IgG4-assoziierte Orbitopathie als wichtige Differenzialdiagnose eines fortgeschrittenen Silent-Sinus-Syndroms

Abstract Background Immunoglobulin (Ig)G4-related disease is classified as an immune-mediated disease. The etiology of this condition has not been explained to date. Manifestations of the disease are diverse, and simultaneous involvement of multiple organs is not unusual. Case report We report the case of a patient referred to us after multiple unsuccessful paranasal sinus operations who presented with enophthalmos and a resultant migratory keratitis with a suspected diagnosis of silent sinus syndrome. Preservation of the orbit was no longer feasible. After five years without a definitive diagnosis, we ascertained that this was a case of IgG4-related disease. Discussion IgG4-related disease represents an important element in the differential diagnosis of chronic advanced diseases of the orbit and paranasal sinuses. The diagnosis should be considered in the case of unclear disease presentations. Typical histological findings include a storiform pattern of fibrosis, vasculopathy, and tissue infiltration by IgG4 plasma cells.

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Localized IgG4-related disease manifested on the tongue: a case report

Upsala Journal of Medical Sciences ◽

10.48101/ujms.v126.6118 ◽

2021 ◽

Vol 126 (1) ◽

Author(s):

Helya Hashemi ◽

Andreas Thor ◽

Erik Hellbacher ◽

Marie Carlson ◽

Miklós Gulyás ◽

...

Keyword(s):

Case Report ◽

Oral Mucosa ◽

Plasma Cells ◽

Clinical Manifestations ◽

Assessment System ◽

Immunoglobulin G4 ◽

Related Disease ◽

Multiple Organs ◽

Igg4 Related Disease ◽

Initial Symptoms

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs. IgG4-RD may show a variety of initial symptoms. In the oral mucosa, lesions present as inflammatory fibrosis with a large number of IgG4-positive plasma cells. Evaluating treatment is a well-known problem in IgG4-RD due to the absence of an established assessment system. There are difficulties in defining the severity of the disease, which is why treatment is primarily based on its clinical manifestations. We present a case report of localized IgG4-RD with ulcerative and proliferative manifestations on the tongue, which clinically mimicked oral squamous cell carcinoma. A tumor-like lesion on the tongue can indicate something else other than the malignant or reactive changes commonly found in the oral mucosa. Multiple differential diagnoses of these atypical oral lesions, including localized IgG4-RD, should be considered.

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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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Inferior vena cava thrombosis as the initial presentation of IgG4-related retroperitoneal fibrosis: Case report and literature review

Journal of Scleroderma and Related Disorders ◽

10.1177/2397198318764793 ◽

2018 ◽

Vol 3 (3) ◽

pp. NP1-NP6 ◽

Cited By ~ 1

Author(s):

Theodora Simopoulou ◽

Serafim Klimopoulos ◽

Dimitrios Sampaziotis ◽

Apostolos Tzortziotis ◽

Dimitrios Bogdanos ◽

...

Keyword(s):

Inferior Vena Cava ◽

Inferior Vena ◽

Plasma Cells ◽

Retroperitoneal Fibrosis ◽

Vena Cava ◽

Idiopathic Retroperitoneal Fibrosis ◽

Multiple Organs ◽

Igg4 Related Disease ◽

Immune Mediated ◽

Vena Cava Thrombosis

IgG4-related disease is an immune-mediated deposition of fibro-inflammatory tissue with IgG4-positive plasma cells and dense fibrosis in a single or multiple organs. It often raises concern for malignancy requiring biopsy for diagnosis. Presentation may vary according to organ involved. IgG4-related retroperitoneal fibrosis accounts for two-thirds of the previously considered idiopathic retroperitoneal fibrosis cases. In IgG4-related retroperitoneal fibrosis, computed tomography scan or magnetic resonance imaging shows periaortic soft tissue that extends from below the kidneys to iliac arteries and entrap ureters causing hydronephrosis and renal failure. We present a rare case of IgG4-related retroperitoneal fibrosis presenting with leg swelling and pain due to inferior vena cava compression and thrombosis, and we review current concepts on disease pathogenesis, diagnosis and treatment.

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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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IgG4-Related Disease With Testicular Involvement: A Case Report and Review of Literature

Frontiers in Immunology ◽

10.3389/fimmu.2021.717902 ◽

2021 ◽

Vol 12 ◽

Author(s):

Gang Wang ◽

Ning Zhuo ◽

Xiaowen Luo ◽

Feng Tian ◽

Zhenhua Wen ◽

...

Keyword(s):

Plasma Cells ◽

Young Male ◽

Reproductive Organ ◽

High Power Field ◽

Serological Tests ◽

Immunoglobulin G4 ◽

Related Disease ◽

Multiple Organs ◽

Igg4 Related Disease ◽

The Right

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune inflammatory disease characterized by infiltration of IgG4+ plasma cells that can simulate a tumor manifesting as a tumor-like mass. This disease involves the pancreas, biliary tract, kidneys, salivary glands, lymph nodes, aorta, and retroperitoneum amongst other organs. However, testicular involvement is a rare entity in this disease. The treatment of testicular involvement in IgG4-RD is currently controversial. We present the case of a 65-year-old man with swelling and pain in his right scrotum three months ago. On examination, a mobile mass of approximately 2 cm in diameter was found in the right scrotum. Serological tests showed elevated levels of IgG4 and negative for tumor markers. Enhanced computed tomography of the scrotum showed a nodular hyperdense shadow with a diameter of approximately 23 mm on the right epididymis. Pathological biopsy of the right epididymis showed infiltration of plasma cells, lymphocytes, and a few neutrophils. IgG4+ plasma cells stained positive, with an IgG4/IgG ratio of more than 40% and more than 30 IgG4+ plasma cells per high-power field. A diagnosis of IgG4-RD involving the testicles was made. Prednisone 30 mg/d was given for three weeks. No scrotum swelling or pain was observed at the follow-up after six months. IgG4-related disease should be considered whenever a mass-like lesion with typical histomorphologic features involving multiple organs/anatomical sites is encountered. The testicles are an important male reproductive organ, especially for young male patients with fertility requirements. For patients with IgG4-RD testicular involvement, surgical or medical treatment requires further study.

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

Case Reports in Rheumatology ◽

10.1155/2018/4057024 ◽

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.

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IgG4-related intracranial disease

The Neuroradiology Journal ◽

10.1177/1971400918806323 ◽

2018 ◽

Vol 32 (1) ◽

pp. 29-35 ◽

Cited By ~ 6

Author(s):

Shahine Goulam-Houssein ◽

Jeffrey L Grenville ◽

Katerina Mastrocostas ◽

David G Munoz ◽

Amy Lin ◽

...

Keyword(s):

Plasma Cells ◽

Surgical Intervention ◽

Case Series ◽

Hypertrophic Pachymeningitis ◽

Diagnostic Challenge ◽

Perineural Spread ◽

Related Disease ◽

The Third ◽

Igg4 Related Disease ◽

Intracranial Involvement

IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature. Our second case depicts another presentation of extensive IgG4 pachymeningitis involving both cavernous sinuses and surrounding Meckel’s caves. The third case describes a patient with presumed lymphocytic hypophysitis, which was later determined to be IgG4-related hypophysitis with concomitant pachymeningitis and perineural spread along the optic nerves. The delayed diagnoses in our cases illustrates the diagnostic challenge that clinicians face in differentiating intracranial IgG4-RD from other infiltrative diseases such as sarcoidosis, granulomatous disease, tuberculosis and lymphoma. Earlier consideration of IgG4-related hypophysitis and hypertrophic pachymeningitis in the differential diagnosis can prevent significant morbidity including unnecessary surgical intervention and organ failure secondary to extensive fibrosis.

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Needle biopsy compared with surgical biopsy: pitfalls of small biopsy in histologial diagnosis of IgG4-related disease

Arthritis Research & Therapy ◽

10.1186/s13075-021-02432-y ◽

2021 ◽

Vol 23 (1) ◽

Author(s):

Yanying Liu ◽

Fei Yang ◽

Xiying Chi ◽

Yuxin Zhang ◽

Jiangnan Fu ◽

...

Keyword(s):

Needle Biopsy ◽

Plasma Cells ◽

Classification Criteria ◽

Histological Diagnosis ◽

Obliterative Phlebitis ◽

Surgical Biopsy ◽

Related Disease ◽

Igg4 Related Disease ◽

Significant Difference ◽

Storiform Fibrosis

Abstract Objective The growing utilization of needle biopsy has challenged the current pathology consensus of IgG4-related disease (IgG4-RD). The aims of this study were to identify the histological characteristics of needle biopsy and surgical specimens and evaluate the ability of needle biopsy in histological diagnosis of IgG4-RD. Methods Biopsies from patients who were referred to as IgG4-RD by the 2019 ACR/EULAR IgG4-RD classification criteria in Peking University People’s Hospital from 2012 to 2019 were re-evaluated. Typical histological features and diagnostic categories were compared between needle biopsy and surgical biopsy. Results In total, 69 patients met the 2019 ACR/EULAR classification criteria and 72 biopsies of them were re-evaluated. All cases showed lymphoplasmacytic infiltrate, while storiform fibrosis and obliterative phlebitis were only present in 35 (48.6%) and 23 (31.9%) specimens, respectively. Storiform fibrosis was more likely to be seen in retroperitoneum lesion (P = 0.033). Surgical biopsy showed significantly higher IgG4+ plasma cells/high-power field (IgG4/HPF) count (P < 0.01) and higher proportion of IgG4/HPF > 10 (P < 0.01). No significant difference was observed with regard to the ratio of IgG4+ plasma cells/IgG+ plasma cells (IgG4/IgG) (P = 0.399), storiform fibrosis (P = 0.739), and obliterative phletibis (P = 0.153). According to the 2011 comprehensive diagnostic criteria, patients who performed a needle biopsy were less likely to be probable IgG4-RD (P = 0.045). Based on the 2011 pathology consensus, needle biopsy was less likely to be diagnosed as IgG4-RD (P < 0.01), especially to be highly suggestive IgG4-RD (P < 0.01). Only 1/18 (5.6%) needle salivary specimens fulfilled the cutoff of IgG4/HPF > 100, which was significantly less than 15/23 (65.2%) of surgical ones (P < 0.01). Conclusions Needle biopsy shows an inferiority in detecting IgG4/HPF count but not in IgG4/IgG ratio, storiform fibrosis, and obliterative phlebitis. Compared with surgical samples, needle biopsy is less likely to obtain a histological diagnosis of IgG4-RD. A different IgG4/HPF threshold for needle biopsy of the salivary glands may be considered.

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Ossifying fibrous epulis as an IgG4-related disease of the oral cavity: a case report and literature review

BMC Oral Health ◽

10.1186/s12903-022-02041-4 ◽

2022 ◽

Vol 22 (1) ◽

Author(s):

Yoshiko Ike ◽

Takahiro Shimizu ◽

Masaru Ogawa ◽

Takahiro Yamaguchi ◽

Keisuke Suzuki ◽

...

Keyword(s):

Oral Cavity ◽

Lymph Nodes ◽

Plasma Cells ◽

Maxillofacial Surgery ◽

The Body ◽

Pathological Diagnosis ◽

Cervical Lymph Nodes ◽

Related Disease ◽

Igg4 Related Disease ◽

Organ Manifestations

Abstract Background Fibrous sclerosing tumours and hypertrophic lesions in IgG4-related disease (IgG4-RD) are formed in various organs throughout the body, but disease in the oral region is not included among individual organ manifestations. We report a case of ossifying fibrous epulis that developed from the gingiva, as an instance of IgG4-RD. Case presentation A 60-year-old Japanese man visited the Department of Oral and Maxillofacial Surgery, Gunma University Hospital, with a chief complaint of swelling of the left mandibular gingiva. A 65 mm × 45 mm pedunculated tumour was observed. The bilateral submandibular lymph nodes were enlarged. The intraoperative pathological diagnosis of the enlarged cervical lymph nodes was inflammation. Based on this diagnosis, surgical excision was limited to the intraoral tumour, which was subsequently pathologically diagnosed as ossifying fibrous epulis. Histopathologically, the ossifying fibrous epulis exhibited increased levels of fibroblasts and collagen fibres, as well as infiltration by numerous plasma cells. The IgG4/IgG cell ratio was > 40%. Serologic analysis revealed hyper-IgG4-emia (> 135 mg/dL). The patient met the comprehensive clinical diagnosis criteria and the American College of Rheumatology and European League Against Rheumatism classification criteria for IgG4-RD. Based on these criteria, we diagnosed the ossifying fibrous epulis in our patient as an IgG4-related disease. A pathological diagnosis of IgG4-related lymphadenopathy was established for the cervical lymph nodes. Concomitant clinical findings were consistent with type II IgG4-related lymphadenopathy. Conclusions A routine serological test may be needed in cases with marked fibrous changes (such as epulis) in the oral cavity and plasma cells, accompanied by tumour formation, to determine the possibility of individual-organ manifestations of IgG4-related disease.

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Clinical and immunological variants of ANCA-associated systemic vasculitis: a look at the phenomenon of IgG4 overproduction. Literature review and own observations

Rheumatology Science and Practice ◽

10.47360/1995-4484-2020-550-559 ◽

2020 ◽

Vol 58 (5) ◽

pp. 550-559

Author(s):

T. V. Beketova ◽

N. V. Kokosadze

Keyword(s):

Clinical Case ◽

Plasma Cells ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Definite Diagnosis ◽

Related Disease ◽

Epitope Specificity ◽

Igg4 Related Disease ◽

Choice Of Treatment ◽

Combined Data

Systemic vasculitis (SV) associated with anti-neutrophilic cytoplasmic antibodies is a multifactorial process characterized by the variability of the epitope specificity of anti-neutrophilic cytoplasmic antibodies and the diversity of clinical phenotypes of the disease. In recent years, there has been an increasing interest in the combination of AAV and the phenomenon of IgG4 hyperproduction, which varies widely within AAV, from anti-neutrophilic cytoplasmic antibodies products of IgG4 subclass of undetermined significance, the presence of IgG4 positive plasma cells in the foci of immunoinflammatory lesions in patients with a definite diagnosis of AAV, to the typical clinical manifestations of an IgG4-related disease (IgG4-RD). We introduce own clinical case and analyze the combined data of the literature, which included 35 cases of a combination of AAV and IgG4-related pathology, indicating existing differences in the clinical manifestations of classical IgG4-RD and when combined with AAV. The currently accumulated data allows us to discuss the allocation of a specific clinical and immunological variant of AAV with IgG4 hyperproduction, characterized by a combination of clinical manifestations of AAV and clinical and / or histological signs of IgG4-related pathology. It is important to emphasize that the allocation of phenotypes of AAV in the future may be important for the personalized choice of treatment tactics for patients.

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