scholarly journals “Stomach mass” as the first manifestation of IgG4-related disease: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Fulong Zhang ◽  
Jing Xu ◽  
Yuandong Zhu ◽  
Qianneng Wu ◽  
Xincheng Xie ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Financial Burden ◽  
Normal Range ◽  
Initial Manifestation ◽  
Case Presentation ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Head Of The Pancreas ◽  
Igg4 Level

Abstract Background IgG4-related disease mainly manifests as organomegaly and is accompanied by tissue fibrosis (Mimori, Mod Rheumatol 29(2):213, 2019) which is frequently confused with tumour (Dawei et al., J Gastroenterol Hepatol 29(12):1375–8, 2020). There are few reports with of IgG4-related disease with the first clinical manifestation involving the stomach. Case presentation We present the case of 46-year-old male patient with a “stomach tumour” as the first manifestation of IgG4-related disease. Gastroscopy showed a mass in the stomach, however, the pathology result was chronic inflammation with IgG4 positivity. CT scans of abdomen showed that the stomach wall was thick, the head of the pancreas was swollen, and retroperitoneal fibrosis was severe.The serum IgG4 level was 75 g/L (normal range 0.03–2.01 g/L).After treatment with methylprednisolone for one month, the symptoms were greatly relieved. Conclusions To reduce the suffering of patients and relieve their financial burden, we should consider the possibility of IgG4-related disease when the initial manifestation is a stomach mass.

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 A Case of IgG4-Related Bilateral Palpebral Conjunctivitis

2019 ◽  
Vol 10 (2) ◽  
pp. 299-303 ◽  
Author(s):  
Toshiya Nagai ◽  
Tatsuya Yunoki ◽  
Atsushi Hayashi
Keyword(s):  
Rare Case ◽  
Plasma Cells ◽  
Lower Eyelid ◽  
Infraorbital Nerve ◽  
Normal Range ◽  
High Power Field ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

The most common sites of IgG4-related ocular disease are the lacrimal glands, infraorbital nerve, and extraocular muscles. Other ocular adnexal sites are relatively rare. We report a rare case of an 83-year-old man who developed palpebral conjunctivitis following bilateral hypertrophic ectropion of the eyelid. Tissue immunostaining revealed many IgG4-positive plasma cells (67 IgG4/74 IgG cells/high-power field). The serum IgG4 level was 76.9 mg/dL, which was within the normal range. The diagnosis was probable IgG4-related disease. The possibility of IgG4-related disease should be considered in a patient presenting with refractory conjunctivitis and hyperemia or hypertrophy of the lower eyelid.

scholarly journals Usefulness of measuring serum IgG4 level as diagnostic and treatment marker in IgG4-related disease

2012 ◽  
Vol 35 (1) ◽  
pp. 30-37 ◽  
Author(s):  
Motohisa YAMAMOTO ◽  
Norihiro NISHIMOTO ◽  
Tetsuya TABEYA ◽  
Yasuyoshi NAISHIRO ◽  
Keisuke ISHIGAMI ◽  
...  
Keyword(s):  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

scholarly journals Relapse predictors and serologically unstable condition of IgG4-related disease: a large Chinese cohort

Rheumatology ◽  
2020 ◽  
Vol 59 (8) ◽  
pp. 2115-2123 ◽  
Author(s):  
Yanying Liu ◽  
Qiaozhu Zeng ◽  
Lijuan Zhu ◽  
Jingyuan Gao ◽  
Ziqiao Wang ◽  
...  
Keyword(s):  
Risk Factors ◽  
Relapse Rate ◽  
Unstable Condition ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Younger Age ◽  
Serum Igg4 ◽  
History Of ◽  
Igg4 Level

Abstract Objectives Patients with IgG4-related disease (IgG4-RD) typically respond well to initial glucocorticoid therapy, but always relapse with tapered or maintenance dosage of steroid. We aimed to identify the risk factors for relapse of IgG4-RD and explore the impact of active intervention on the serologically unstable condition. Methods We performed a retrospective study of 277 IgG4-RD patients at Peking University People’s Hospital from February 2012 through February 2019. They were all followed for >4 months. The primary outcome was patient relapse. Data on recurrence of IgG4-RD symptoms, laboratory and image findings were recorded, along with information on treatment in the serologically unstable condition. Results The cumulative relapse rate was 12.86%, 27.84% and 36.1% at 12, 24 and 36 months, respectively. Younger age at onset, younger age at diagnosis, longer time from diagnosis to treatment and history of allergy were associated with relapse. Identified independent risk factors were longer time from diagnosis to treatment and history of allergy. When serum IgG4 level was 20%, 50% or 100% higher than that of the remission period, similar percentages of patients finally relapsed, regardless of whether they were in the immunosuppression intensified or non-intensified group. Median duration from serum IgG4 level instability to relapse in the intensified and non-intensified group was not statistically different. Conclusion The risk factors of relapse were longer time from diagnosis to treatment and history of allergy. Intervention in the serologically unstable condition was not helpful for reducing relapse rate.

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.

A Case of IgG4-Related Retroperitoneal Fibrosis from the Renal Pelvis Mimicking Bilateral Hydronephrosis

2014 ◽  
Vol 97 (1) ◽  
pp. 118-122 ◽  
Author(s):  
Seiya Inoue ◽  
Chihiro Takahashi ◽  
Katsuya Hikita
Keyword(s):  
Computed Tomography ◽  
Malignant Disease ◽  
Ureteropelvic Junction Obstruction ◽  
Retroperitoneal Fibrosis ◽  
Retrograde Pyelography ◽  
Bilateral Hydronephrosis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Show Evidence

We describe a 49-year-old woman who presented with continuous bilateral lumbago. As the patient's ultrasonography manifestations were very similar to those of bilateral hydronephrosis, we performed retrograde pyelography and ureteroscopy. However, apart from slight left ureteropelvic junction obstruction, there was no hydronephrosis. Since malignant disease could not be completely denied, computed tomography-guided biopsy was performed. However, the tissue did not show evidence of malignancy. As the patient continued to have lumbago, we measured serum IgG4 levels because of suspicion of retroperitoneal fibrosis secondary to IgG4-related disease, which proved to be high. Further, immunostaining of the renal pelvic biopsy samples showed IgG4-positive cells. Therefore, diagnosing IgG4-related retroperitoneal fibrosis, we administered corticosteroids. The patient responded favorably to the drug, with gradual regression of the lesion.

Salivary gland involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 428 patients

Rheumatology ◽  
2019 ◽  
Author(s):  
Yanying Liu ◽  
Miao Xue ◽  
Zhenfan Wang ◽  
Qiaozhu Zeng ◽  
Limin Ren ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Disease Onset ◽  
Allergic Diseases ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Younger Age ◽  
Serum Igg4 ◽  
Peking University ◽  
Igg4 Level

Abstract Objectives IgG4-related disease (IgG4-RD) has recently been recognized as a fibro-inflammatory condition featuring tumefactive lesions in multiple organs, and the salivary gland is one of the most commonly involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with salivary gland lesions (IgG4-RD SG+) and salivary-gland-free IgG4-RD (IgG4-RD SG−) in a large cohort. Methods We carried out a retrospective review of the medical records of 428 cases of IgG4-RD diagnosed at Peking University People’s Hospital between March 2006 and May 2018. Results Among 428 patients, 249 had salivary glands that were affected. IgG4-RD SG+ patients showed younger age at disease onset and diagnosis, and a longer interval between symptom onset and diagnosis. The IgG4-RD SG+ group involved more female patients, and allergic diseases were more common in this group. In terms of organ involvement, the IgG4-RD SG+ group were more frequently presented with lacrimal gland involvement, while lymph node, retroperitoneal fibrosis, pancreas, biliary system, kidney and aorta were more prominent in the IgG4-RD SG− group. In addition, the serum IgG4 level, IgG4/IgG ratio and IgE level were significantly higher in IgG4-RD SG+ patients. Patients with eosinophilia were more common in the IgG4-RD SG+ group, while elevated ESR, CRP and positive ANA were more common in the IgG4-RD SG− group. Conclusion We have revealed demographic, clinical and laboratory differences between IgG4-RD SG+ and SG− patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

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

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