scholarly journals Reliability Exercise for the Polymyalgia Rheumatica Classification Criteria Study: The Oranjewoud Ultrasound Substudy

2009 ◽  
Vol 2009 ◽  
pp. 1-5 ◽  
Author(s):  
Alexander K. Scheel ◽  
Eric L. Matteson ◽  
Bhaskar Dasgupta ◽  
George A. W. Bruyn ◽  
Sarah Ohrndorf ◽  
...  

Objective. A study supported by the EULAR and the ACR being conducted to establish classification criteria for polymyalgia rheumatica (PMR) will include ultrasound examination of the shoulders and hips. Ultrasound (US) depicts glenohumeral joint effusion, biceps tenosynovitis, subdeltoid bursitis, hip joint synovitis, and trochanteric bursitis in PMR. These findings may aid in distinguishing PMR from other diseases. The purpose of this study was to assess standards and US interreader agreement of participants in the PMR classification criteria study.Methods. Sixteen physicians in four groups examined shoulders and hips of 4 patients and 4 healthy adults with ultrasound. Overall agreement and interobserver agreement were calculated.Results. The overall agreement (OA) between groups was 87%. The OA for healthy shoulders was 88.8%, for healthy hips 100%, for shoulders with pathology 85.2%, and 74.3% for hips with pathology, respectively.Conclusion. There was a high degree of agreement found for the examination of healthy shoulders and pathologic hips. Agreement was moderate for pathologic shoulders and perfect for healthy hips. US of shoulder and hips performed by different examiners is a reliable and feasible tool for assessment of PMR related disease pathology and can be incorporated into a classification criteria study.

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Yanying Liu ◽  
Fei Yang ◽  
Xiying Chi ◽  
Yuxin Zhang ◽  
Jiangnan Fu ◽  
...  

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.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1224.1-1224
Author(s):  
I. Mizushima ◽  
T. Yamano ◽  
H. Kawahara ◽  
S. Hibino ◽  
R. Nishioka ◽  
...  

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


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 374-375
Author(s):  
A. Aoki ◽  
H. Kobayashi

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


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Stavros Chrysidis ◽  
Philip Rask Lage-Hansen ◽  
Nikoletta Svendsen ◽  
Andreas P. Diamantopoulos

Abstract Objectives This study aimed to investigate the hospitalisation rates and the reasons for hospitalisation in patients with polymyalgia rheumatica (PMR). Furthermore, it aimed to clarify the impact of a newly established Fast Track Clinic (FTC) approach on hospitalisation rates in connection with PMR diagnosis. Methods Patients diagnosed with PMR at South-West Jutland Hospital, Denmark, between 2013 and 2018 were included retrospectively. Only patients fulfilling the 2012 EULAR/ACR classification criteria were included in our cohort. An FTC for patients suspected of having PMR was established in the rheumatologic department of South-West Jutland Hospital in January 2018. Results Over 6 years (2013 to 2017), 254 patients were diagnosed with PMR, 56 of them while hospitalised. Hospitalised patients were more likely to have a higher initial CRP mean ± standard deviation (SD) 99.53 ± 59.36 vs 45.82 ± 36.96 mg/lt (p <  0.0001) and a shorter duration of symptoms (p = 0.0018). After implementing the FTC, a significant decrease in hospitalisation rates (from 20.4% to 3,5%) and inpatient days of care (mean ± SD 4.15 ± 3.1 vs 1 ± 0) were observed. No differences between the two groups were observed regarding clinical symptoms, laboratory values and initial prednisolone dose. Conclusion A substantial number of patients are hospitalised in connection with the PMR diagnosis. The FTC approach can decrease the hospitalisation rates significantly among these patients. Trial registration Retrospectively registered.


Author(s):  
Daniel Aletaha ◽  
Helga Radner

Rheumatoid arthritis (RA) is among the most disabling form of chronic inflammatory joint disease. Not all forms of arthritis develop into RA; on the contrary, it may be very challenging to differentiate RA from cases of arthritis that are self-limiting or caused by another disease. Evaluation of early arthritis includes some basic steps, such as excluding trauma, crystal, or infectious-related disease, as well as considering additional features that may guide towards a specific diagnosis. If no specific diagnosis can then be made, the presentation can be labelled as undifferentiated arthritis. Typical differential diagnoses of RA include viral polyarthritis, seronegative spondylarthropathies, polymyalgia rheumatic, and other systemic rheumatic diseases. In 2010, new classification criteria were published that led to a change in the approach to RA. Compared to the previous criteria, the American College of Rheumatology (ACR) 1987 criteria, a scoring system was devised, appreciating the type and number of affected joints (up to 5 points), as well as serology (up to 3 points), elevated acute-phase reactants (1 point), and a symptom persistence of 6 weeks or longer (1 point). If 6 or more points are reached, then classifiable RA is present. Importantly, classification status, which is used for study purposes, is not always identical to the diagnostic status, which often leads to clinical treatment.


2015 ◽  
Vol 104 (12) ◽  
pp. 2576-2580
Author(s):  
Kei Sasaki ◽  
Hiroshi Ohtani ◽  
Mikio Fujii ◽  
Hideki Yamada ◽  
Shinichiro Otsuka ◽  
...  

2019 ◽  
Vol 79 (1) ◽  
pp. 77-87 ◽  
Author(s):  
Zachary S Wallace ◽  
Ray P Naden ◽  
Suresh Chari ◽  
Hyon K Choi ◽  
Emanuel Della-Torre ◽  
...  

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.


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