scholarly journals AB1020 APPLICATION OF THE 2019 ACR/EULAR CLASSIFICATION CRITERIA CONTRIBUTES TO THE DIAGNOSIS OF IgG4-RELATED DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1802.2-1803
Author(s):  
L. F. Chen ◽  
J. J. Liang ◽  
Z. M. Ouyang ◽  
J. D. MA ◽  
D. H. Zheng ◽  
...  
Keyword(s):  
Salivary Glands ◽  
General Surgery ◽  
Elevated Serum ◽  
Classification Criteria ◽  
New Classification ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Median Serum ◽  
Serum Igg4

Background:IgG4-related disease (IgG4-RD) is a rare and systemic disease characterized by swelling or masses in the involved organs, elevated serum IgG4, marked IgG4+ plasma cells infiltration and fibrosis. However, lacking of pathological data limited the definite diagnosis of IgG4-RD. As results of high-quality clinical and epidemiological investigations collected, new classification criteria for IgG4-RD were approved by ACR and EULAR in 2019. Whether this new criteria improve the diagnosis efficiency needs to be validated in clinical practice.Objectives:To applicate the 2019 ACR/EULAR classification criteria for the diagnosis of IgG4-RD in previously suspected patients and explore the clinical characteristics of patients with IgG4-RD according to the new classification criteria.Methods:Patients suspected of having IgG4-RD due to elevated serum IgG4 and swelling or masses in single or multiple organs were recruited in Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University from May 2013 and November 2019. Demographic and clinical data were collected. The diagnosis was reevaluated with the 2011 comprehensive diagnostic criteria (CDC) for IgG4-RD and the 2019 ACR/EULAR classification criteria for IgG4-RD, respectively.Results:(1) There were 68 patients recruited and 59(86.8%) of them had elevated serum IgG4 (≥135mg/dl) and 53(77.9%) patients showed swelling or masses in single or multiple organs. Most patients first visit general surgery (17.6%), gastroenterology (16.2%), respiratory medicine (16.2%) and rheumatology (14.7%).(2) According to the 2011 CDC for IgG4-RD, 4(5.9%) patients were definite IgG4-RD, 1(1.5%) was probable and 42(61.8%) were possible. According to the 2019 ACR/EULAR criteria, 20(29.4%) patients were diagnosed as IgG4-RD, including the 4 definite patients using the 2011 CDC.(3) Among the 20 IgG4-RD patients according to the 2019 ACR/EULAR criteria, 19(95.0%) were male and median age of symptom onset was 62(46~69) years. There were 6(30.0%) patients diagnosed at rheumatology, 5(25.0%) at gastroenterology, 3(15.0%) at general surgery, 2(10.0%) at respiratory medicine and 1(5.0%) at stomatology, endocrinology, orthopedics and urinary surgery, respectively. There were 9(45.0%) patients with bilateral lacrimal or salivary glands involved, 9(45.0%) with pancreas and biliary tree involved, 5(25.0%) with retroperitoneum involved, 2(10.0%) with kidney involved and 1(5.0%) with chest involved.(4) The median serum IgG4 of the 20 IgG4-RD patients was 15.40(4.14~55.10)g/L, median serum IgG was 27.9(17.2~50.2)g/L. There were 20.0%(4/20) patients had elevated serum eosinophil and 93.3%(14/15) had elevated serum IgE. There were 60.0%(9/15) patients had elevated C-reactive protein, 85.7%(12/14) had elevated erythrocyte sedimentation rate and 26.7%(4/15) had hypocomplementemia. There were 35.7%(5/14) patients had positive rheumatoid factor and 14.3%(2/14) had positive antinuclear antibodies. There were 6(30.0%) patients didn’t received biopsies, 8(40.0%) patients received surgical removal of salivary glands, lacrimal glands or pancreatic masses and 6(30.0%) patients received needle biopsies of the salivary glands or biopsies by bronchoscopy, gastroscopy or enteroscopy.(5) Among the patients didn’t fulfill the 2011 CDC and the 2019 ACR/EULAR criteria, there were 40 patients had elevated serum IgG4, whose serum IgG4 level were significant lower than those of IgG4-RD patients [3.66(2.39~7.68)g/L vs. 15.40(4.14~55.10)g/L,P<0.001], and percentage of serum IgG4 ≥5× upper limit of normal were also lower than those of IgG4-RD patients (27.5% vs. 73.7%,P=0.002).Conclusion:The 2019 ACR/EULAR criteria can help diagnosing patients with IgG4-RD even lacking the tissue confirmation. Patients with moderately elevated serum IgG4 need more clinical evidence to diagnose IgG4-RD and exclude mimics.References:This work was supported by Guangdong Medical Scientific Research Foundation (grant no. A2017093).Disclosure of Interests:None declared

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 FRI0503 VALIDATION OF THE 2019 ACR/EULAR CLASSIFICATION CRITERIA FOR IGG4-RELATED DISEASE IN A JAPANESE KIDNEY DISEASE COHORT: A MULTI-CENTER RETROSPECTIVE STUDY BY THE IGG4-RELATED KIDNEY DISEASE (IGG4-RKD) WORKING GROUP OF THE JAPANESE SOCIETY OF NEPHROLOGY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 849.1-850
Author(s):  
T. Saeki ◽  
M. Kawano ◽  
T. Nagasawa ◽  
Y. Ubara ◽  
Y. Taniguchi ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Kidney Biopsy ◽  
Elevated Serum ◽  
Classification Criteria ◽  
Inclusion Criteria ◽  
Exclusion Criteria ◽  
Drug Induced ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

Background:The 2019 ACR/EULAR classification criteria for IgG4-RD have recently been published1). In the criteria, patients with an inclusion criteria score of >20 without exclusion criteria are classified as having IgG4-RD.Objectives:To validate the 2019 ACR/EULAR classification criteria for IgG4-RD in a Japanese kidney disease cohort.Methods:The study involved Japanese patients diagnosed as having kidney disease between April 2012 and May 2019, for whom sufficient clinical information and data on serum IgG4 values and/or immunohistological staining for IgG4 in renal biopsy samples were known. These patients were classified as having IgG4-RKD or non-IgG4-RKD (mimickers) based on the 2019 ACR/EULAR classification criteria for IgG4-RD, and the results were evaluated by expert opinion.Results:Among 105 included patients, the expert panel diagnosed 55 as having true IgG4-RKD and 50 as mimickers. The final diagnoses among the mimickers were vasculitis (n=11), idiopathic tubulointerstitial nephritis (TIN) (n=5), drug-induced TIN (n=5), Sjögren’s syndrome (n=4) and others. Among the 55 true IgG4-RKD patients, 4 had exclusion criteria, and 50 of the remaining 51 had an inclusion criteria score of ≥20 points (sensitivity 90.9%). On the other hand, 49 of the 50 mimickers were classified as having non-IgG4-RKD (specificity 98.0%) (Table 1).Table 1.General characteristics and prevalence of individual items of true IgG4-RKD and non-IgG4-RKD (mimicker)IgG4-RKD(true IgG4-RKD)(n=55)Non-IgG4-RKD(mimicker)(n=50)PAge at diagnosis,mean ±SD (years)69.9 ± 9.456.7 ± 17.4<0.001Male (%)76.4440.001Elevated serum IgG454/55 (98.2%)18/50 (36.0%)<0.001Serum IgG4 (mg/dl), mean±SD1028 ± 796226 ± 261<0.001Dense IgG4+Plasma cells (>10/hpf) in the kidney biopsy48/51 (94.1%)13/40 (32.5%)<0.001Storiform fibrosis in the kidney biopsy28/51 (54.9%)3/50 (6%)<0.001Hypocomplementemia39/55 (70.1%)7/42 (16.7%)<0.001Renal pelvis thickening/soft tissue5/55 (9%)1/50 (2%)0.20Bilateral renal cortex low-density areas29/55 (52.7%)7/50 (14.0%)<0.001Exclusion criteria present4/55 (7.3%)22/50 (44%)<0.001Total inclusion criteria points >20 without exclusion criteria50/55 (90.9%)1/50 (2%)<0.001Conclusion:The 2019 ACR/EULAR classification criteria for IgG4-RD showed good agreement with expert classification in this Japanese kidney disease cohort.References:[1] Wallace ZS, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 79:77-87, 2020Disclosure of Interests:None declared

scholarly journals SAT0532 POSITIVE DISEASE-SPECIFIC AUTOANTIBODIES LOWER DIAGNOSTIC SENSITIVITY BUT HAVE LITTLE CLINICAL SIGNIFICANCE IN DIAGNOSING IgG4-RELATED DISEASE USING THE 2019 ACR/EULAR CLASSIFICATION CRITERIA IN DAILY CLINICAL PRACTICE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1224.1-1224
Author(s):  
I. Mizushima ◽  
T. Yamano ◽  
H. Kawahara ◽  
S. Hibino ◽  
R. Nishioka ◽  
...  
Keyword(s):  
Clinical Practice ◽  
False Negative ◽  
Classification Criteria ◽  
Daily Clinical Practice ◽  
Exclusion Criterion ◽  
Inclusion Criteria ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Disease Specific

Background:Recently, the 2019 ACR/EULAR classification criteria for IgG4-related disease (IgG4-RD) were published mainly to identify more homogeneous subjects for inclusion in clinical trials and observational studies [1]. However, although their high specificity is presumed to be useful to differentiate IgG4-RD from various mimickers, their value in daily clinical practice needs to be evaluated.Objectives:This study aimed to clarify the usefulness of the 2019 ACR/EULAR classification criteria for IgG4-RD and characteristics of false-negative patients in daily clinical practice.Methods:We retrospectively reviewed the medical records of 162 patients with IgG4-RD and 130 consecutive non-IgG4-RD patients (mimickers) diagnosed by experts whose serum IgG4 levels were measured at a single center in Japan. Using the collected data, we calculated sensitivity, specificity, and fulfillment rates for the entry criteria, exclusion criteria, and threshold of inclusion criteria points. In addition, to clarify the characteristics of false-negative cases in IgG4-RD, we performed an intergroup comparison of their clinical features including disease-specific autoantibodies.Results:Both the patients with IgG4-RD and mimickers were relatively old (66 and 65 years) with male predominance (67% and 60%). The final diagnoses of mimickers mainly consisted of cancer, lymphoma, vasculitis, sarcoidosis, multicentric Castleman’s disease, and atherosclerotic or infectious aortic aneurysm. The classification criteria had a sensitivity of 72.8% and a specificity of 100%. Of the 44 false-negative cases, one did not fulfill the entry criteria, 20 fulfilled one exclusion criterion, and 27 did not achieve sufficient inclusion criteria points. Compared with the true-positive cases, the false-negative cases had significantly fewer affected organs, lower serum IgG4 levels, higher serum CH50 levels, and lower prevalence of salivary/lacrimal gland and renal parenchymal lesions. They were also less likely to have had biopsies (61% vs 97%). Of note, positivity of disease-specific autoantibodies including SSA/Ro antibody, ANCA, ds-DNA antibody, and ACPA was the most common exclusion criterion fulfilled in 18 patients, only 2 of whom were diagnosed with a specific autoimmune disease (rheumatoid arthritis) complicated by IgG4-RD. The remaining 16 patients had no specific clinical symptoms related to such autoantibodies. In addition, compared with IgG4-RD patients without disease-specific autoantibodies, the 18 patients with them had almost equal serum IgG4 and complement levels, number of affected organs, and histopathology and immunostaining scores despite higher serum IgG and CRP levels.Conclusion:The present study suggests that the 2019 ACR/EULAR classification criteria for IgG4-RD has excellent diagnostic specificity and moderate sensitivity in daily clinical practice. Positive disease-specific autoantibodies alone, which lowered the sensitivity in this study, may have little clinical significance concerning the diagnosis of IgG4-RD.References:[1]Wallace ZS et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020 Jan;79(1):77-87.Disclosure of Interests:None declared

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 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 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. 

Clinical case of membranous nephropathy in a patient with IgG4-related disease

2020 ◽  
Vol 24 (4) ◽  
pp. 110-121
Author(s):  
M. O. Pyatchenkov ◽  
O. A. Vorobyeva ◽  
A. N. Belskykh ◽  
M. V. Zakharov ◽  
M. Y. Dendrikova
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
The Body ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pathologic Features ◽  
Wide Range ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) currently is considered as a chronic fibroinflammatory immune-mediated multisystemic condition of unidentified etiology, which can imitate a wide range of malignant, infectious, rheumatologic, and other diseases. It can affect almost any organ system in the body synchronously or sequentially, but the most often affected are the pancreas, hepatobiliary tract, periorbital structures, salivary glands, kidneys, and lymph nodes. The most frequent renal manifestations of IgG4-RD is IgG4-related tubulointerstitial nephritis. Membranous nephropathy is the most common glomerular disease accompanied by IgG4-RD. Regardless of the organ localization, patients with IgG4-RD are characterized by elevated serum IgG4, but this laboratory abnormality is not specific and can be changed in other diseases. In all suspected cases of IgG4-RD the diagnosis should be confirmed by histological examination. Characteristic pathologic features include diffuse or focal lymphoplasmacytic infiltration with prominent IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. Patients with IgG4- RD usually have an excellent clinical response to glucocorticoids, but relapse rates after steroid withdrawal are high, which may require additional use of immunosuppressants or rituximab. Due to the low prevalence and multitude of clinical manifestations the disease often remains underdiagnosed on time. This case report describes middle-aged patients with a history of chronic recurrent pancreatitis complicated by the nephrotic syndrome. Kidney biopsy showed membranous nephropathy and diagnosis IgG4-RD with multiorgan involvement was made. Partial remission was achieved on corticosteroid therapy. The presented case clearly demonstrates the difficulties of diagnosis and treatment of IgG4-RD. IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with nephrotic syndrome accompanied by multiorgan dysfunction.

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