Curious Case of a Retroperitoneal Mass With an Elevated Serum IgG3 Level—An Exception to the 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4‐Related Disease: Comment on the Article by Wallace et al

2020 ◽  
Vol 72 (11) ◽  
pp. 1957-1958
Author(s):  
Jaclynn Levine ◽  
Phot Luisiri
Keyword(s):  
Elevated Serum ◽  
Classification Criteria ◽  
Retroperitoneal Mass ◽  
Related Disease ◽  
American College Of Rheumatology ◽  
Igg4 Related Disease ◽  
European League Against Rheumatism ◽  
European League

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 Performance of the 2019 ACR/EULAR classification criteria for IgG4-related disease and clinical phenotypes in a Spanish multicentre registry (REERIGG4)

Rheumatology ◽  
2020 ◽  
Vol 60 (1) ◽  
pp. 217-223
Author(s):  
Andreu Fernández-Codina ◽  
Blanca Pinilla ◽  
Iago Pinal-Fernández ◽  
Iago Carballo ◽  
Carlos Feijoo-Massó ◽  
...  
Keyword(s):  
Classification Criteria ◽  
Geographical Differences ◽  
Cross Sectional ◽  
Related Disease ◽  
American College Of Rheumatology ◽  
Igg4 Related Disease ◽  
European League Against Rheumatism ◽  
Phenotype Distribution ◽  
Patient Series ◽  
Pathology Reports

Abstract Objectives Several IgG4-related disease (IgG4-RD) phenotypes have been proposed and the first set of classification criteria have been recently created. Our objectives were to assess the phenotype distribution and the performance of the classification criteria in Spanish patients as genetic and geographical differences may exist. Methods We performed a cross-sectional multicentre study (Registro Español de Enfermedad Relacionada con la IgG4, REERIGG4) with nine participating centres from Spain. Patients were recruited from November 2013 to December 2018. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria (AECC) were used. Results We included 105 patients; 88% had Caucasian ethnicity. On diagnosis, 86% met the international pathology consensus while 92% met the Japanese comprehensive criteria. The phenotype distribution was head and neck 25%, Mikulicz and systemic (MS) 20%, pancreato-hepato-biliary (PHB) 13%, retroperitoneal and aorta (RA) 26%. Sixteen per cent had an undefined phenotype. Seventy-seven per cent of the cases met the AECC. From the 24 patients not meeting the AECC, 33% met exclusion criteria, and 67% did not get a score ≥20 points. Incomplete pathology reports were associated to failure to meet the AECC. Conclusions The PHB phenotype was rare among Spanish IgG4-RD patients. The MS phenotype was less frequent and the RA phenotype was more prevalent than in other, Asian patient series. An undefined phenotype should be considered as some patients do not fall into any of the categories. Three quarters of the cases met the 2019 AECC. Incomplete pathology reports were the leading causes of failure to meet the criteria.

THU0293 PREDICTORS OF LONG-TERM THERAPY WITH GLUCOCORTICOID IN POLYMYALGIA RHEUMATICA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 374-375
Author(s):  
A. Aoki ◽  
H. Kobayashi
Keyword(s):  
Polymyalgia Rheumatica ◽  
Clinical Symptoms ◽  
Therapy Group ◽  
Classification Criteria ◽  
Term Therapy ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
Long Term Therapy ◽  
European League

Background:Polymyalgia rheumatica (PMR) is a common inflammatory condition of elderly persons. Clinical symptoms respond to low-dose glucocorticoids (GC), but treatment is often required for several years. 2015 EULAR/ACR recommendations1)recommend considering early introduction of methotrexate (MTX) in addition to GC, particularly in patients at a high risk for relapse and/or prolonged therapy. However, risk factors for prolonged therapy are not clear yet.Objectives:We investigated predictive factors which corresponded to the long-term GC therapy.Methods:This was a retrospective study in a single general hospital in Japan. We reviewed the medical records of the Japanese patients with PMR between April 2011 and January 2020. Diagnosis of PMR was based on Bird’s criteria or 2012 EULAR/ACR Classification Criteria2). All patients were treated with prednisolone (PSL), according to the BSR and BHPR guidelines3), for more than 6 months. Patients treated with MTX and accompanied by the giant cell arteritis were excluded from this study. Relapse was defined as the reappearance of symptoms associated with elevated C-reactive protein (CRP) levels in patients receiving GC that required an increase in GC dose. Remission was defined as the absence of clinical symptoms and normal CRP with discontinuation of GC. We compared the clinical findings, laboratory data at baseline and clinical course between those who achieved remission within 2 years (early-remission group) and those who required GC therapy for more than 2 years (long-therapy group). Comparisons between groups were made using Student’s t-test and chi-square test (IBM SSPE statistics version 26). This study was approved by the ethics committee of Tokyo Medical University (T2019-0079).Results:As of January 2020, 89 patients have been treated with PSL for more than 6 months. 50 patients have achieved a remission, 29 were undergoing treatment, and 10 have transferred to other hospitals or died (Table 1). The median time required for the patients to achieve remission was 16 months (Interquartile Range 12-21). After one-year GC therapy, remission was achieved in 14% (11/77), 66% (41/62) after 2-year, 84% (47/56) after 3-year, and 91.0% (49/54) after 4-years. Forty-one patients, who achieved remission within 2 years, were included in the early-remission group. Twenty-one were included in the long therapy group (Table 1). There were no differences in sex, age at onset, body mass index, clinical features, and serum albumin at diagnosis. Serum CRP of long-therapy group was significantly higher than those of the early-remission group (Table 2). Mean relapse times in the full follow-up times were 0.4 in the early-remission group and 3.1 in the long-therapy group. Multivariate logistic regression analysis showed that history of relapse till 6 months was significant predictors of the long-term GC therapy (odds ratio, 6.48; 95%CI 1.44-29.12).Conclusion:The remission rates of our study are lower than those of the previous reports. We have tapered GC gradually according to the BSR and BHPR guidelines3). However, some patients need the long-term therapy for more than 2 years. We might consider additional MTX therapy in patients who experience a relapse during the first six months.References:[1]Dejaco C, et al. 2015 recommendations for the management of polymyalgia rheumatica: a European League against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis 2015; 74:1799-1807.[2]Dasgupta B, et al: 2012 provisional classification criteria for polymyalgia rheumatica: a European League against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis 2012;71: 484-492.[3]Dasgupta, B, et al. BSR and BHPR guidelines for the management of polymyalgia rheumatica. Rheumatology 2010; 49:186-190.Disclosure of Interests:None declared

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