scholarly journals AB0760 HIGH SERUM IGA LEVELS HAVE LIMITED CLINICAL SIGNIFICANCE IN PATIENTS WITH IGG4-RELATED DISEASE DIAGNOSED BY EXPERTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1407.2-1407
Author(s):  
S. Tsuge ◽  
I. Mizushima ◽  
S. Shin ◽  
T. Yoshinobu ◽  
R. Hoshiba ◽  
...  
Keyword(s):  
Clinical Significance ◽  
Clinical Course ◽  
Elevated Serum ◽  
High Serum ◽  
Classification Criteria ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Iga ◽  
Organ Specific ◽  
Serum Crp

Background:While the diagnostic and classification criteria for IgG4-related disease (IgG4-RD) have been recently developed [1-3], it is known that, without appropriate exclusions, some non-IgG4-RDs may meet these criteria. In particular, hyper IL-6 syndromes, including Castleman disease, can be misdiagnosed as IgG4-RD. Some clinical findings, including elevated serum levels of C-reactive protein (CRP) or IgA, have been suggested to be useful for differentiating hyper IL-6 syndromes from IgG4-RD [4]. However, since few clinical studies have focused on IgG4-RD with high serum IgA levels, its clinical significance has not been well known.Objectives:This study aimed to clarify the clinical significance of high serum IgA levels in patients with IgG4-RD.Methods:We retrospectively investigated the clinical features of 170 patients with IgG4-RD on the basis of the presence or absence of elevated serum IgA levels (>410 mg/dL) at the time of diagnosis. The diagnosis of IgG4-RD was made by experts on the basis of the fulfillment of the comprehensive diagnostic criteria and/or each organ-specific diagnostic criteria.Results:Elevated serum IgA levels were observed in 18 patients (10.6%). In the patients with elevated serum IgA levels, serum CRP levels were higher (1.14 ± 1.18 vs. 0.31 ± 0.63 mg/dL, p=0.003) and the prevalence of relapse during the clinical course was lower (5.6% vs. 27.6%, p=0.046) than in those without elevated serum IgA levels. However, there were no significant differences in the other clinical features including the number of involved organs (2.4 ± 1.3 vs. 2.8 ± 1.6, p=0.443) and inclusion scores of the ACR/EULAR classification criteria (32 ± 14 vs. 36 ± 17, p=0.374). To evaluate the influence of serum IgA elevation on relapse, we performed Cox regression analysis, which showed that the elevated serum IgA levels had no significant association with lower incidence of relapse but a tendency of it (hazard ratio 0.997, 95% confidence interval 0.994-1.000, p=0.055) during the clinical course. In addition, a prompt improvement in the IgG4-RD responder index [5] during the clinical course was seen in the patients with serum IgA elevation, suggesting a similar good response to glucocorticoids as in those without it.Conclusion:The findings of the present study suggest that IgG4-RD patients with high serum IgA levels can be diagnosed and treated in the same way as those without it, although they may be characterized by mild serum CRP elevation.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;79:77-87.[2]Umehara H et al. The 2020 Revised Comprehensive Diagnostic (RCD) Criteria for IgG4-RD. Mod Rheumatol. 2020 Dec 4:1-14. doi: 10.1080/14397595.2020.1859710. Online ahead of print.[3]Umehara H et al. Current approach to the diagnosis of IgG4-related disease - Combination of comprehensive diagnostic and organ - specific criteria. Mod Rheumatol. 2017;27:381-91.[4]Sato Y et al. Systemic IgG4-related lymphadenopathy: A clinical and pathologic comparison to multicentric Castleman’s disease. Mod Pathol.2009;22:589-99.[5]Carruthers MN et al. Development of an IgG4-RD Responder Index. Int J Rheumatol. 2012;2012:259408.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 Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Kazuichi Okazaki ◽  
Hisanori Umehara
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Levels ◽  
Mikulicz’S Disease ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Biopsy Specimens ◽  
Organ Specific ◽  
Low Sensitivity

Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.

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

Positive disease-specific autoantibodies have limited clinical significance in diagnosing IgG4-related disease in daily clinical practice

Rheumatology ◽  
2020 ◽  
Author(s):  
Ichiro Mizushima ◽  
Takahiro Yamano ◽  
Hiroyuki Kawahara ◽  
Shinya Hibino ◽  
Ryo Nishioka ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Clinical Significance ◽  
False Negative ◽  
Classification Criteria ◽  
Exclusion Criterion ◽  
True Positive ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Disease Specific

Abstract Objectives The 2019 ACR/EULAR classification criteria for IgG4-related disease (IgG4-RD) have exclusion criteria including positive disease-specific autoantibodies, and these have been documented to have a high specificity. This study aimed to further validate these criteria as well as identify characteristics of patients showing false-negative results. Methods We retrospectively analysed 162 IgG4-RD patients and 130 mimickers. The sensitivity, specificity and fulfilment rates for each criterion were calculated, and intergroup comparisons were performed to characterize the false-negative cases. Results Both the IgG4-RD patients and mimickers were aged ≥65 years with male predominance. The final diagnoses of mimickers were mainly malignancy, vasculitis, sarcoidosis and aneurysm. The classification criteria had a sensitivity of 72.8% and specificity of 100%. Of the 44 false-negative cases, one did not fulfil the entry criteria, 20 fulfilled one exclusion criterion and 27 did not achieve sufficient inclusion criteria scores. The false-negative cases had fewer affected organs, lower serum IgG4 levels, and were less likely to have received biopsies than the true-positive cases. Notably, positive disease-specific autoantibodies were the most common exclusion criterion fulfilled in 18 patients, only two of whom were diagnosed with a specific autoimmune disease complicated by IgG4-RD. In addition, compared with the true-positive cases, the 18 had comparable serum IgG4 levels, number of affected organs, and histopathology and immunostaining scores despite higher serum IgG and CRP levels. Conclusions The ACR/EULAR classification criteria for IgG4-RD have an excellent diagnostic specificity in daily clinical practice. Positive disease-specific autoantibodies may have limited clinical significance for the diagnosis of IgG4-RD.

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 Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Teresa Carbone ◽  
Ricardo Azêdo Montes ◽  
Beatriz Andrade ◽  
Pedro Lanzieri ◽  
Luis Mocarzel
Keyword(s):  
Elderly Patient ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Lymphocytic Infiltration ◽  
Serum Levels ◽  
High Serum ◽  
Orbital Pseudotumor ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.

scholarly journals Needle biopsy compared with surgical biopsy: pitfalls of small biopsy in histologial diagnosis of IgG4-related disease

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Yanying Liu ◽  
Fei Yang ◽  
Xiying Chi ◽  
Yuxin Zhang ◽  
Jiangnan Fu ◽  
...  
Keyword(s):  
Needle Biopsy ◽  
Plasma Cells ◽  
Classification Criteria ◽  
Histological Diagnosis ◽  
Obliterative Phlebitis ◽  
Surgical Biopsy ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Significant Difference ◽  
Storiform Fibrosis

Abstract Objective The growing utilization of needle biopsy has challenged the current pathology consensus of IgG4-related disease (IgG4-RD). The aims of this study were to identify the histological characteristics of needle biopsy and surgical specimens and evaluate the ability of needle biopsy in histological diagnosis of IgG4-RD. Methods Biopsies from patients who were referred to as IgG4-RD by the 2019 ACR/EULAR IgG4-RD classification criteria in Peking University People’s Hospital from 2012 to 2019 were re-evaluated. Typical histological features and diagnostic categories were compared between needle biopsy and surgical biopsy. Results In total, 69 patients met the 2019 ACR/EULAR classification criteria and 72 biopsies of them were re-evaluated. All cases showed lymphoplasmacytic infiltrate, while storiform fibrosis and obliterative phlebitis were only present in 35 (48.6%) and 23 (31.9%) specimens, respectively. Storiform fibrosis was more likely to be seen in retroperitoneum lesion (P = 0.033). Surgical biopsy showed significantly higher IgG4+ plasma cells/high-power field (IgG4/HPF) count (P < 0.01) and higher proportion of IgG4/HPF > 10 (P < 0.01). No significant difference was observed with regard to the ratio of IgG4+ plasma cells/IgG+ plasma cells (IgG4/IgG) (P = 0.399), storiform fibrosis (P = 0.739), and obliterative phletibis (P = 0.153). According to the 2011 comprehensive diagnostic criteria, patients who performed a needle biopsy were less likely to be probable IgG4-RD (P = 0.045). Based on the 2011 pathology consensus, needle biopsy was less likely to be diagnosed as IgG4-RD (P < 0.01), especially to be highly suggestive IgG4-RD (P < 0.01). Only 1/18 (5.6%) needle salivary specimens fulfilled the cutoff of IgG4/HPF > 100, which was significantly less than 15/23 (65.2%) of surgical ones (P < 0.01). Conclusions Needle biopsy shows an inferiority in detecting IgG4/HPF count but not in IgG4/IgG ratio, storiform fibrosis, and obliterative phlebitis. Compared with surgical samples, needle biopsy is less likely to obtain a histological diagnosis of IgG4-RD. A different IgG4/HPF threshold for needle biopsy of the salivary glands may be considered.

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

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