BACKGROUND The 2023 ACR/EULAR Antiphospholipid Syndrome (APS) Classification Criteria development, aiming to identify patients with high likelihood of APS for research, employed a four-phase methodology. Phase I and II resulted in 27 proposed candidate criteria, organized into laboratory and clinical domains. Here, we summarize the last stage of Phase III efforts employing a consensus-based multi-criteria decision analysis (MCDA) to weigh candidate criteria and identify an APS classification threshold score. METHODS We evaluated 192 unique, international real-world cases referred for "suspected APS" with a wide range of APS manifestations. Using proposed candidate criteria, subcommittee members rank-ordered 20 representative cases from highly unlikely to highly likely APS. During an in-person meeting, the subcommittee refined definitions and participated in an MCDA exercise to identify relative weights of candidate criteria. Using consensus decisions and pairwise criteria comparisons, 1000Minds™ software assigned criteria weights, and we rank ordered 192 cases by their additive scores. A consensus-based threshold score for APS classification was set. RESULTS Pre-meeting evaluation of 20 representative cases demonstrated variability in APS assessment. MCDA resolved 81 pairwise decisions; relative weights identified domain item hierarchy. After assessing 192 cases by weights and additive scores, the Steering Committee reached consensus that APS classification should require separate clinical and laboratory scores, rather than a single aggregate score, to ensure high specificity. CONCLUSION Using MCDA, candidate criteria preliminary weights were determined. Unlike other disease classification systems using a single aggregate threshold score, separate clinical and laboratory domain thresholds were incorporated into the new APS classification criteria.

Background: Data suggest that food allergies greatly impact a child’s health and growth due to inadequate nutrient intake. Our study aimed to establish the long-term outcome of children with food allergies compared to a control group. Methods: This study was a retrospective cohort study with longitudinal follow-up with a mean period of 4.85 years from the diagnosis to the last study visit. The patients’ nutritional intake was assessed using a three-day food diary and analysed by a dietitian. Patients (61 boys and 33 girls, mean age 6.9 years) had a single food allergy including 21 patients with cow’s milk, 34 with egg, and 39 with peanut allergies. The control group included 36 children (19 boys and 17 girls, mean age 8.03 years). Blood analysis was performed on all participants. Results: Data from our study showed that patients with cow’s milk, egg or peanut allergies had normal growth and achieved catch-up growth from the diagnosis until the last study visit. In the cow’s milk allergy group, the allergy was shown to affect calcium intake (p < 0.05), while egg and peanut allergies did not impact the dietary intake of nutrients. None of the investigated food allergies affected blood results (p < 0.05). Conclusions: In the present study, we showed that single food allergies do not compromise growth in children if they are provided with appropriate support and that the affected children reach catch-up growth from the diagnosis.

Objective To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR. Methods This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and literature review; (2) Phase II, criteria reduction by modified Delphi and nominal group technique exercises; (3) Phase III, criteria definition, further reduction with the guidance of real-world patient scenarios, and weighting via consensus-based multicriteria decision analysis, and threshold identification; and (4) Phase IV, validation using independent adjudicators’ consensus as the gold standard. Results The 2023 ACR/EULAR APS classification criteria include an entry criterion of at least one positive antiphospholipid antibody (aPL) test within 3 years of identification of an aPL-associated clinical criterion, followed by additive weighted criteria (score range 1–7 points each) clustered into six clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and two laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti–β2-glycoprotein I antibodies). Patients accumulating at least three points each from the clinical and laboratory domains are classified as having APS. In the validation cohort, the new APS criteria vs the 2006 revised Sapporo classification criteria had a specificity of 99% vs 86%, and a sensitivity of 84% vs 99%. Conclusion These new ACR/EULAR APS classification criteria were developed using rigorous methodology with multidisciplinary international input. Hierarchically clustered, weighted, and risk-stratified criteria reflect the current thinking about APS, providing high specificity and a strong foundation for future APS research.

To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR.

Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients there are significant differences between the clinical presentation of diffuse and limited subtypes. We reviewed clinical differences in presentation of subtypes in patients in the juvenile systemic scleroderma inception cohort (jSScC).To study the clinical presentation of jSSc patients with diffuse (djSSc) and limited (ljSSc) subtypes.We reviewed the baseline clinical characteristics of the patients, who were recruited to the jSScC till December 2022. jSScC is a prospective cohort of jSSc patients, who developed the first non-Raynaud´s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion.The JSScC included 232 patients, 68% (n=159) had diffuse subtype. The median age at onset of Raynaud phenomenon was 10.4 years (7.3-12.9), at the first non-Raynaud symptom 10.9 years (7.3-13.0) and median disease duration 2.5 years (1.0-4.6). The female/male ratio was significantly lower in the djSSc subtype (3:1 versus 5:1, p<0.001). Antibody profile was similar, with the exception of a significantly higher number of anticentromere positive patients in the ljSSc. Decreased FVC<80% was found in approximately 30% and decreased DLCO<80% was found in around 40% in both subtypes. Abnormal HRCT findings were found in 44% of patients. Pulmonary hypertension assessed by ultrasound occurred in approximately 5% in both groups and gastrointestinal involvement in 43% of djSSc and 36% in ljSSc (p=0.303). Patients with djSSc had significantly higher modified Rodnan Skin Score, more frequently sclerodactyly, a history of digital ulceration active ulceration, telangiectasia, a decreased Body Mass Index z score ≤ -2 and decreased joint range of motion. Patients with ljSSc had significantly higher rate of cardiac involvement. Regarding patient related outcomes assessed by VAS 0-100 djSSc patients had more severe disease also physician related outcome assessed by VAS 0-100 were significantly higher in djSSc (see Table 1).Table 1.Comparison of subtypes at time of inclusion in the cohortWhole Group N=232Diffuse Subtype N=159Limited Subtype N=73P value Anticentromere5% (7/156)2% (2/106)10% (5/50)0.022 MRSS, median (IQR)10 (4 – 20)16 (8 - 27)4 (0 – 8)0.001 Gottron Papules26% (59/228)31% (48/155)15% (11/73)0.011 Sclerodactyly75% (165/219)85% (127/150)55% (38/69)<0.001 Telangiectasia37% (77/209)44%(62/141)22% (15/68)0.002 History of ulceration52% (119/229)62% (98/158)30% (21/71)<0.001 Active ulceration17% (39/229)21% (33/158)8% (6/71)0.021 Only Cardiac involvement5% (12/232)3% (4/159)11% (8/73)0.007 BMI<- 2 z score15% (33/217)20% (29/148)6% (4/69)0.008 Joints with decreased range59% (136/231)64% (101/158)48% (35/73)0.022Physician Reported (Median, IQR) Physician global disease activity30 (20 – 45)n=19735 (20– 50)n=13820 (10 – 30)n=590.001 Physician global disease damage30 (15 – 40)n=19530 (20 – 45)n=13820 (5 – 30)n=570.004 Physician ulceration activity0 (0 – 16)n=2165 (0 – 20)n=1540 (0 – 0)n=620.018Patient Reported (Median, IQR) Patient global disease activity40 (20 – 50)n=17840 (20 – 50)n=12930 (15 – 55)n=490.024 Patient global disease damage30 (15 – 60)n=17740 (20 – 60)n=12825 (5 – 55)n=490.001 Patient Raynaud activity30 (10 – 60)n=20230 (10 – 60)n=14515 (0 – 55)n=570.001 Patient ulceration activity0 (0 – 30)n=20310 (0 – 30)n=1450 (0 – 20)n=580.001In the largest jSSc cohort in the world, djSSc patients have a significantly more severe disease. Patients and physician related outcomes were significantly more severe in djSSc group. Interestingly, we found no differences regarding interstitial lung disease, pulmonary hypertension or gastrointestinal involvement, although the number of patients with decreased BMI ≤ -2 z score was significantly higher in the djSSc patients.NIL.NIL.None Declared.