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Vascular involvement in IgG4-related disease (IgG4-RD), is a well-recognized feature and large vessel commitment, especially the aorta, can be the only manifestation of the disease. Being a newly recognized disease, its diagnosis and workup still represents a challenge in clinical practice. A 47-year-old-man with two aortic aneurysms ruptures, one at abdominal and the other at thoracic level, was referred to our rheumatology department. The initial analysis of the surgical specimen obtained 3 years earlier revealed a nonspecific aortitis. Re-evaluation of the biopsy with immunohistology now demonstrated the presence of IgG4 deposits. Evidence-based recommendations regarding diagnosis, treatment and follow-up of IgG4-related large-vessel involvement are lacking. In this particular case, histopathology were crucial. The authors review and discuss vascular involvement in IgG4-RD and respective treatment options.

ObjectiveTo compare physical disability, mental health, fatigue and health-related quality of life (HRQoL) across juvenile idiopathic arthritis (JIA) categories in adulthood and between JIA and adult-onset rheumatic diseases.MethodsCross-sectional analysis nested in a cohort of adult patients with JIA registered in the Rheumatic Diseases Portuguese Register (Reuma.pt). Physical disability (Health Assessment Questionnaire—Disability Index), mental health symptoms (Hospital Anxiety and Depression Scale), fatigue (Functional Assessment of Chronic Illness Therapy—Fatigue Scale (FACIT-F)) and HRQoL (EuroQol-5D (EQ5D) and Short Form (SF-36)) were compared across JIA categories. Patients with polyarticular JIA and enthesis-related arthritis (ERA) JIA were compared respectively to patients with rheumatoid arthritis (RA) and spondyloarthritis (SpA), matched for gender and age, adjusted for disease duration and activity.Results585 adult patients with JIA were included. Comparison across JIA categories showed that persistent oligoarthritis and patients with ERA reported a higher score in EQ5D and SF-36 physical component when compared with other JIA categories.Polyarticular JIA reported less disability and fatigue than patients with RA (median Health Assessment Questionnaire of 0.25 vs 0.63; p<0.001 and median FACIT-F score 42 vs 40 ; p=0.041). Polyarticular JIA had also better scores on EQ5D and all domains of SF-36, than patients with RA. Patients with ERA reported less depression and anxiety symptoms (0% vs 14.8%; p=0.003% and 9% vs 21.3%; p=0.002) and less fatigue symptoms (45 vs 41; p=0.01) than patients with SpA.ConclusionPersistent oligoarticular JIA and ERA are the JIA categories in adulthood with better HRQoL. Overall, adult polyarticular and patients with ERA JIA have lower functional impairment and better quality-of-life than patients with RA and SpA.

ObjectivesIdiopathic inflammatory myopathies (IIM) are a group of rare disorders that can affect the heart. This work aimed to find predictors of cardiac involvement in IIM.MethodsMulticenter, open cohort study, including patients registered in the IIM module of the Rheumatic Diseases Portuguese Register (Reuma.pt/Myositis) until January 2022. Patients without cardiac involvement information were excluded. Myo(peri)carditis, dilated cardiomyopathy, conduction abnormalities, and/or premature coronary artery disease were considered.Results230 patients were included, 163 (70.9%) of whom were females. Thirteen patients (5.7%) had cardiac involvement. Compared with IIM patients without cardiac involvement, these patients had a lower bilateral manual muscle testing score (MMT) at the peak of muscle weakness [108.0 ± 55.0 vs 147.5 ± 22.0, p=0.008] and more frequently had oesophageal [6/12 (50.0%) vs 33/207 (15.9%), p=0.009] and lung [10/13 (76.9%) vs 68/216 (31.5%), p=0.001] involvements. Anti-SRP antibodies were more commonly identified in patients with cardiac involvement [3/11 (27.3%) vs 9/174 (5.2%), p=0.026]. In the multivariate analysis, positivity for anti-SRP antibodies (OR 104.3, 95% CI: 2.5-4277.8, p=0.014) was a predictor of cardiac involvement, regardless of sex, ethnicity, age at diagnosis, and lung involvement. Sensitivity analysis confirmed these results.ConclusionAnti-SRP antibodies were predictors of cardiac involvement in our cohort of IIM patients, irrespective of demographical characteristics and lung involvement. We suggest considering frequent screening for heart involvement in anti-SRP-positive IIM patients.

Background:In rheumatoid arthritis (RA), global disease activity is commonly assessed, from the patient’s and the physician’s perspective, through a 100mm VAS. Previous studies have commonly shown a considerable discrepancy between the patient’s and physician’s assessment.Objectives:This study aimed evaluating patient-physician discordance in the assessment of disease activity and to explore its determinants.Methods:Cross sectional study including RA patients (ACR/EULAR 2010 classification criteria), aged ≥ 18 years, followed in a single tertiary centre. Data were collected from the most recent evaluation including sociodemographic features, disease duration (years), disease activity (DAS 28 3V-PCR), tender and swollen joint count 0-28 (TJC and SJC), VAS-pain-patient, patient and physician global assessment (PGA and PhGA respectively), erythrocyte sedimentation rate (ESR), C-reactive protein (CPR), Health assessment questionnaire (HAQ) and EuroQol five-dimension scale (EQ5D). The discrepancy between patients and physicians (ΔPPhGA) was defined as PGA minus PhGA, and a difference > |20mm| was considered as “discordant”. A descriptive analysis was performed and variables described as proportions or means (+/- SD), as adequate. Correlation between ΔPPhGA and other variables was assessed through Pearson’s correlation and comparison between groups through t-test. Variables with p<0.05 or otherwise considered clinically relevant were included in multiple linear regression analysis to identify predictors for ΔPPhGA. A p≤0.05 was considered statistically significant.Results:In total, 467 patients with RA were included (81.2% female; mean age 63.9% ± 12.2 years). PGA and PhGA were discordant in 61.7% of the cases, the patient scoring higher than the physician in 95% of these cases. The proportion of concordance increased (p< 0.01) when considering only patients in remission (DAS 28 3V <2.6), (Graph 1). ΔPPhGA was moderately correlated with VAS-pain-patient (r = 0.59) and weakly correlated with SJC (r = -0.12), HAQ (r= 0.27), EQ5D (r = -0.28) and age (r = 0.21); all p<0.01. In multivariate analysis, VAS-pain-patient (β 0.74, 95% CI 0.62-0.88, p=0.00) and TJC (β 0.16, 95% CI 0.45-0.48, p=0.02) remained associated with a higher ΔPPhGA.Conclusion:Our study confirmed that a significant discrepancy between patients and physicians in the assessment of global disease activity is frequent in clinical practice, probably due to valorization of different parameters. This was much less pronounced among patients in remission. Higher VAS-pain-patient and TJC were independent predictors of greater discrepancy between patients and physician’s assessment.Disclosure of Interests:Luisa Brites: None declared, Flavio Costa: None declared, João Dinis de Freitas: None declared, Mariana Luis: None declared, Ana Rita Prata: None declared, Helena Assunção: None declared, LILIANA SARAIVA: None declared, Marlene Sousa: None declared, Ana Rita Cunha: None declared, José Antonio P. da Silva Grant/research support from: Pfizer, Abbvie, Consultant of: Pfizer, AbbVie, Roche, Lilly, Novartis, Catia Duarte: None declared