IgG4-related disease: How to place it in the spectrum of immune-mediated and rheumatologic disorders?

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.

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IgG4-Related Disease as Mimicker of Malignancy

SN Comprehensive Clinical Medicine ◽

10.1007/s42399-021-00957-6 ◽

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.

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

European Journal of Case Reports in Internal Medicine ◽

10.12890/2021_002981 ◽

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.

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

10.21203/rs.3.rs-169125/v1 ◽

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.

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IgG4-Related Disease as Mimicker of Malignancy

10.21203/rs.3.rs-58790/v1 ◽

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.

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

Journal of Clinical Images and Medical Case Reports ◽

10.52768/2766-7820/1161 ◽

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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Ultrasound and Sialogram Correlates to Parotid Immunoglobulin G4-Related Disease

Ear Nose & Throat Journal ◽

10.1177/01455613211051651 ◽

2021 ◽

pp. 014556132110516

Author(s):

Abigail E. Moore ◽

Kathryn S. Marcus ◽

Anand Rajan KD ◽

Joan E. Maley ◽

Henry T. Hoffman

Keyword(s):

Multimodality Imaging ◽

Clinical History ◽

Immunoglobulin G4 ◽

Related Disorder ◽

Radiographic Findings ◽

Process Diagnosis ◽

Related Disease ◽

Igg4 Related Disease ◽

Immune Mediated

Immunoglobulin G4 (IgG4)-related disease is an immune-mediated disorder that commonly manifests in the salivary glands. As a recently described disorder, the description and classification of IgG4-related disease is an ongoing process. Diagnosis of IgG4-related disease requires integration of clinical history, histopathology, and radiographic findings, including ultrasonography and sialography. In this case report, we correlate parotid ultrasonographic and sialographic findings in a patient with proven IgG4-related disorder confirmed from analysis of previous submandibular gland resections. We aim to highlight the utility of multimodality imaging in the diagnosis of IgG4-related disease.

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IgG4-related disease: can non-classical histopathological features or the examination of clinically uninvolved tissues be helpful in the diagnosis?

Journal of Clinical Pathology ◽

10.1136/jclinpath-2012-200932 ◽

2012 ◽

Vol 65 (11) ◽

pp. 963-969 ◽

Cited By ~ 16

Author(s):

Emma L Culver ◽

Adrian C Bateman

Keyword(s):

Cell Count ◽

Plasma Cell ◽

Supporting Evidence ◽

Diagnostic Features ◽

Histopathological Features ◽

Related Disease ◽

Igg4 Related Disease ◽

Multisystem Involvement ◽

Immune Mediated ◽

Serum Igg4

IgG4-related disease (IgG4-RD) is an increasingly recognised inflammatory and fibrosing condition that commonly shows multisystem involvement. The disease may mimic malignancy and other inflammatory or immune-mediated disorders, but usually has a good response to corticosteroid therapy, underlining the requirement for recognition of the condition. Accurate diagnosis requires careful interpretation of varying combinations of serum IgG4 levels, radiological features and characteristic histopathological appearances within an appropriate clinical setting. The presence of ‘classical’ histopathological features together with an elevated tissue IgG4+ plasma cell count and IgG4 to IgG ratio is often diagnostic and at the very least can strongly support a clinicopathological diagnosis of IgG4-RD. The authors describe the most recent diagnostic criteria for IgG4-RD, especially the histopathological features. The authors then discuss the merits of examining tissues that may be more easily obtainable than those that commonly show the ‘classical’ histopathological features, but within which not all of these ‘diagnostic’ features may be present. The authors conclude that while a combination of ‘classical’ histopathological features and an elevated tissue IgG4+ plasma cell count is the gold standard for diagnosis, examination of tissues that show some but not all of these features can, in the appropriate context, provide useful supporting evidence for a clinicopathological diagnosis of IgG4-RD.

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An old disease, a new diagnosis; A 49 year-old man with nephrotic syndrome

Journal of Nephropathology ◽

10.15171/jnp.2019.32 ◽

2019 ◽

Vol 8 (3) ◽

pp. 32-32

Author(s):

Santiago Cedeño ◽

Ursula Verdalles ◽

Marian Goicoechea ◽

Soledad García de Vinuesa ◽

José Luño

Keyword(s):

Nephrotic Syndrome ◽

T Lymphocyte ◽

Transforming Growth Factor ◽

Organ System ◽

Related Disease ◽

Igg4 Related Disease ◽

Immune Mediated ◽

Mikulicz Disease ◽

New Diagnosis ◽

Regulatory T Lymphocyte

Background: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that typically manifests as fibro-inflammatory masses that can affect nearly any organ system. Case Report: We present here a case of a 49-year old man with forgotten old disease (Mikulicz disease) with membranous nephropathy (MN). Conclusions: This entity is currently included within the spectrum of IgG4-related disease. The development of renal disease shortly after the suspension of rituximab suggests another probable pathway involved. To our knowledge the transforming growth factor may be responsible for existing pattern of fibrosis in this disease. The lack of response or at least partial response to rituximab can be explained by greater involvement of regulatory T lymphocyte in the pathophysiology of this entity.

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Relapsing Polychondritis or RP-like Symptoms Associated With IgG4-RD. Case Reports and Review of the Literature

Modern Rheumatology Case Reports ◽

10.1093/mrcr/rxab033 ◽

2021 ◽

Author(s):

Jesica Romina Gallo ◽

Maria Marcela Schmid ◽

Josefina Gallino Yanzi ◽

Sergio Oscar Paira

Keyword(s):

Autoimmune Disease ◽

Plasma Cells ◽

Case Reports ◽

Relapsing Polychondritis ◽

Review Of The Literature ◽

Related Disease ◽

Igg4 Related Disease ◽

Immune Mediated ◽

Systemic Disorder

Abstract Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by the infiltration of IgG4-bearing plasma cells into systemic organs. Although 25% to 35% of patients with RP have a concurrent autoimmune disease. The coexistence of RP and IgG4 is rare considering that, to the best of our knowledge, there are only four previous reports of RP or RP-like symptoms associated with IgG4-RD. (1-4) We herein report two cases which could be RP or RP-like symptoms associated with IgG4-RD.

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