OBJECTIVES:Juvenile idiopathic arthritis (JIA) leads to significant long-term morbidity from articular and extra-articular complications, yet the burden of comorbidities in adults with long-standing disease is not well characterised. This study aimed to determine the prevalence and incidence of key comorbidities in adults with JIA and assess their association with demographic and clinical features. METHODS:We performed a national multicentre retrospective cohort study using data from adults with JIA, defined by the 2001 ILAR criteria, enrolled in the Portuguese Rheumatic Diseases Register (Reuma.pt). Demographic and clinical data, along with comorbidities, were collected. Comorbidities included cardiovascular disease, hypertension, dyslipidaemia, diabetes, thyroid disease, amyloidosis, inflammatory bowel disease, allergy and asthma, osteoporosis, psychiatric disease, and autoimmune disease. Rare conditions were grouped into broader categories. Extra-articular JIA manifestations were excluded. Incidence rates were calculated as the number of new events per 1,000 person-years (95% CI), and prevalence was assessed using frequencies. RESULTS:The cohort included 748 patients, 65.6% female, with a median age of 27.7 years and a median disease duration of 20.6 years. Oligoarticular JIA was the most common subtype (29.9%). Autoimmune diseases had the highest incidence rate (7.1/1,000 person-years), followed by hypertension (5.1/1,000 person-years) and psychiatric disease (4.0/1,000 person-years). Hypertension (9%), psychiatric disease (8%), and osteoporosis (5%) were the most prevalent comorbidities. Biologic DMARD use was associated with reduced risk of psychiatric disease (OR=0.38, p=0.03), and no significant association with malignancy or infection was found. CONCLUSIONS:JIA patients with long-standing disease frequently develop comorbidities, particularly hypertension. Biologic therapy seems to reduce the risk of comorbidities. Long-term monitoring of comorbidities in JIA patients is paramount.

IgG4-related disease (IgG4-RD) is an uncommon fibro-inflammatory pseudotumoral entity characterized by slowly progressive systemic manifestations.It affects mainly middle-aged and older men 1 , involving various organs like the pancreas, salivary and lacrimal glands, retroperitoneal region, and vessels.Diagnosing IgG4-RD is challenging since it mimics malignancies, vasculitis and granulomatous disorders.The available diagnostic criteria 2 still lack validation, and the 2019 ACR/ EULAR criteria 3 purpose classification only.Corticosteroid (CCT) treatment has shown positive outcomes 4 , although relapses are common 5 .Therefore, maintenance treatment with immunomodulatory drugs is often necessary.To characterize the Portuguese population of IgG4-RD patients under Rheumatology care, we conducted a national multicenter observational study focused on patients with a clinical IgG4-RD diagnosis (62.5% met the 2019 ACR/EULAR classification criteria) followed-up in Rheumatology departments.Data collection occurred from 30/03/2022-20/12/2022.This work was previously presented at EULAR congress 2023, as abstract number AB1507.We included twenty-four patients with a mean current age of 59.95 years (standard deviation [SD]=13.35).The mean age at diagnosis was 56.09 years (SD=14.13),and the mean age at the onset of symptoms was 53.96 years (SD=14.19).Twelve (50%) patients were male.Table 1 depicts this cohort' s characteristics.The most common overall manifestations involved salivary glands (37.5%), followed by orbits, lacrimal glands and aorta (25% each), and pancreas (20.8%).We documented single organ disease in six (25%) patients.

Purpose: Psoriatic arthritis (PsA) and psoriasis (Pso) are highly heterogeneous inflammatory diseases. Multidisciplinary approaches are associated with improved results in both musculoskeletal (MSK) and skin manifestations. We describe the experience and main diagnostic and therapeutic outcomes of one of the largest and longest-running Rheumatology/Dermatology multidisciplinary PsA Clinic. Methods: Single center, cross-sectional study of all patients observed at the PsA Clinic of Hospital de Santa Maria, Portugal, between November 2010 and February 2021. The total number of visits/ patients, demographics, referral indications, and definite skin and MSK diagnosis were registered. In patients with PsA confirmed diagnosis, PsA and Pso characteristics, previous treatments and their modifications were captured using Reuma.pt. Results: Eight hundred and two visits were performed, corresponding to 505 patients, 51.3% female, with a mean age of 51.0 +/- 13.8 years. The main indication for referral was diagnosis uncertainty (56.4%), and a definitive PsA diagnosis was established in 28.9% of these cases. For patients in whom PsA was not identified, the main alternative diagnoses were osteoarthritis [peripheral (n = 70) or axial (n = 29)], fibromyalgia (n = 22), axial spondylarthritis without Pso (n = 21), tendinitis/enthesitis (n = 20), and carpal tunnel syndrome (n = 19). The main alternative dermatological diagnoses were seborrheic dermatitis (n = 15), nail dystrophy not due to Pso (n = 15), onychomycosis (n = 14) and eczema (n = 9). In patients with confirmed PsA (n = 308), 54.5% had already been treated with disease-modifying antirheumatic drugs (DMARD), and 15.9% had received at least one biologic DMARD. Treatment was modified in 78.9% of PsA patients, 58.0% due to uncontrolled skin activity, 34.5% MSK activity, and 7.7% both. Most of the treatment changes occurred due to lack of efficacy (56.4%). Conclusion: This study shows the impact, through diagnostic precision (by increasing the number of psoriatic and non-psoriatic definite diagnoses), and treatment modifications, for skin and MSK manifestations, from over 10 years of the implementation of a multidisciplinary PsA Clinic.

INTRODUCTIONDespite years of experience with biological disease modifying anti-rheumatic drugs (bDMARD) in rheumatoid arthritis (RA), little is known about differences in infectious risk among bDMARDs. The aim of this study was to assess the incidence and type of infections in RA patients on bDMARDs and to determine possible predictors.METHODSA retrospective multicenter cohort study that included patients registered in the Rheumatic Diseases Portuguese Registry (Reuma.pt) with RA, and exposed to at least one bDMARD until April 2021. RA patients under bDMARD and with at least one episode of severe infection (SI), defined as infection that requires hospitalization, use of parenteral antibiotics or that resulted in death, were compared to patients with no report of SI. Demographic and clinical data at baseline and at the time of each SI were collected to establish comparisons between different groups of bDMARDs. Comparisons between different bDMARDs were assessed and logistic regression was performed to identify predictors of SI.RESULTSWe included 3394 patients, 2833 (83.5%) female, with a mean age at RA diagnosis of 45.5±13.7 years. SI was diagnosed in 142 of the 3394 patients evaluated (4.2%), totaling 151 episodes of SI. At baseline, patients with SI had a significantly higher proportion of prior orthopedic surgery, asthma, interstitial lung disease, chronic kidney disease and corticosteroid use, higher mean age and longer median disease duration at first bDMARD. Nine patients died (6.0%). Ninety-two SI (60.9%) occurred with the first bDMARD, the majority leading to discontinuation of the bDMARD within 6 months (n=75, 49.7%), while 65 (43.0%) restarted the same bDMARD and 11 (7.3%) switched to another bDMARD (6 of them to a different mechanism of action). In the multivariate analysis, we found that chronic kidney disease, asthma, infliximab, corticosteroid use, interstitial lung disease, previous orthopedic surgery, higher Health Assessment Questionnaire and DAS284V-ESR are independent predictors of SI.CONCLUSIONThis study described the incidence and types of SI among Portuguese RA patients on biologics, identifying several predictors of SI, both globally and with different bDMARDs. Physicians should be aware of the real-word infectious risk in RA patients on bDMARDs when making treatment decisions.