scholarly journals International classification criteria created for IgG4-related disease

2020 ◽  
Vol 16 (3) ◽  
pp. 126-126
Author(s):  
Joanna Clarke
Keyword(s):  
Classification Criteria ◽  
International Classification ◽  
Related Disease ◽  
Igg4 Related Disease

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

The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease

2019 ◽  
Vol 79 (1) ◽  
pp. 77-87 ◽  
Author(s):  
Zachary S Wallace ◽  
Ray P Naden ◽  
Suresh Chari ◽  
Hyon K Choi ◽  
Emanuel Della-Torre ◽  
...  
Keyword(s):  
Test Performance ◽  
Clinical Findings ◽  
Classification Criteria ◽  
Related Disease ◽  
American College Of Rheumatology ◽  
Igg4 Related Disease ◽  
European League Against Rheumatism ◽  
Wide Range ◽  
Multispecialty Group ◽  
European League

IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.

scholarly journals The new 2019 American college of rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-related disease

2020 ◽  
Vol 58 (4) ◽  
pp. 368-375
Author(s):  
E. V. Sokol
Keyword(s):  
Clinical Practice ◽  
Diagnostic Criteria ◽  
Classification Criteria ◽  
Related Disease ◽  
American College Of Rheumatology ◽  
Igg4 Related Disease ◽  
European League Against Rheumatism ◽  
Potential Use ◽  
European League

This paper considers the new 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease, discusses essential differences with IgG4-RD comprehensive diagnostic criteria (Umehara H., 2011) and comments their potential use in clinical practice. 

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 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

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