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 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

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

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.

Suspected IgG4-related disease of the submandibular salivary gland and periorbital soft tissue in a man with latent tuberculosis

2021 ◽  
Vol 14 (8) ◽  
pp. e242415
Author(s):  
Aimée McGreal-Bellone ◽  
Susan Lapthorne ◽  
Corinna Sadlier ◽  
Geraldine Moloney
Keyword(s):  
Bone Marrow Aspiration ◽  
Unknown Origin ◽  
Submandibular Salivary Gland ◽  
Cross Sectional ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Upper Limit ◽  
Serum Igg4 ◽  
Igg4 Level

A 77-year-old Lithuanian man presented to our institution with recurrent episodes of periorbital cellulitis, submandibular swelling and sialadenitis. Investigations revealed a positive QuantiFERON, raised inflammatory markers and normal autoimmune screen. Cross-sectional imaging showed no signs of occult malignancy, and work-up for mycobacterial infection including imaging and bronchoalveolar lavage did not show active tuberculosis. During hospitalisation, the patient developed fevers of unknown origin, which were investigated with a positron emission tomography (PET) scan and a bone marrow aspiration, without evidence of occult infection or malignancy. Serum IgG4 level was three times the upper limit of normal. The patient responded well to oral steroids but relapsed after completing a slow taper. Serum IgG4 level was three times the upper limit of normal. He had an American College of Rheumatology/European League Against Rheumatism score of 20, in conjunction with involvement of orbital and salivary tissue. Therefore, IgG4-related disease was considered the most likely diagnosis, despite prominent fevers, which are among the exclusion criteria for this diagnosis. After a multidisciplinary review including rheumatology and ophthalmology, the patient was commenced on maintenance methotrexate with remission of symptoms.

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 Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Jay H. Ryu ◽  
Ryohei Horie ◽  
Hiroshi Sekiguchi ◽  
Tobias Peikert ◽  
Eunhee S. Yi
Keyword(s):  
Clinical Practice ◽  
Wide Spectrum ◽  
Systemic Vasculitis ◽  
Elevated Serum ◽  
Serum Igg4 Level ◽  
Igg4 Related Disease ◽  
Clinical Presentations ◽  
Serum Igg4 ◽  
Disease Associations ◽  
Igg4 Level

IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8%) had an elevated serum IgG4 level (>140 mg/dL). IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4%) had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

scholarly journals IgG4-Related Disease: A Multispecialty Condition

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Iuri Usêda Santana ◽  
Emanuela Pimenta da Fonseca ◽  
Mittermayer Barreto Santiago
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Diffuse Abdominal Pain ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Serum Igg4 ◽  
Polyclonal Hypergammaglobulinemia ◽  
History Of ◽  
Systemic Condition

IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases.

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