffuse Abdominal Pain ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Serum Igg4 ◽  
Polyclonal Hypergammaglobulinemia ◽  
History Of ◽  
Systemic Condition

IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases.

scholarly journals Long-term effects of intensive B cell depletion therapy in severe cases of IgG4-related disease with renal involvement

2020 ◽  
Vol 68 (6) ◽  
pp. 340-352
Author(s):  
Giacomo Quattrocchio ◽  
Antonella Barreca ◽  
Andrea Demarchi ◽  
Roberta Fenoglio ◽  
Michela Ferro ◽  
...  
Keyword(s):  
B Cells ◽  
B Lymphocyte ◽  
Plasma Cells ◽  
Renal Involvement ◽  
Elevated Serum ◽  
Long Term Effects ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis

AbstractIgG4-related disease (IgG4-RD) is an immune-mediated disorder often showing elevated serum IgG4 concentrations, dense T and B lymphocyte infiltration, and IgG4-positive plasma cells and storiform fibrosis. We prospectively evaluated for 4 years 5 patients with histologically proven IgG4-RD of whom 3 had tubulointerstitial nephritis (TIN) and 2 had retroperitoneal fibrosis (RPF). They received an intensive B depletion therapy with rituximab. The estimated glomerular filtration rate of TIN patients after 1 year increased from 9 to 24 ml/min per 1.73 m2. IgG/IgG4 dropped from 3236/665 to 706/51 mg/dl, C3/C4 went up from 49/6 to 99/27 mg/dl, and the IgG4-RD responder index fell from 10 to 1. CD20+ B cells decreased from 8.7 to 0.5%. A striking drop in interstitial plasma cell infiltrate as well as normalization of IgG4/IgG-positive plasma cells was observed at repeat biopsy. Both clinical and immunological improvement persisted over a 4-year follow-up. Treating these patients who were affected by aggressive IgG4-RD with renal involvement in an effort to induce a prolonged B cells depletion with IgG4 and cytokine production decrease resulted in a considerable rise in eGFR, with IgG4-RD RI normalization and a noteworthy improvement in clinical and histological features. Furthermore, the TIN subgroup was shown not to need for any maintenance therapy.

scholarly journals The Utility of Serum IgG4 Concentrations as a Biomarker

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Shigeyuki Kawa ◽  
Tetsuya Ito ◽  
Takayuki Watanabe ◽  
Masahiro Maruyama ◽  
Hideaki Hamano ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
High Serum ◽  
Wall Thickening ◽  
Systemic Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Long Term Follow Up ◽  
Characteristic Features

IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation and having 6 characteristic features: (1) systemic involvement; (2) solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3) high serum IgG4 concentration >135 mg/dL; (4) abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5) a positive response to corticosteroid therapy; and (6) complications of other IgG4-related diseases. To date, most IgG4-related diseases have been recognized as extrapancreatic lesions of autoimmune pancreatitis. This paper will discuss the utility of IgG4 as a biomarker of IgG4-related diseases, including in the diagnosis of autoimmune pancreatitis and its differentiation from pancreatic cancer, in the prediction of relapse, in the long-term follow-up of patients with autoimmune pancreatitis and normal or elevated IgG4 concentrations, and in patients with autoimmune pancreatitis and extrapancreatic lesions, as well as the role of IgG4 in the pathogenesis of IgG4-related disease.

IgG4-related disease: an analysis of the clinicopathological spectrum: UK centre experience

2021 ◽  
pp. jclinpath-2021-207748
Author(s):  
Wajira Dassanayaka ◽  
Kanchana Sanjeewani Liyanaarachchi ◽  
Aftab Ala ◽  
Izhar N Bagwan
Keyword(s):  
Plasma Cells ◽  
High Serum ◽  
High Power Field ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Common Clinical Presentation ◽  
Characteristic Features

AimTo retrospectively evaluate the characteristic clinicopathological spectrum in patients with suspicion of IgG4-related disease (IgG4RD).MethodsWinpath histology database from January 2011 to April 2018 identified all suspected IgG4RD cases wherein IgG4 immunohistochemistry was performed. The histology slides were reviewed to categorise cases into Boston criteria groups—highly suggestive of IgG4RD, probable IgG4RD and insufficient evidence. Information regarding clinical data, treatment received, follow-up and serum IgG4 levels was obtained from medical records and AllScripts Patient Administration System (APAS) clinical database.ResultsThe study included 204 patients and the most common sites of biopsy/resection were pancreas and duodenum. The most common clinical presentation was fibroinflammatory lesion or mass/lump. On histology, 54/204 (26.47%) cases showed typical storiform fibrosis, 65/204 (32.64%) had >10 IgG4+ plasma cells per high power field and only one case showed thrombophlebitis (0.49%). There were 14/204 (6.78%) cases categorised as highly suggestive of IgG4RD; 8 of these showed high serum IgG4 levels and were managed clinically as true IgG4RD.ConclusionHistological diagnosis of IgG4RD remains challenging, as not all characteristic features are always present especially in small biopsies. Due to the novelty of its experience, fear of over diagnosis in the context of malignancy and features overlapping with diseases of similar clinical scenario, diagnosis of IgG4RD has become more puzzling. Further multicentre clinical trials/studies are advisable.

scholarly journals IgG4-Related Disease in a Urachal Tumor

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Travis W. Dum ◽  
Da Zhang ◽  
Eugene K. Lee
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Needle Aspiration ◽  
Lung Lesion ◽  
High Rate ◽  
Mediastinal Lymphadenopathy ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease is a newly recognized fibroinflammatory disorder that has the ability to affect nearly every organ system. It is characterized by tumefactive lesions and fibrosis and closely mimics neoplasms. Only one case of IgG4-related bladder mass has been reported in the literature, but there are no reports of IgG4-related disease in a urachal mass. Herein, we report a 26-year-old male who initially presented with symptoms of recurrent UTI. Work-up revealed a 6 cm urachal tumor, a 1.4 cm pulmonary lesion, and mediastinal lymphadenopathy; all metabolically active on PET scan and suspicious for urachal adenocarcinoma. Lung lesion fine needle aspiration and TURBT pathology revealed inflammation but no evidence of malignancy. The patient underwent a partial cystectomy and umbilectomy with pathology demonstrating dense plasmacytic cells, a high rate of immunohistochemistry staining positive for IgG4 plasma cells, a storiform pattern of fibrosis, and an obliterative phlebitis. Furthermore, the patient had an elevated serum IgG4 level of 227 mg/dL (range 2.4–121 mg/dL). IgG4-related disease is a newly recognized fibroinflammatory disorder that can mimic neoplastic processes and a high index of suspicion and accurate tissue pathology is necessary for an accurate diagnosis.

IgG4-related Diseases Including Mikulicz’s Disease and Sclerosing Pancreatitis: Diagnostic Insights

2010 ◽  
Vol 37 (7) ◽  
pp. 1380-1385 ◽  
Author(s):  
YASUFUMI MASAKI ◽  
SUSUMU SUGAI ◽  
HISANORI UMEHARA
Keyword(s):  
Differential Diagnosis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Treatment Strategies ◽  
Mikulicz’S Disease ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Sclerosing Pancreatitis ◽  
Systemic Condition

Since the first report of serum IgG4 elevation in sclerosing pancreatitis in 2001, various systemic disorders have been reported to elevate IgG4, and many names have been proposed from the perspective of the systemic condition. Despite similarities in the organs damaged in IgG4-related Mikulicz’s disease and Sjögren’s syndrome, there are marked clinical and pathological differences between the 2 entities. The majority of cases diagnosed with autoimmune pancreatitis in Japan are IgG4-related sclerosing pancreatitis, and it should be recognized that this is distinct from the Western type. Diagnosis of IgG4-related disease is defined by both elevated serum IgG4 (> 1.35 g/l) and histopathological features, including lymphocyte and IgG4+ plasma cell infiltration (IgG4+ plasma cells/IgG+ plasma cells > 50% on a highly magnified slide checked at 5 points). Differential diagnosis from other distinct disorders is necessary: these include sarcoidosis, Castleman’s disease, Wegener’s granulomatosis, lymphoma, cancer, and other existing conditions. The Japanese IgG4 research group has begun multicenter prospective studies to improve diagnostic criteria and treatment strategies.

scholarly journals The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease

2020 ◽  
Vol 21 (14) ◽  
pp. 5082
Author(s):  
Song-Chou Hsieh ◽  
Chieh-Yu Shen ◽  
Hsien-Tzung Liao ◽  
Ming-Han Chen ◽  
Cheng-Han Wu ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Malignant Neoplasms ◽  
Th2 Response ◽  
Obliterative Phlebitis ◽  
Inflammatory Reactions ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) is a spectrum of complex fibroinflammatory disorder with protean manifestations mimicking malignant neoplasms, infectious or non-infectious inflammatory process. The histopathologic features of IgG4-RD include lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis together with increased in situ infiltration of IgG4 bearing-plasma cells which account for more than 40% of all IgG-producing B cells. IgG4-RD can also be diagnosed based on an elevated serum IgG4 level of more than 110 mg/dL (normal < 86.5 mg/mL in adult) in conjunction with protean clinical manifestations in various organs such as pancreato–hepatobiliary inflammation with/without salivary/lacrimal gland enlargement. In the present review, we briefly discuss the role of genetic predisposition, environmental factors and candidate autoantibodies in the pathogenesis of IgG4-RD. Then, we discuss in detail the immunological paradox of IgG4 antibody, the mechanism of modified Th2 response for IgG4 rather than IgE antibody production and the controversial issues in the allergic reactions of IgG4-RD. Finally, we extensively review the implications of different immune-related cells, cytokines/chemokines/growth factors and Toll-like as well as NOD-like receptors in the pathogenesis of tissue fibro-inflammatory reactions. Our proposals for the future investigations and prospective therapeutic strategies for IgG4-RD are shown in the last part.

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