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 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.

scholarly journals Unique Ultrasonographic Findings of Isolated IgG4-Related Lymphadenopathy

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2213
Author(s):  
Jae Sung Yun ◽  
Seoyun Choi ◽  
Kyu Yun Jang ◽  
Eun Hae Park
Keyword(s):  
Lymph Node ◽  
Lower Extremity ◽  
Imaging Finding ◽  
Elevated Serum ◽  
The Body ◽  
Histological Structure ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
The Right

IgG4-related disease is a rare immune-mediated disease that can involve many organs in the body. The lymph node is also where IgG4-related diseases occur, but its histological structure is different from that of other organs. For this reason, pathologists have difficulty diagnosing IgG4-related lymphadenopathy. If there were specific imaging findings of IgG4-related lymphadenopathy, it would be of great help to pathologists. A 64-year-old male visited our hospital with right ankle pain. On physical examination, the right lower extremity showed severe swelling with wound dehiscence, and infection was suspected. On CT (128-MDCT, Somatom Definition Flash, Siemens Healthcare) taken at the lower extremity, multiple enlarged lymph nodes were incidentally noted in the right inguinal area. On ultrasonography, a “starry night sign” resembling hyperechoic follicles was observed in the enlarged lymph node. A core needle biopsy was performed, and IgG4-related lymphadenopathy was diagnosed. Laboratory examination showed hypergammaglobulinemia with marked elevated serum IgG4, corresponding to IgG4-related disease. Chest and abdominal imaging were evaluated, but there was no extranodal IgG4-related disease. IgG4-related lymphadenopathy showed a very unique ultrasonography imaging finding. The cortex was filled with diffusely scattered hyperechoic foci and some bright foci gathered to form a follicle. This imaging finding may help diagnose IgG4-related lymphadenopathy.

scholarly journals Evaluate the Differences in CT Features and Serum IgG4 Levels between Lymphoma and Immunoglobulin G4-Related Disease of the Orbit

2020 ◽  
Vol 9 (8) ◽  
pp. 2425
Author(s):  
Wei-Hsin Yuan ◽  
Anna Fen-Yau Li ◽  
Shu-Yi Yu ◽  
Ying-Yuan Chen ◽  
Chia-Hung Wu ◽  
...  
Keyword(s):  
Elevated Serum ◽  
Hounsfield Unit ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Serum Igg4 ◽  
Orbital Lymphoma ◽  
Serum Igg Levels ◽  
Igg Level ◽  
Ct Features ◽  
Higher Sensitivity

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.

scholarly journals Salivary Gland Pathology in IgG4-Related Disease: A Comprehensive Review

2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Ilaria Puxeddu ◽  
Riccardo Capecchi ◽  
Filippo Carta ◽  
Antonio Gaetano Tavoni ◽  
Paola Migliorini ◽  
...  
Keyword(s):  
Salivary Glands ◽  
Imaging Techniques ◽  
Elevated Serum ◽  
Clinical Aspects ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Organ Specific ◽  
Clinical Pictures

IgG4-related disease (IgG4-RD) is a rare fibroinflammatory condition that can affect almost any organ, characterized by swollen lesions and often by eosinophilia and elevated serum IgG4 concentrations. The diagnosis of IgG4-RD is a challenging task: in fact, single or multiple organs can be affected and clinical, serological, and histological findings can be heterogeneous. In IgG4-RD, the involvement of salivary glands is observed in 27% to 53% of patients. Several organ-specific conditions, now recognized as different manifestations of IgG4-related sialadenitis (IgG4-RS), were viewed in the past as individual disease entities. The study of salivary glands may sometimes be complex, because of the number of pathological conditions that may affect them, often with overlapping clinical pictures. Integration of different imaging techniques is often required in the case of swelling of salivary glands, even though biopsy remains the gold standard for a definite diagnosis of IgG4-RS. Thus, in this review, we discuss new insights in the pathogenesis of IgG4-RD, focusing on its clinical aspects and the tools that are currently available for a correct differential diagnosis when the salivary glands are involved.

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 Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

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 Soluble Interleukin-2 Receptor: A Potential Marker for Monitoring Disease Activity in IgG4-Related Disease

2018 ◽  
Vol 2018 ◽  
pp. 1-6 ◽  
Author(s):  
A. F. Karim ◽  
L. E. M. Eurelings ◽  
R. D. Bansie ◽  
P. M. van Hagen ◽  
J. A. M. van Laar ◽  
...  
Keyword(s):  
Disease Activity ◽  
Interleukin 2 ◽  
Elevated Serum ◽  
Related Disease ◽  
Interleukin 2 Receptor ◽  
Igg4 Related Disease ◽  
Potential Marker ◽  
Potential Biomarker ◽  
Serum Igg4 ◽  
Soluble Interleukin 2 Receptor

Background. IgG4-related disease (IgG4-RD) is a fibroinflammatory condition. T-cells play a crucial role in the pathogenesis, and therefore, serum soluble interleukin-2 receptor (sIL-2R) may be a potential biomarker. Method. We studied the levels of sIL-2R in 26 histologically proven IgG4-RD patients with available serum sIL-2R and compared them to those in newly diagnosed and untreated sarcoidosis patients (n=78) and controls (n=101) and the serum sIL-2R levels in patients after treatment of IgG4-RD (n=15). The disease activity was measured using the IgG4-Related Disease Responder Index (IgG4-RD RI). Results. Median serum sIL-2R in IgG4-RD patients was 4667 pg/ml compared to 1515 pg/ml in controls (P<0.001) and 6050 pg/ml in sarcoidosis patients (P=0.004 compared to IgG4-RD). All IgG4-RD patients had elevated serum sIL-2R levels compared to the reference value of <2500 pg/ml in controls and 85% elevated serum IgG4; however, these did not correlate with each other. Both serum sIL-2R and IgG4 levels declined significantly after treatment (P=0.001 and P=0.01, resp.). Before treatment, serum sIL-2R level and IgG4-RD RI did not correlate with each other. However, the decrease in serum sIL-2R upon treatment did correlate significantly (P=0.04) with the decrease in disease activity assessed by IgG-RD RI. Conclusion. Serum sIL-2R is elevated in IgG4-RD reflecting the inflammatory process with enhanced T-cell activation. Furthermore, serum sIL-2R might serve as a potential marker of response to treatment in IgG4-RD.

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. 

Sign in / Sign up

Export Citation Format

Share Document