scholarly journals Multi-Organ Involvement of Immunoglobulin G4-Related Disease

2021 ◽  
Vol 12 (3) ◽  
pp. 350-357
Author(s):  
Elleuch Nour ◽  
Aya Hammami ◽  
Nabiha Missaoui ◽  
Ahlem Bdioui ◽  
Wafa Dahmani ◽  
...  
Keyword(s):  
Bile Ducts ◽  
Clinical Manifestations ◽  
Organ Involvement ◽  
Unknown Etiology ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Abdominal Magnetic Resonance Imaging ◽  
Intrahepatic Bile Ducts ◽  
Multiple Organs ◽  
Igg4 Related Disease

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition of unknown etiology, with presumed autoimmune mechanisms. It is characterized by high levels of IgG4 and variable clinical manifestations. It can involve one or multiple organs. Herein, we reported the case of a 62-year-old man with three organs involvement. He initially presented with recurrent jaundice. Laboratory analysis revealed cholestasis, high gamma-globulin levels, renal failure, and proteinuria. Abdominal Magnetic Resonance Imaging (MRI) showed segmental strictures of the left intrahepatic bile ducts and the wirsung duct with an increased volume of the pancreas and diffuse bilateral enlargement of the kidneys. Laboratory tests revealed high IgG4 levels (770 mg/dL). Based on the biological and radiological findings, we have suggested the diagnosis of systemic IgG4-related disease involving bile ducts, the pancreas, and probably the kidneys. Renal biopsy revealed lymphoplasmacytic infiltrate and fibrosis, but no IgG4-positive cell. The patient received corticosteroid therapy with a complete resolution of all symptoms and a rapid normalization of all blood tests. The present case underlines the complexity of IgG4-RD because of its variable clinical presentation. The diagnosis is challenging and should be carefully assessed for possible multi-organ involvement.

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

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.

scholarly journals Different factors underlie recurrent and de novo organ involvement in immunoglobulin G4–related disease

Rheumatology ◽  
2019 ◽  
Author(s):  
Ichiro Mizushima ◽  
Syunsuke Tsuge ◽  
Yuhei Fujisawa ◽  
Satoshi Hara ◽  
Fae Suzuki ◽  
...  
Keyword(s):  
Risk Factors ◽  
Multivariate Analysis ◽  
Cox Regression ◽  
De Novo ◽  
Univariate Analysis ◽  
Organ Involvement ◽  
Blood Eosinophil ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease

Abstract Objectives In IgG4-related disease (IgG4-RD), relapse including recurrent organ involvement (ROI) and de novo organ involvement (DNOI) occurs frequently during the clinical course. This study aimed to clarify the differences between the risk factors underlying ROI and DNOI in IgG4-RD. Methods We retrospectively investigated factors related to ROI and DNOI in 86 IgG4-RD patients. For assessment of factors related to ROI and DNOI, we performed uni- and multivariate Cox regression analyses. On stepwise multivariate analysis, we applied the variables with P < 0.1 in the univariate analysis and the predictors of relapse suggested in past reports. Results During the mean follow-up period of 63.1 months, ROI was detected at 1.0–120 months after diagnosis in 20 patients, 4 of whom were not receiving glucocorticoid (GC) at the time of ROI. In contrast, DNOI was detected at 5.0–120 months after diagnosis in 15 patients, 8 of whom were not receiving GC at the time of DNOI. In the multivariate analysis, blood eosinophil counts at diagnosis [per 100/μl; hazard ratio (HR) 1.072 (95% CI 1.018, 1.129)] and continuation of GC [vs discontinuation or observation without GC; HR 0.245 (95% CI 0.076, 0.793)] had a significant impact on the time to DNOI, whereas age [HR 0.942 (95% CI 0.899, 0.986)] and ANA positivity [vs negativity; HR 6.632 (95% CI 1.892, 23.255)] had a significant impact on the time to ROI. Conclusion The present study suggests that the risk factors of ROI and DNOI are different in IgG4-RD, highlighting the need for different preventative strategies.

scholarly journals IgG4-Related Disease Manifesting as Hypocomplementemic Tubulointerstitial Nephritis: A Rare Case Report and Literature Review

2020 ◽  
Vol 8 ◽  
pp. 232470962095221
Author(s):  
Pradnya Brijmohan Bhattad ◽  
David L. Joseph ◽  
Eric Peterson
Keyword(s):  
Kidney Disease ◽  
Vision Loss ◽  
Tubulointerstitial Nephritis ◽  
Organ Damage ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Rare Case Report ◽  
Progression Of Kidney Disease

Immunoglobulin G4–related disease (IgG4-RD) is a chronic fibrosing inflammatory systemic disorder that has been recognized relatively recently in the medical literature. Little is known about the exact disease pathogenesis and epidemiology. IgG4-RD may be asymptomatic or may have minimal symptoms or involve multiple organs with overt symptoms. The different phenotypes of IgG4-RD can lead to delayed or incorrect diagnosis. We report the case of a 66-year-old male with coal worker’s pneumoconiosis who presented with progressive kidney disease and was diagnosed with tubulointerstitial nephritis due to IgG4-RD. The patient was noted to have progressive kidney disease, skin involvement, worsening interstitial lung disease, complete vision loss in the left eye, and retroperitoneal fibrosis. Serologic workup revealed elevated inflammatory markers, IgG4 and IgG1 levels, and hypocomplementemia. A tissue biopsy helped us establish a definitive diagnosis of IgG4-RD and initiate treatment with glucocorticoids to prevent further progression of kidney disease and other end-organ damage.

scholarly journals AB1036 CLINICAL MANIFESTATIONS, CLINICAL COURSE, AND OUTCOMES OF IMMUNOGLOBULIN G4 RELATED DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1809.1-1809
Author(s):  
W. Katchamart ◽  
K. Phaopraphat ◽  
P. Ngamjanyaporn ◽  
P. Narongroeknawin ◽  
N. Kasitanon
Keyword(s):  
Disease Duration ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
Systemic Autoimmune Disease ◽  
Immunoglobulin G4 ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

Background:Immunoglobulin G4 related disease (IgG4-RD) is an uncommon chronic systemic autoimmune disease, pathologically characterized by lymphoplasma cell, IgG4 plasma cell or storiform fibrosis infiltration with elevated serum IgG4 level. IgG4-RD is a new disease and not widely recognized.Objectives:The aim of this study was to describe clinical manifestations and outcomes of IgG4-RD in Thai patientsMethods:This multicenter retrospective cohort study included patients who aged ≥ 18 years and were diagnosed with IgG4-RD according to 2011 comprehensive or consensus diagnostic criteria, between 2000 and 2019 in four academic centers in Thailand. Baseline characteristic, laboratory and pathologic findings, treatments, and outcomes were systematically reviewed.Results:Of the 110 patients included, 71% were male with mean age (SD) of 59.6 (13.3) years and median disease duration (IQR) of 28.8 (14.6-53.5) months. Single organ involvement was observed in 60 patients (54.5%). The most common presenting organ involvement was the orbit (29%), followed by the salivary glands (19%), lacrimal glands (18%), bile duct (16%), and pancreas (11%). The most frequently affected organs were the orbits (34%), followed by the salivary glands (26%), lacrimal glands (20%), bile duct (19%), and lymph nodes (19%). Ninety-six percent (96%) had IgG4 level of more than 135 mg/dl at presentation. Most patients (92%) were treated with corticosteroid (CS) alone or in combination with immunosuppressive agents. Azathioprine (47%) and methotrexate (11%) were the most commonly used immunosuppressive agents. Additionally, 20% required surgery, and 6.4% underwent stent insertion. One-fourth (26%) were in remission with successfully CS tapering, while 37%, and 29% had complete, and partial response. Nevertheless, 22% relapse with median time to relapse (IQR) of 22.2 (12.8-41.1) months. Relapse was common in patients with orbital (p = 0.001) and lung (p= 0.007) involvement, and patients with longer disease duration (median 44.1 and 23.1 months, P=0.001), while serum IgG4 level was insignificantly higher in relapse group (median 1,085 vs. 850 mg/dL, p=0.28).Conclusion:IgG4-RD is a chronic systemic autoimmune disease with diverse manifestations, response to treatment, and outcomes. Most patients responded well to CS and immunosuppressive agents with notable relapse rate, while minority required surgery or mechanical intervention.References:[1]Wallace ZS, Zhang Y, Perugino CA, Naden R, Choi HK, Stone JH. Clinical phenotypes of IgG4-related Disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019;78(3):406-12.[2]Martinez-Valle F, Fernandez-Codina A, Pinal-Fernandez I, Orozco-Galvez O, Vilardell-Tarres M. IgG4- related disease: Evidence from six recent cohorts. Autoimmun Rev. 2017;16(2):168-72Acknowledgments:NoneDisclosure of Interests:None declared

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 Delayed Radiographic Presentation of IgG4 Vasculitis

2019 ◽  
pp. 1-2
Author(s):  
Naureen Osman ◽  
Julie Schwartzman-Morris
Keyword(s):  
Inflammatory Process ◽  
Interesting Case ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Radiographic Imaging ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Radiographic Presentation

Immunoglobulin G4 (IgG4) related disease is a systemic inflammatory process that affects multiple organs and can commonly present with large vessel vasculitis. We present an interesting case of a patient with delayed manifestation of IgG4 related vasculitis on radiographic imaging.

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

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.

scholarly journals Otological IgG4-Related Disease With Inner Ear Involvement: A Case Report and Review of Literature

2020 ◽  
pp. 014556132097641
Author(s):  
Qinzhan Ren ◽  
Jinfei Su ◽  
Daoxing Zhang ◽  
Xiuyong Ding
Keyword(s):  
Inner Ear ◽  
Cochlear Implants ◽  
Chronic Inflammatory Disease ◽  
Review Of Literature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Cochlear Ossification ◽  
Control Disease

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory disease involving multiple organs. Some studies have reported otological manifestations of IgG4-RD, although most studies describe involvement of the middle ear, and reports on inner ear manifestations are limited. Here, we describe a case of a 30-year-old man with IgG4-RD involving the inner ear. This case demonstrated that IgG4-RD affected the inner ear and caused cochlear ossification. Cochlear implants may be considered for milder cases, and hormone and immunosuppressive therapy may control disease progression.

scholarly journals Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations

2016 ◽  
Vol 49 (2) ◽  
pp. 122-125 ◽  
Author(s):  
Daniel Alvarenga Fernandes ◽  
Ricardo Yoshio Zanetti Kido ◽  
Ricardo Hoelz de Oliveira Barros ◽  
Daniel Lahan Martins ◽  
Thiago José Penachim ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Magnetic Resonance ◽  
Autoimmune Disease ◽  
Systemic Autoimmune Disease ◽  
Imaging Findings ◽  
Resonance Imaging ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease

Abstract We present a case of immunoglobulin G4 (IgG4)-related disease with pancreatic and extrapancreatic involvement, including the biliary and renal systems. Given the importance of imaging methods for the diagnosis of IgG4-related disease and its differentiation from pancreatic adenocarcinoma, we emphasize important abdominal computed tomography and magnetic resonance imaging findings related to this recently recognized systemic autoimmune disease.

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