scholarly journals IgG4-Related Disease as Mimicker of Malignancy

2021 ◽  
Author(s):  
WanLi Zhou ◽  
Timothy Murray ◽  
Liliana Cartagena ◽  
Howard Lim ◽  
David F. Schaeffer ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Clinical Laboratory ◽  
Early Recognition ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Single Center Study ◽  
Pancreatic Masses ◽  
Histological Data ◽  
Mass Lesions

Abstract Background IgG4-related disease (IgG4-RD) is an immune-mediated disease that may present as a tumefactive lesion in nearly any organ. These mass lesions often resemble malignancy both clinically and radiologically, and some patients undergo surgical resection which could possibly be avoided with early recognition of IgG4-RD. We performed a retrospective single-center study examining how many patients with IgG4-RD were initially believed to have malignancy, with particular attention to those who underwent potentially avoidable surgical procedures. Methods Sixty-three patients with biopsy confirmed IgG4-related disease were included. Clinical, laboratory, radiological, and histological data were collected and analyzed. Results Over 60% of patients (38/63) were initially thought to have a malignancy when they initially presented with symptomatic IgG4-RD. The most common types of malignancy suspected were lymphoma (18/38) and pancreatic cancer (11/38). Of the 38 patients with suspected malignancy, 14 underwent an invasive intervention either to alleviate the severity of their symptoms or as treatment for their presumed malignancy. These included Whipple resection/attempted Whipple (3), nephrectomy (3), bile duct resection and reconstruction (1), removal of other abdominal/retroperitoneal masses (3), and stenting of obstructed organs (4). Conclusion IgG4-RD should be on the differential diagnosis of patients with mass lesions, in particular those with pancreatic masses and obstructive jaundice, extensive lymphadenopathy, or retroperitoneal masses. Oncologists and other physicians involved in cancer care should be aware of the various manifestations and diagnostic approach to IgG4-RD in order to provide accurate diagnosis and minimize unnecessary invasive procedures. While some procedures in this study, such as stenting of obstructed organs, were required regardless of diagnosis, others could have potentially been avoided or attenuated with early recognition of IgG4-RD.  Patients with mass lesions suspicious for IgG4-RD should have serum protein electrophoresis, IgG subclass measurement, and, where possible, tissue biopsy before undergoing major surgical resection. Consultation with a physician experienced in IgG4-RD is recommended.

scholarly journals IgG4-Related Disease as Mimicker of Malignancy

2020 ◽  
Author(s):  
WanLi Zhou ◽  
Timothy Murray ◽  
Liliana Cartagena ◽  
Howard Lim ◽  
David F. Schaeffer ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
International Consensus ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Single Center Study ◽  
Pancreatic Masses ◽  
Histological Data ◽  
Mass Lesions ◽  
Whipple Resection

Abstract Background: IgG4-related disease is an immune-mediated disease that may present as a tumefactive lesion in nearly any organ. These mass lesions often resemble malignancy both clinically and radiologically, and some patients undergo unnecessary surgical resection. We performed a retrospective single center study examining how many patients with IgG4-RD were initially believed to havemalignancy,with particular attention to those who underwent potentially avoidable surgical procedures. Methods: 63 patients with biopsy confirmed IgG4-Related Disease based on International Consensus Criteria were included. Clinical, laboratory, radiological and histological data were collected and analyzed.Results: Over 60% of patients (38/63) were initially thought to have a malignancywhen they initially presented with symptomatic IgG4-RD. The most common types of malignancy suspected were lymphoma (18/38) and pancreatic cancer (11/38). Of the 38 patients with suspected malignancy, 14 underwent an invasive intervention either to alleviate the severity of their symptoms or as treatment for their presumed malignancy.These included Whipple resection/attempted Whipple (3), nephrectomy (3), bile duct resection and reconstruction (1), removal of other abdominal/retroperitoneal masses (3), and stenting of obstructed organs (4).Conclusion: IgG4-RD should be on the differential diagnosis of patients with mass lesions, in particular those with pancreatic masses and obstructive jaundice, extensive lymphadenopathy, or retroperitoneal masses. Oncologists and other physicians involved in cancer care should be aware of the various manifestations and diagnostic approach to IgG4-RD in order to provide accurate diagnosis and minimize unnecessary invasive procedures.

scholarly journals Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case–control study based on 450 patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Qiaozhu Zeng ◽  
Jingyuan Gao ◽  
Xinyu Zhang ◽  
Aichun Liu ◽  
Zhenfan Wang ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Clinical Laboratory ◽  
Imaging Finding ◽  
Age At Onset ◽  
Female Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Peking University ◽  
Chinese Cohort ◽  
Male To Female

AbstractWe aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD−) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+. We retrospectively analyzed the medical records of 470 IgG4-related disease (IgG4-RD) patients at Peking University People’s Hospital from January 2004 to January 2020. The demographic, clinical, laboratory, radiological and pathological characteristics between IgG4-RKD+ and IgG4-RKD− were compared. Twenty IgG4-RD patients who had definite etiology of renal impairment including diabetes, hypertension and etc. were excluded. Among the remained 450 IgG4-RD patients, 53 were diagnosed with IgG4-RKD+ . IgG4-RKD+ patients had older age at onset and at diagnosis. Male to female ratio of IgG4-RKD+ patients is significantly higher. In the IgG4-RKD+ group, the most commonly involved organs were salivary gland, lymph nodes and pancreas. It was found that renal function was impaired in approximately 40% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions. Male sex, more than three organs involved, and low serum C3 level were risk factors for IgG4-RKD+ in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

scholarly journals Immunoglobulin G4-related disease - diagnostic dilemma and importance of clinical judgement: a case report

2020 ◽  
Vol 8 (11) ◽  
pp. 4144
Author(s):  
Jagadeesh Chandrasekaran ◽  
Phani Krishna Machiraju
Keyword(s):  
Early Recognition ◽  
Elevated Serum ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Inflammatory Disorder ◽  
Imaging Features ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
The Right

Immunoglobulin G4 (IgG4)-related disease is a multi-organ, immune-mediated, fibro-inflammatory disorder characterized by tumefactive masses in the affected organs. Incidence and prevalence of IgG4-related disease (RD) are not clearly known and have slight male preponderance. It often involves multiple organs at the time of presentation or over the course of disease mimicking malignancy, Sjogren's syndrome, antineutrophil cytoplasmic antibodies associated vasculitis, infections. A thorough workup is needed to rule out these mimickers. A 33-year-old gentleman presented to us with history of progressive swelling in the right peri-orbital region for four years. On evaluation, abdominal imaging was notable for the sausage-shaped pancreas and hypoenchancing nodules in bilateral kidneys. Histological examination of right lacrimal gland revealed lymphoplasmacytic infiltrate and storiform fibrosis. Serum IgG4 levels were normal, and immunostaining was negative. A diagnosis of IgG4-RD was suggested because of multi-organ involvement, classical radiological and histopathological features. Awareness about IgG4-RD, an under-recognized entity is essential, as it is treatable, and early recognition may help in a favourable outcome. Appropriate use of clinicopathological, serological and imaging features in the right clinical context may help in accurate diagnosis. Elevated serum IgG4 levels and biopsy are not mandatory for the diagnosis.

scholarly journals Clinical Manifestations of Orbital Involvement in IgG4-Related Disease: A Retrospective Study of 573 Patients

2021 ◽  
Author(s):  
Linyang Gan ◽  
Xuan Luo ◽  
Yunyun Fei ◽  
Linyi Peng ◽  
Jiaxin Zhou ◽  
...  
Keyword(s):  
Lacrimal Gland ◽  
Clinical Laboratory ◽  
Clinical Manifestations ◽  
Chinese Patients ◽  
Laboratory Findings ◽  
Male Predominance ◽  
Orbital Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

Abstract Purpose: To investigate the clinical manifestations of orbital involvement in a large cohort of Chinese patients with IgG4-related disease (IgG4-RD). Methods: A total of 573 patients with IgG4-related disease were included. We described and compared the demographic, clinical, laboratory and histopathologic findings from 314 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 259 with extra-ophthalmic IgG4-RD.Results: Male predominance was found significant in extra-ophthalmic IgG4-RD only. Patients with IgG4-ROD showed younger age at diagnosis and longer duration from onset till diagnosis. In patients with extra-ophthalmic IgG4-RD, the most commonly involved extra-ophthalmic organ was pancreas; while in IgG4-ROD patients, salivary gland was most frequently affected. Multivariate analysis exhibited IgG4-ROD was associated with allergy history, higher serum IgG4/IgG ratio, multiple organs involvement and sialoadenitis. Orbital images were reviewed in 173 (55.1%) IgG4-ROD patients. Fifty-one (29.5%) patients had multiple lesions. Lacrimal gland involvement was detected in 151 (87.3%) patients, followed by extraocular muscles (40, 23.1%), other orbital soft tissue (40, 23.1%) and trigeminal nerve (8, 4.6%). Biopsy was performed from various organs in 390 cases. A dense lymphoplasmacytic infiltration and fibrosis were the main feature in orbital specimens. Storiform fibrosis and obliterative phlebitis were absent in lacrimal gland.Conclusions: Lacrimal gland involvement was the most common orbital manifestation of IgG4-ROD. Patients with IgG4-ROD showed different characteristic in demographic, clinical, laboratory findings compared to patients with extra-ophthalmic IgG4-RD. These features might indicate potential differences in the pathogenesis of these two subgroups of IgG4-RD.

Thoracic Involvement in IgG4-Related Disease

2020 ◽  
Vol 41 (02) ◽  
pp. 202-213 ◽  
Author(s):  
Marta Casal Moura ◽  
Ria Gripaldo ◽  
Misbah Baqir ◽  
Jay H. Ryu
Keyword(s):  
Clinical Laboratory ◽  
Wide Spectrum ◽  
The Body ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
First Line Therapy ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Histopathologic Features

AbstractImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that has been recognized to involve virtually any organ in the body and typically manifests mass-like lesions (tumefactive). Although initial reports of this disease (autoimmune pancreatitis [AIP]) were described in the Japanese population, it has since been reported worldwide. It is most commonly seen in adults of middle age or older, more often men than women. The pathogenesis of IgG4-RD is largely unknown, but genetic factors, microorganisms, and autoimmunity are thought to play important roles. Serum IgG4 concentration is elevated in the majority of patients with IgG4-RD but is a nonspecific finding. Characteristic histopathologic features include dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis. Lung involvement in IgG4-RD was first reported in 2004 in two patients with AIP and coexisting interstitial lung disease. Since then, a wide spectrum of intrathoracic involvement has been reported and includes not only parenchymal lung diseases but also pleural, airway, vascular, and mediastinal lesions. Thoracic involvement in IgG4-RD is often found incidentally during the workup of extrathoracic lesions but can sometimes be the presenting abnormality. The diagnosis of IgG4-RD requires correlation of clinical, laboratory, imaging, and histopathologic features. Glucocorticoids are the first-line therapy but other options including B cell depletion are being investigated. IgG4-RD is generally associated with an indolent clinical course and most patients improve with glucocorticoid therapy.

scholarly journals Systemic Amyloidosis as a Rare Complication of IgG4 Related Disease: Case Report and Literature Review

2021 ◽  
Author(s):  
Leonardo Oliveira Mendonca ◽  
Henrikki Gomes Antila ◽  
Alex Isidoro Prado ◽  
Luiz Augusto Marcondes Fonseca ◽  
Miton de Arruda Martins ◽  
...  
Keyword(s):  
Rare Complication ◽  
Systemic Amyloidosis ◽  
Aa Amyloidosis ◽  
Amyloid A ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Inflammatory Conditions ◽  
Immune Mediated ◽  
Brazilian Patient ◽  
Disease Case

Abstract Immunoglobulin 4 Related Disease (IgG4-RD) is immune-mediated fibroinflammatory disease and despite recent advances the immunological process involved in the disease pathogenesis is still unclear. Serum amyloid A (SAA) the precursor protein in AA amyloidosis is induced by inflammatory mediators such as IL-1, IL-6 and TNF cytokines. The treatment of AA amyloidosis is directed by the theoretical cytokine involved in the underlying inflammatory condition. Many inflammatory conditions has already been associated to AA amyloidosis and secondary to IgG4-RD seems to be rare. Here we report the case of a Brazilian patient with IgG4-RD with a fatal evolution of systemic amyloidosis. We also revised the cases already reporte in the literature with IgG4-RD and systemic amyloidosis.

scholarly journals IgG4-related Disease Presenting With Ataxia 

2021 ◽  
Author(s):  
George Hondag Tse ◽  
Marios Hadjivassiliou ◽  
Vikrant Devgire ◽  
Esther Hobson ◽  
Daniel JA Connolly ◽  
...  
Keyword(s):  
Radiological Feature ◽  
Restricted Diffusion ◽  
Wall Thickening ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Ct Angiogram ◽  
Cerebellar Peduncles ◽  
System Disorder ◽  
Mass Lesions

Abstract Background:Immunoglobulin G4 related disease is a relatively rare multi-system disorder which can present with diverse manifestations including mass-lesions and, or, organ dysfunction. Although the orbits, salivary glands and sinuses are comparatively common sites of involvement there are few reports of isolated intracranial presentation. Case presentation:Although large vessel IgG4-RD vasculitis is an uncommon entity we report a case of a 84-year old male presenting with cerebellar ataxia. CT at presentation demonstrated bilateral low-density change in the middle cerebellar peduncles, which corelated to areas of elevated T2 signal demonstrating restricted diffusion on MRI. CT angiogram demonstrated marked thickening of the common carotid arteries and branches extending to the skull base. In addition, the vertebral arteries also demonstrated significant wall thickening and occlusive narrowing. Serum immunoglobulin IgG and IgA were elevated and in particular IgG immunoglobulin was extremely high.Conclusions:Bilateral symmetrical restricted diffusion in the middle cerebellar peduncles is a striking radiological feature and reported to occur with multiple aetiologies, we believe this is the first report to associate this with IgG4-RD and ataxia.

scholarly journals IgG4-Related Disease Affecting Testicle and Myelodysplastic Syndrome: Just a Coincidence?

2021 ◽  
Author(s):  
Carla Alvarez Gonzalez ◽  
Juan Luis Carrillo Linares ◽  
Isabel García Muñoz ◽  
Andrea Escalona García ◽  
Pedro Valdivielso
Keyword(s):  
Myelodysplastic Syndrome ◽  
Blood Cells ◽  
Serum Concentrations ◽  
Immunoglobulin G4 ◽  
Medical Treatments ◽  
Hematopoietic Stem ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Uncommon Case

Immunoglobulin G4-related disease (IgG4-RD) is progressive immune-mediated fibrotic condition characterised by a tendency to form tumefactive lesions in different organs and by elevated IgG4 serum concentrations. Urological manifestations are rare and normally occur together with other systemic affections. Myelodysplastic syndromes (MDS) are hematopoietic stem cell neoplasms, with different subtypes based on the type of blood cells involved. MDS can be associated with other pathologies or medical treatments. We present an uncommon case of testicular manifestation associated with myelodysplastic syndrome here.

scholarly journals Case Report of Patient with IgG4-Related Disease and Renal Cell Carcinoma

2021 ◽  
Author(s):  
Zhihao Li ◽  
Zongyu Zheng ◽  
Meishan Jin ◽  
Weigang Wang
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Left Kidney ◽  
Surgical Removal ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Surgical Methods

Abstract Cases of immunoglobulin G4-related disease (IgG4-RD) along with renal cell carcinoma are extremely rare. In this study, we report on a 61-year-old male who was diagnosed with both IgG4-RD and renal cell carcinoma. We examined the period beginning 13 years before the renal cancer diagnosis to two months after surgical removal of the tumor. IgG4-RD is an immune-mediated disease that leads to the formation of multi-system masses. It affects almost every organ and is challenging to diagnose and cure in clinical settings [1]. In this case, we report the clinical data, surgical methods, pathology, and immunohistochemical characteristics of a patient who was diagnosed with IgG4-RD 7 years ago and diagnosed with a tumor in the left kidney by our department in August 2020.

scholarly journals Case report of isolated gastric IgG4-related lesion and series of literature review

2021 ◽  
Vol 2 (3) ◽  
Author(s):  
Xiang-Xi Ye ◽  
◽  
Tian-Hui Zou ◽  
Juan Tan ◽  
Zheng Wang ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Gastrointestinal Stromal Tumors ◽  
Gastrointestinal Involvement ◽  
Stromal Tumors ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Orbital Tissue ◽  
Fibrous Tumor

IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that responds to glucocorticoids, which is gradually well known in recent years. It can involve multiple organs of patients, including pancreas, bile duct, gallbladder, salivary gland, orbital tissue, lung, liver, lacrimal gland, kidney, retroperitoneal, aorta, thyroid and lymph node, [1] in which gastrointestinal involvement is relatively rare [2]. Most of the known reports of isolated gastric IgG4-related lesions were accidental found (in physical examination or other site examination), and often treated as Gastrointestinal Stromal Tumors (GIST) lead to surgically resection. In addition, Calcified Fibrous Tumor (CFT) in gastrointestinal tract often occurs with submucosa [3] is histologically similar to IgG4-RD, [4] whether it belongs to IgG4-RD is still controversial.

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