Preliminary Classification Criteria Research Articles

Overlap of International League of Associations for Rheumatology and Preliminary Pediatric Rheumatology International Trials Organization Classification Criteria for Nonsystemic Juvenile Idiopathic Arthritis in an Established UK Multicentre Inception Cohort.

The goal was to assess the degree of overlap between existing International League of Associations for Rheumatology (ILAR) and preliminary Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria for juvenile idiopathic arthritis (JIA). Participants from the Childhood Arthritis Prospective Study, a multicenter UK JIA inception cohort, were classified using the PRINTO and ILAR classification criteria into distinct categories. Systemic JIA was excluded because several classification items were not collected in this cohort. Adaptations to PRINTO criteria were required to apply to a UK health care setting, including limiting the number of blood biomarker tests required. The overlap between categories under the two systems was determined, and any differences in characteristics between groups were described. A total of 1,223 children and young people with a physician's diagnosis of JIA were included. Using PRINTO criteria, the majority of the patients had "other JIA" (69.5%). There was a high degree of overlap (91%) between the PRINTO enthesitis/spondylitis- and ILAR enthesitis-related JIA categories. The PRINTO rheumatoid factor (RF)-positive category was composed of 48% ILAR RF-positive polyarthritis and 52% undifferentiated JIA. The early-onset antinuclear antibodies-positive PRINTO category was largely composed of ILAR oligoarthritis (50%), RF-negative polyarthritis (24%), and undifferentiated JIA (23%). A few patients were unclassified under PRINTO (n=3) and would previously have been classified as enthesitis-related JIA (n=1) and undifferentiated JIA (n=2) under ILAR. Under the preliminary PRINTO classification criteria for childhood arthritis, most children are not yet classified into a named category. These data can help support further delineation of the PRINTO criteria to ensure homogenous groups of children can be identified.

Single-Factor Comprehensive Reservoir Quality Classification Evaluation-Taking the Hala'alat Mountains at the Northwestern Margin of the Junggar Basin as an Example.

In the process of petroleum geology exploration and development, reservoir quality evaluation is an essential component. However, conventional reservoir quality evaluation methods are no longer able to provide accurate and comprehensive assessments for all types of reservoirs. Therefore, the comprehensive evaluation of reservoir quality using multiple single factors is of significant importance in improving the level of reservoir quality assessment and enhancing the effectiveness of oil and gas exploration techniques. Conventional reservoir quality evaluation methods can assess only the quality of individual reservoir properties, resulting in limited classification outcomes. Taking the Cretaceous formations in the southern margin of the Hala'alat Mountain in the Junggar Basin as the research object, preliminary classification criteria were established based on the principles of formation coefficient, storage coefficient, and flow unit index. Combining experimental data such as core observation, thin-section identification, pore permeability analysis, and scanning electron microscopy, a comprehensive set of reservoir quality classification and evaluation criteria were developed. Furthermore, the corresponding reservoir classification evaluation maps were generated to illustrate the spatial distribution of reservoir quality. The study reveals that the area can be classified into four types of reservoirs, namely, Class I, Class II, Class III, and Class IV, corresponding to the best reservoir, relatively good reservoir, relatively poor reservoir, and poor reservoir, respectively. Among them, the second (K1q2) and third (K1q3) members of the Cretaceous Qingshuihe Formation, as well as the first (K1h1) and third (K1h3) members of the Cretaceous Hutubi Formation, exhibit the best reservoir quality as Class II. On the other hand, the second member of the Cretaceous Hutubi Formation (K1h2) exhibits the best reservoir quality as Class III, with relatively poorer reservoir quality overall. The research findings of this study can provide an important theoretical basis for oil and gas exploration and development in the region.

E36 Juvenile scleroderma in rheumatology unit Tripoli children hospital

Abstract Introduction Juvenile scleroderma is a rare chronic systemic connective tissue disease. It is classified into two main forms: localized scleroderma and systemic sclerosis. Since it is rare in children, many aspects of the disease remain undiscussed. Aim To study the clinical and demographic characteristics of patients diagnosed with various types of scleroderma who presented at the pediatric rheumatology unit in Tripoli Children hospital from the year 2000–2022 Materials and methods This is a retrospective case series conducted by reviewing the files of patients with scleroderma diagnosed and followed up in pediatric rheumatology unit from year 2000–2022 and collecting the data regarding patients’ demographics, time of diagnosis, clinical features, and laboratory results. Results Seven patients who met preliminary classification criteria for JSS were included. 2 males and 5 females. 1 patient with systemic sclerosis, 1 patient with linear morphea, 2 patients with generalized morphea, 1 patient with Parry Romberg syndrome, 1 patient with CREST syndrome, and 1 patient with deep morphea. The mean age at diagnosis was 8.2 ± 4.5 years. The first symptom attributable to JSS was hyperpigmented skin patches in 5 cases, no one presented with Raynaud's phenomenon. Respiratory involvement occurred in one patient, and it manifested as dyspnea as well as abnormal imaging and/or pulmonary function tests showed mild restrictive lung disease. Dysphagia was the commonest gastrointestinal symptom (4 patients, 57%). The most frequent musculoskeletal symptom was arthritis found in in 4 (57.1%) patients. Uveitis in 1 (14.3%), ANA was positive in 4 (57%) children: RF positive in 2 (28.6%) patients, ENA negative in the 7 (100%) patients. Skin biopsy was done in 2 patients and was consistent with scleroderma. Regarding treatment, six patients received MTX, one patient received prednisolone and one patient received MMF. Conclusion This report is the first describing Libyan patients with scleroderma. As reported in the literature, localized forms are more common than systemic sclerosis. Also a rare form of scleroderma (Parry Romberg syndrome) was present. Data about the disease mainly based on studies on adults and we need more studies on pediatric patients. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

ALLERGIC DISEASES IN ADULT-ONSET STILL'S DISEASE AND RHEUMATOID ARTHRITIS

A high prevalence of allergic diseases was found in patients with adult-onset Still's disease (AOSD). However, the relative prevalence is unknown compared with other diseases. We sought to compare the prevalence of allergic diseases in the control group of patients with rheumatoid arthritis (RA). We retrospectively examined consecutive patients diagnosed with AOSD or RA in our hospital from 2010 to 2020. The patients with AOSD met the preliminary criteria for classification of AOSD. The patients with RA met the EULAR/ACR 2010 criteria. We included patients with RA without other rheumatic diseases. The analysis was performed on six types of allergic reactions: food allergy, drug allergy, allergic contact dermatitis, allergic rhinitis and/or allergic conjunctivitis, and asthma. Twenty-four patients with AOSD and 409 patients with RA were enrolled. The median ages (AOSD, RA) were 46.6, 68.2 years old. Females were 83.3%, 78.0%. Fifty% of AOSD patients and 34.5% of RA patients presented at least one type of allergic diseases (p = 0.12). These included food allergy (4.2%, 6.4%: p = 1.0), drug allergy (37.5%, 16.6%: p = 0.02), allergic rhinitis/allergic conjunctivitis (25.0%, 8.6%: p = 0.02), contact dermatitis (4.2%, 4.4%: p = 1.0), and asthma (4.2%, 5.9%: p = 1.0). Patients with AOSD had a higher prevalence of drug allergy, and allergic rhinitis/allergic conjunctivitis than patients with RA.

Clinical and Laboratory Characteristics and Mortality in Korean Patients with Systemic Sclerosis: A Nationwide Multicenter Retrospective Cohort Study.

We aimed to investigate demographic and clinical features and predictors of mortality in Korean patients with systemic sclerosis (SSc). We performed a retrospective multicenter medical chart review in Korean patients diagnosed with SSc from 1986 to 2016 at 11 university hospitals representing each geographic area of Korea. SSc patients were defined according to the American College of Rheumatology preliminary classification criteria and subtyped as limited cutaneous (lcSSc) or diffuse cutaneous (dcSSc) SSc. We enrolled 751 patients (female, 86.7%; mean age at diagnosis, 48.9 yrs). The most common organ involvement was interstitial lung disease (52.7%), followed by gastroesophageal reflux disease (32.9%) and pulmonary arterial hypertension (13.6%). Patients with lcSSc were more common than those with dcSSc (64.8 vs 35.2%), whereas anti-Scl-70 and anticentromere antibody positivity were identified in 302 (42.5%) and 175 (25.5%) patients, respectively. In the 46 (6.1%) patients who developed a malignancy, lung cancer (23.9%) was the most common diagnosis, followed by gastric (13%) and breast cancer (13%). During the study period, 57 (7.6%) patients died, and the 5- and 10-year survival rates were 94% and 87%, respectively. Increased age at diagnosis, cardiovascular involvement, and anti-Scl-70 antibody positivity were significant predictors of death. Clinical manifestations and survival rates in Korean SSc patients are similar to those of other populations. However, the prevalence of anti-Scl-70 antibody is higher in Korean SSc patients compared with whites, while the prevalence of anticentromere antibody is lower.

Automatic Identification and Classification of Fetal Heart-Rate Decelerations from Cardiotocographic Recordings.

Cardiotocography (CTG) consists in the simultaneous recording of two distinct traces, the fetal heart rate (FHR; bpm) and the maternal uterine contractions (UCs; mmHg). CTG analysis consists in the evaluation of specific features of traces, among which fetal decelerations (DECs) are considered the "center-stage" since possibly related to fetal distress. DECs are classified based on their duration and occurrence in relation to UCs as prolonged, early, late and variable; each class associates to a specific status of the fetus health. Typically, CTG traces are visually interpreted; however, computerized CTG analysis may overcome subjectivity in CTG interpretation. Thus, this study proposes a new automatic algorithm for computerized identification and classification of DECs. The algorithm was tested on the 552 CTG recordings constituting the "CTU-CHB intra-partum CTG database" of Physionet. Of these, 470 (85.15%) were found suitable for automatic DECs identification and classification. Overall, 5888 DECs were identified, of which 3255 (55.28%) were classified while the other 2633 (44.72%) remained unclassified due to very strict preliminary classification criteria (now required for avoiding misclassifications). Among the classified DECs, 468 (14.38%) were classified as prolonged, 1498 (46.02%) as early, 32 (0.98%) as late, 1257 (38.62%) as variable. Thus, among the classified DECs, the most common are the early and the variable ones (overall 84.64%), the occurrence of which ranged from 0 to 14 DECs per recording. These findings are in agreement with what reported in literature. In conclusion, the proposed algorithm for automatic DECs identification and classification represents a useful tool for computerized CTG analysis.

Classification of Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 ACR/EULAR classification criteria for SSc

Objective: To classify Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Classification Criteria (new 2013 criteria).Methods: We assessed 120 patients who visited Kanazawa University Hospital suspected of SSc and who did not meet the 1980 ACR preliminary classification criteria for SSc. We clinically diagnosed 16 patients with primary Raynaud’s disease and 104 with mild/early SSc prior to being assessed by the new 2013 criteria.Results: None of the 16 patients with primary Raynaud’s disease met the new 2013 criteria. On the other hand, 94 out of the 104 patients (90.3%) with mild/early SSc by our clinical diagnosis met the new 2013 criteria. Among the 94 SSc patients, sclerodactyly was detected in 58 (62%), puffy fingers in 62 (66%), abnormal nailfold capillaries in 89 (95%), Raynaud’s phenomenon in 93 (99%), and SSc-related autoantibodies (Abs) in 85 (90%). The median (range) score of these 94 patients was 12 (9–14). Ten mild/early SSc patients who did not meet the new 2013 criteria had the following clinical features: puffy fingers in 1 (10%), abnormal nailfold capillaries in 8 (80%), Raynaud’s phenomenon in 9 (90%), and SSc-related autoAbs in 8 (80%). The median (range) score of these 10 patients was 7 (5–8).Conclusion: The new 2013 criteria can classify most mild/early Japanese SSc patients, which may contribute to early treatment interventions.

AB0558 What is the Prevalence of NON Criteria Antiphospholipid Antibodies in Patients with Antiphospholipid Syndrome?

BackgroundIncreasing interest has focussed upon assays which are not currently included in the Antiphospholipid syndrome (APS) classification criteria to detect antibodies directed against other phospholipids (PL), PL binding proteins and/or...

The Concept of Early Systemic Sclerosis Following 2013 ACR\EULAR Criteria for the Classification of Systemic Sclerosis

For many years observational studies and clinical trials on systemic sclerosis (SSc) have been carried out only on patients who met the 1980 American College of Rheumatology-ACR preliminary classification criteria. However, this lead to the exclusion from all those studies of subset patients, particularly those with a limited cutaneous SSc-sine scleroderma subset, because, despite a diagnosis of SSc based on Raynaud's phenomenon (RP) associated with digital pitting scars/ulcers, or by sclerodactyly and telangiectasia and/or typical esophagopathy and/or interstitial fibrosis detected by High Resolution Computed Tomography of the lungs, they did not satisfy the classification criteria. In that setting, LeRoy and Medsger proposed to label as affected by limited SSc (lSSc) or early SSc, cases presenting with RP associated with an SSc-type nailfold capillary pattern and/or SSc-selective autoantibodies. In 2008, Koenig et al. validated these criteria and proposed to name early SSc, or pre-scleroderma, patients with RP and either a scleroderma marker autoantibody or typical capillaroscopy abnormalities or both, who had been clearly shown to have high probabilities but not the certainty to develop definite SSc during a 20-year follow-up. This definition has been recently challenged by the development of the new ACR/EULAR criteria for the classification of SSc. At present, to be labeled as affected by early SSc, or pre-scleroderma, a patient should suffer from RP associated with either scleroderma marker autoantibodies or typical capillaroscopy findings and should not meet the 2013 ACR/EULAR criteria for the classification of SSc nor should he/she meet the criteria for SSc sine scleroderma.

Generalized morphea: A rare variant of localized scleroderma

Morphea, a rare type of localized scleroderma, is a fibrosing disorder of skin and subcutaneous tissue. It has a bimodal presentation and is common in both children and adults. The patient may show positivity for antinuclear antibodies, rheumatoid factor, or antibodies to ssDNA. The disease lesions rarely undergo spontaneous resolution. The below given images depict the diverse disease presentations observed in a 35-year-old female affected with generalized morphea. The patient was having a history of skin thickening and discoloration over her forearms since 3 years. Physical examination revealed the occurrence of new lesions on the arms, abdominal surface, and legs. The patient fulfilled the Padua preliminary classification criteria for generalized morphea (≥4 large plaques and each >3 cm) on at least 2 of 7 anatomic areas: head/neck, right upper extremity, left upper extremity, right lower extremity, left lower extremity, anterior trunk, posterior trunk. 1 She had no active arthritis or myositis, though she complained of multiple joint pains. Her ANA was positive (1.9) and skin biopsy showed thick collagen deposition in dermis, flattened epidermis, and no inflammatory infiltrates.

Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease

Antiphospholipid syndrome (APS) is a multisystem autoimmune condition characterized by vascular thromboses and/or pregnancy loss associated with persistently positive antiphospholipid antibodies (aPL). Catastrophic APS (CAPS) is the most severe form of APS with multiple organ involvement developing over a short period of time, usually associated with microthrombosis. 'Definite' and 'probable' CAPS have been defined based on the preliminary classification criteria; however, in a real-world setting, aPL-positive patients with multiple organ thromboses and/or thrombotic microangiopathies exist who do not fulfill these criteria. Previous APS diagnosis and/or persistent clinically significant aPL positivity is of great importance for the CAPS diagnosis; however, almost half of the patients who develop CAPS do not have a history of aPL positivity. The purpose of this paper is to summarize the diagnostic challenges and the recently updated diagnostic algorithms for CAPS providing a 'step-by-step' approach for clinicians (and researchers) in the assessment of patients with multiple organ thromboses.

Prevalence and incidence of systemic sclerosis in southern Sweden: population-based data with case ascertainment using the 1980 ARA criteria and the proposed ACR-EULAR classification criteria

ObjectivesTo estimate the prevalence and incidence of systemic sclerosis (SSc) in southern Sweden.MethodsIn Skåne, the southernmost region of Sweden (total population 1.2 million), healthcare provided is registered in the Skåne...

THU0388 Tocilizumab strongly suppresses the serum amyloid a level in refractory adult – onset still’s disease associated amyloidosis

BackgroundA amyloidosis, is a rare but serious complication of chronic inflammatory conditions including Adult-onset Still’s disease (AOSD). Treatment can be frustrating too.ObjectivesWe describe the case of refractory AOSD who developed...

OP0034 Performance of the New EULAR/ACR Classification Criteria for Systemic Sclerosis in Clinical Practice

BackgroundThe preliminary classification criteria for systemic sclerosis (SSc) developed in 1980 lack sensitivity for early and mild SSc patients. Therefore, the ACR-EULAR committee developed new classification criteria for SSc (1)....

FRI0252 Severity of skin, tendon and joint damage in chinese patients with systemic sclerosis (SSC) is associated with higher disability and poorer health-related quality of life: A case-control study

ObjectivesTo study the disability and health-related quality of life (HRQoL) in Chinese patients with SSc, and its relationship with the extent of skin involvement and organ damage.MethodsConsecutive patients who fulfilled...

SAT0175 Results of the Classification Criteria for Cryoglobulinemic Vasculitis Validation Study

Backgroundthe preliminary Classification Criteria for cryoglobulinemic vasculitis (CV) have been developed in 2011 by an European cooperative study (1).Objectivesto validate these Classification Criteria for CV with the participation of non-European...

SAT0010 Common epitope structure of HLA-DPB1 in systemic sclerosis

BackgroundSpecific alleles of HLA-DPB1 (HLA-DPB1*0901, *1301, *0301) were found to be most strongly associated with anti-topoisomerase I (Topo-I)-positive systemic sclerosis (SSc) in multiethnic genome-wide association study and HLA-analysis (1, 2)....

Classification Criteria and Diagnostic Tests for Vasculitides

Most rheumatic diseases, in adults or children, do not have objective clinical or laboratory measures that allow physicians to diagnose a specific disease with certainty. In order to overcome the clinical dilemma of a proper diagnosis, classification criteria have been proposed for several rheumatic diseases. Classification criteria are therefore an essential tool for clinical research since they allow comparison of patients with similar clinical and laboratory characteristics across studies. In the vasculitis research field several classification criteria have been proposed for the adult population, and more recently for children. These criteria have been derived through 2 major methodological approaches: consensus and consensus/data-driven. In brief, the consensus-based criteria are specifically derived through the combination of judgments from a group of experts in a particular field after thorough literature review and sometimes developed using consensus techniques such as the Delphi technique and nominal group technique1,2. To the consensus-based criteria we can ascribe the Chapel Hill International Consensus Conference3 that essentially provided proper nomenclature for systemic vasculitides, and the Childhood Vasculitis Working Group of the Paediatric Rheumatology European Society (PRES) for preliminary classification criteria of childhood vasculitis4. These criteria have their main strength in the panel of contributors who are usually very well recognized experts/experienced clinicians in that particular research/clinical field. The second approach of consensus/data-driven usually starts from consensus-based criteria and adds a formal statistical validation based on collected data in order to provide accuracy measures (classic methods), or other alternative methods such as the classification tree5. Only the classical approach is discussed here. With the classic approach, the accuracy measures that are considered include sensitivity (ability of a classification criterion to identify a patient as having the disease based on a gold standard; also called true-positive rate); specificity (ability of classification criteria … Address correspondence to Dr. Ruperto; E-mail: nicolaruperto{at}ospedale-gaslini.ge.it

Report from the EULAR ACR Scleroderma Classification Criteria Committee

Classification criteria for SSc are being updated as previous ACR preliminary classification criteria did not include some lcSSc patients and early SSc.Objectives. Items from two large European and NA surveys were reduced by experts using Delphi and nominal group techniques that resulted in 23 items. The face, discriminant and construct validity were studied using many databases in Europe and NA. Further item reduction occurred ranking 20 cases of SSc and mimickers by experts with variable probabilities of having SSc and weighing items relative to each other after agreeing on further item reduction using the 1000 minds exercise.Results. In the Delphi exercise, skin thickening, SSc-specific autoantibodies, abnormal nailfold capillary pattern, RP and some organ involvement ranked highest. Compared with diseases that mimic SSc in multiple databases, SSc patients are more likely to have skin thickening (OR = 427), telangiectasias (OR = 91), RNA polymerase III (OR = 75), puffy fingers (OR = 35), finger flexion contractures (OR = 29), tendon/bursal friction rubs (OR = 27), topo I (OR = 25), RP (OR = 24), finger tip ulcers/pitting scars (OR = 19), anti-centromere (OR = 14), abnormal nailfold capillaries (OR = 10), GERD (OR = 8) and ANA, calcinosis, dysphagia, oesophageal dilation (all OR = 6), ILD/pulmonary fibrosis (OR = 5) and PM-Scl antibody (OR = 2). Low DL CO and FVC and PAH had OR <2. Renal crisis and digital pulp loss/acro-osteolysis did not occur in SSc mimickers. The 1000 minds exercise had experts ranked 20 cases and eliminated some of the 23 items. A scoring system found variable weighting of several items.Future direction. Thousand minds cases will be rescored by experts and then further item reduction will occur by consensus and statistical item reduction for redundant criteria, with validation in databases and prospectively collected patients with standardized case report forms at multiple sites of cases and mimickers.

Letter to the Editor Catastrophic antiphospholipid syndrome in a patient with V Leiden variant

Letter to the Editor Catastrophic antiphospholipid syndrome in a patient with V Leiden variant