scholarly journals IgG4-Related Disease, the Malignancy Mimicker: Case Series from Bahrain

2018 ◽  
Vol 2018 ◽  
pp. 1-9
Author(s):  
Majeed Haider ◽  
Fatima Haji ◽  
Osama Alalwan ◽  
Eman Aljufairi ◽  
Tejal S. Shah
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Case Series ◽  
Variable Degree ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Serum Igg4 ◽  
Igg4 Level ◽  
Single Organ

IgG4-related disease is an evolving immune-mediated condition. The hallmark of this condition is IgG4(+) plasma cells infiltration of the affected organs accompanied by a variable degree of fibrosis and occasionally elevated serum IgG4 level. It links many conditions that were once recognized as isolated unrelated idiopathic single organ disorders (e.g., autoimmune pancreatitis, Mikulicz syndrome, and retroperitoneal fibrosis) under one umbrella. It usually presents clinically as tumor-like swelling of the involved organs that can be misdiagnosed as neoplasia. In this case series, we present four cases that were considered as neoplasia but turned out to be IgG4-related disease, we demonstrate the protean manifestations of this condition and variable organs involvement, and we share our experience in using rituximab as the steroid sparing immunosuppressant agent to control this disease.

scholarly journals IgG4-related disease in a patient with HIV infection

2019 ◽  
Vol 12 (4) ◽  
pp. e226809
Author(s):  
Raquel Ron ◽  
Ignacio Ruz-Caracuel ◽  
Eugenia García ◽  
María Luisa Montes-Ramírez
Keyword(s):  
Lymph Nodes ◽  
Elevated Serum ◽  
Lung Damage ◽  
Axillary Lymph Nodes ◽  
Hiv Positive ◽  
Axillary Lymph ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

A 47-year-old HIV-positive man with good immune and virological status presented with chronic multiple enlarged lymph nodes, lung disease and eosinophilia. Radiologic tests showed enlarged cervical, thoracic and axillary lymph nodes, with interstitial lung damage. After several non-specific histologic studies, an elevated serum IgG4 level led us to request immunohistochemistry of a lymph node sample. The test confirmed the diagnosis of IgG4-related disease.

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 of the Oral Cavity. Case Series from a Large Single-Center Cohort of Italian Patients

2020 ◽  
Vol 17 (21) ◽  
pp. 8179
Author(s):  
Andrea Rampi ◽  
Marco Lanzillotta ◽  
Gaia Mancuso ◽  
Alessandro Vinciguerra ◽  
Lorenzo Dagna
Keyword(s):  
Oral Cavity ◽  
Plasma Cells ◽  
Clinical Symptoms ◽  
Case Series ◽  
Histological Evaluation ◽  
High Power Field ◽  
Entire Cohort ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

A series of destructive and tumefactive lesions of the oral cavity are increasingly recognized as part of the IgG4-related disease (IgG4-RD) spectrum. We herein examined the clinical, serological, radiological, and histological features of a series of patients referred to our clinic because of oral cavity lesions ultimately attributed to IgG4-RD. In particular, we studied 6 consecutive patients out of 200 patients referred to the immunology outpatient unit who presented with erosive and/or tumefactive lesions of the oral cavity. All patients underwent serum IgG4 measurement, nasal endoscopy, radiological studies, and histological evaluation of tissue specimens. The histological studies included immunostaining studies to assess the number of IgG4+ plasma cells/High-Power Field (HPF) for calculation of the IgG4+/IgG+ plasma cell ratio. Six patients (3% of the entire cohort) were diagnosed with IgG4-RD of the oral cavity based on histological evaluation. A major complaint at presentation was oral discomfort due to bulging mass. A mild to no increase in serum IgG4 was observed. Different patterns of organ involvement were associated with oral lesions. Five patients were treated with immunosuppressive therapy and two patients promptly responded to B-cell depletion with rituximab. Watchful waiting was decided in one patient with no major clinical symptoms. Involvement of the oral cavity is an infrequent manifestation of IgG4-RD but should be taken into consideration as a possible differential diagnosis of tumefactive or erosive lesions once neoplastic conditions are excluded. A histological examination of biopsy samples from the oral cavity represents the mainstay for diagnosis of IgG4-RD.

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 IgG4 Related Disease of The Larynx Mimicking Malignancy: A Case Report and Review of the Literature

2021 ◽  
Author(s):  
Sevda Akyol ◽  
Ozlem Saraydaroglu ◽  
Omer Afsin Ozmen
Keyword(s):  
Plasma Cells ◽  
Clinical Laboratory ◽  
Review Of The Literature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration ◽  
Igg4 Level

Abstract Objectives: Immunoglobulin G4–related disease is characterized by increased serum IgG4 level, enlargement in the relevant organs and histopathologically intense storiform fibrosis, lymphoplasmacytic infiltration rich in IgG4 positive plasma cells, and obliterative phlebitis.Methods and Results: In this report, a patient who underwent a laryngeal biopsy with a pre-diagnosis of malignancy, but had findings consistent with immunoglobulin G4–related disease in the biopsy sample, is described.Conclusion: Immunoglobulin G4–related disease can be seen in very rare localizations. It should be kept in mind in differential diagnosis when tissues especially containing inflammation rich in plasma cells are encountered. Clinical, laboratory and pathological correlation is extremely important in the diagnosis of an IgG4-related disease.

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.

scholarly journals A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Kazuhiko Higashioka ◽  
Kenji Yoshida ◽  
Kensuke Oryoji ◽  
Kazuo Kamada ◽  
Shinichi Mizuki ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Elevated Serum ◽  
Clinical Findings ◽  
Organ Involvement ◽  
Immunoglobulin G4 ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

We report a case of IgG4-related disease (IgG4-RD) with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

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.

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