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Background:Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by joint inflammation and cartilage destruction. In addition to its impact on musculoskeletal system, RA has been recognized as a systemic disease associated with an increased risk of cardiovascular disease. Current CV risk screening and management strategies underestimate the actual CV risk in RA. Thus, an adequate CV risk stratification has special relevance in RA to identify patients at risk of CV disease.Objectives:To evaluate the cardiovascular risk profile in patients with RA by assessing both traditional CVD risk factors, carotid ultrasound finding and risk estimation using SCORE and SCORE2.Methods:A cross-sectional study was performed including adult moderate to severe RA patients who had initiated treatment with JAK inhibitors or anti TNF. Traditional risk factors, including age, gender, smoking status, hypertension, dyslipidemia, and diabetes were evaluated. The widely used scoring systems, such as the Systematic Coronary Risk Evaluation (SCORE) and its updated version, SCORE2, were employed to estimate the global cardiovascular risk in this population. Furthermore, carotid ultrasound examinations were conducted to investigate subclinical atherosclerosis in RA patients. Measurements of carotid intima-media thickness (IMT) were obtained as a surrogate marker of early vascular damage. Additionally, the presence of atherosclerotic plaques, a hallmark of advanced vascular disease, was documented. This study was performed from September-2022 to April-2023. The study was approved by the Ethics Committee and statistical analysis was performed using R.Results:A total of 122 patients were included in the study. Demographical and clinical variables are shown in Table 1. Among the RA patients, a high prevalence of CVD risk factors was observed. The mean values of SCORE and SCORE2 indicated a moderate to high estimated 10-year risk of fatal CVD events in this population (mean SCORE: 2.77%, mean SCORE2: 4.07%).Carotid ultrasound measurements revealed an increased mean IMT in RA patients (mean right carotid cIMT: 0.64 [0.12]; mean left carotid cIMT 0.69 [0.11]). Furthermore, a substantial proportion of patients (34.1%) displayed the presence of atherosclerotic plaques.The concordance correlation coefficient between SCORE and SCORE2 demonstrated a significant moderate positive relationship (estimate 0.32 [0.19-0.43] IC 95%). ROC curves were calculated for SCORE and SCORE2 regarding their predictive capacity for plaques, showing a significant difference between them (p<0.001) indicating a superior predictive capacity of SCORE2. A linear regression model, adjusted for confounding factors, was used to assess the relationship between SCORE and SCORE2 with IMT. Both SCORE (estimate 1.41 p=0.02, R2=0.045) and SCORE2 (estimate 1.55 p<0.01, R2=0.015) were positively associated with IMT.Table 1. Demographic, clinical characteristics and Ultrasound ResultsFigure 1.Conclusion:The relationship between SCORE and SCORE2 was found to be satisfactory, indicating their adequacy for prediction plaque development. However, the predictive capacity of both scores may be further enhanced by incorporating multiplication factors. Additionally, both SCORE and SCORE2 were positively associated with IMT, but SCORE2 demonstrated a greater predictive capacity in this regard. Furthermore, the SCORE2 explained a higher proportion of the IMT variation compared with SCORE.REFERENCES:[1] Smolen JS, Aletaha D, Barton A, et al. Rheumatoid arthritis. Nat Rev Dis Prim. 2018;4:1–23.[2] Seoane-Mato D, Sánchez-Piedra C, Silva-Fernández L, et al. Prevalencia de enfermedades reumáticas en población adulta en España (estudio EPISER 2016). Objetivos y metodología. Reumatol Clin. 2019;15(2):90-6[3] González Mazarío R, Fragío Gil JJ, Martínez Calabuig P, et al. Cardiovascular risk assessment with carotid ultrasound in rheumatoid arthritis. Med Clin (Barc). 2022 Nov 25;159(10):470-474. English, Spanish.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Idiopathic inflammatory myopathies (IIM) constitute a heterogeneous group of systemic rheumatic diseases characterized by chronic muscle weakness and the infiltration of mononuclear cells into muscle tissue. The etiology is unknown; however, the association between IIM and neoplastic processes is recognized, being more pronounced in dermatomyositis (DM) compared to polymyositis (PM). Nonetheless, the nature and extent of this association are not fully elucidated.Objectives:To analyze the clinical phenotype and compare the clinical and serological characteristics of patients with neoplasia-associated inflammatory myopathy.Methods:An observational retrospective case-control study was conducted at a single center from 2016 to 2023, including patients diagnosed with IIM. The case group consisted of patients with paraneoplastic DM and those with non-neoplasia-associated IIM as controls; demographic, serological, clinical variables, and outcomes were assessed. Patients with less than 12 months of disease progression were excluded.Results:A total of 52 patients were analyzed, of which 9 (17.3%) had paraneoplastic DM and 43 (82.7%) had non-neoplasia-associated IIM. The median age in the paraneoplastic cohort was 67 years (CI) 50-92), while in the non-paraneoplastic cohort it was 62 years (CI 21-90). The paraneoplastic IIM group was 66.7% female, compared to 76.7% in the non-paraneoplastic group. The average follow-up was 3 years (CI 1-43) for patients with paraneoplastic DM and 4 years (CI 1-31) for those without neoplasms. Several autoantibodies, in addition to those already known to be associated with paraneoplastic IIM, were observed in the case group, with 66.67% (n=6) positive for anti-Ro52, 33.3% (n=3) positive for anti-TIF1 gamma, and 11.1% (n=1) for anti-NXP2, anti-MDA5, anti-Mi2-b, anti-Jo1, anti-PL7, anti-PL12, and anti-SAE antibodies. In contrast, the control group showed a more variable representation of other positive autoantibodies. The median number of positive autoantibodies in both groups was 2 per patient. The rest of the clinical, analytical, and demographic variables are shown in Table 1. Regarding serological markers, no statistically significant differences were observed between the two groups in any of the measured variables (Table 1). Notable variables include the initial ESR, which had a median of 29 mm/h (CI 2-104) for paraneoplastic IIM and 22 mm/h (CI 1-90) for non-paraneoplastic IIM (p = 0.796). Similarly, the final ESR was 20 mm/h (CI 3-94) versus 20 mm/h (CI 2-88) respectively (p = 0.969). As for CPK, the median peak value in the case group was 1234 U/L (CI 159-10000) compared with 1998 U/L (CI 159-9980) in the control group (p = 0.178), and the post-treatment CPK was 135 U/L (CI 42-800) versus 174 U/L (CI 41-1230), respectively (p = 0.079).Table 1. Demographic, clinical, and analytical variables of patients with inflammatory myopathies.Conclusion:Notable disparities were evident between the groups when evaluating the spectrum of autoantibodies associated with idiopathic inflammatory myopathies. The case group predominantly consisted of patients positive for multiple autoantibodies, prominently including anti-Ro52, as well as autoantibodies such as anti-TIF1 gamma and anti-NXP2, which are classically linked to neoplastic conditions. Interestingly, similar autoantibody positivity was observed among control group patients, despite the absence of any malignant neoplastic correlation. Serological variables did not demonstrate statistically significant differences across the cohorts, underscoring the complexity of correlating serological markers with neoplastic associations in IIM.neoplasms. No statistically significant differences were observed regarding serological variables in both groups.REFERENCES:[1] Selva-O’Callaghan A, Pinal-Fernandez I, Trallero-Araguás E, Milisenda JC, Grau-Junyent JM, Mammen AL. Classification and management of adult inflammatory myopathies. Lancet Neurol. 2018 Sep;17(9):816-828.[2] McHugh NJ, Tansley SL. Autoantibodies in myositis. Nat Rev Rheumatol. 2018 Apr 20;14(5):290-302.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of systemic autoimmune diseases with significant multi-domain impact, including muscle, skin, lungs, and the cardiovascular system. Myositis-associated autoantibodies, present in approximately 70% of patients with IIM, are associated with clinical phenotypes within IIM. This study focuses on the actual association between these autoantibodies and IIM.Objectives:To determine the prevalence and clinical correlation of various autoantibodies in patients with suspected IIM, establishing a connection with different forms of IIM and their relationship with other comorbidities (including neoplasms and other systemic autoimmune diseases).Methods:A retrospective observational study was conducted at a single hospital between 2016 and 2023, examining positive myositis western blots in patients suspected of inflammatory myopathies (antigens studied were CN-1A, Mi-2a, Mi-2b, TIFg, MDA5, NXP2, SAE1, Ku, PM-Scl100, PM-Scl75, Jo1, SRP, PL-7, PL-12, EJ, OJ, Ro-52). Included patients met the 2017 ACR/EULAR classification criteria for IIM. Demographic, clinical, and analytical data were also collected from the clinical stories’ of these patients including the presence of neoplasms and other systemic autoimmune diseases and the associated clinical phenotype.Results:Among the 520 positive antibodies by western blot (Table 1), 52 cases of IIM were confirmed. Differences were observed in the prevalence of specific autoantibodies, such as anti-MDA5 (46.15%), anti-TIF1 gamma (25.81%), anti-NXP2 (17.65%), among others. The association of specific forms of IIM was noted, as in anti-TIF1 gamma and anti-NXP2 autoantibodies with paraneoplastic dermatomyositis (n=4) or antisynthetase antibodies (anti-Jo1, PL-7, PL12…) which showed an association with antisynthetase syndrome (n=6). However, the expected clinical correlations of certain autoantibodies did not always manifest (e.g., Anti-SRP with necrotizing myopathy (n=0)). The presence of multiple autoantibodies was notable, with a median of 2 positive autoantibodies per patient. Additionally, 67.31% presented positivity for antinuclear antibodies (ANAs), with a mostly granular immunofluorescence pattern (25%). Furthermore, malignant neoplasms were recorded in 8 patients with IIM, though not exclusively in those with autoantibodies typically associated with cancer (anti-MDA5, anti-Mi2b, anti-Jo1…). The rest of the demographic results are gathered in Table 2.Table 1. Prevalence among patients with positive autoantibodies and patients with inflammatory myopathy.Table 2. Demographic, clinical, and analytical variables of patients with inflammatory myopathies.Conclusion:Among the total patients with positive autoantibodies by western blot, only 10% were related to IIM, with a higher autoantibody-IIM correlation observed with anti-MDA5, anti-TIF1 gamma, and anti-NXP2; the latter two being most commonly associated with the phenotype classically related to them (in this case, paraneoplastic dermatomyositis). In total, 8 malignant neoplasms were detected, although not exclusively in those with autoantibodies typically associated with cancer. Furthermore, in patients with IIM, the presence of multiple antibodies was observed (with a median of 2), and in the majority of patients, there was coexistence of positive ANAs with a granular pattern.REFERENCES:[1] Selva-O’Callaghan A, Pinal-Fernandez I, Trallero-Araguás E, Milisenda JC, Grau-Junyent JM, Mammen AL. Classification and management of adult inflammatory myopathies. Lancet Neurol. 2018 Sep;17(9):816-828.[2] McHugh NJ, Tansley SL. Autoantibodies in myositis. Nat Rev Rheumatol. 2018 Apr 20;14(5):290-302.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:IgG4-related disease (IgG4-RD) is a rare, systemic, immunomediated fibroinflammatory process with uncertain etiology and pathophysiology that can affect multiple organs, presenting common clinical, radiological, and serological characteristics. Although the disease is associated with IgG4, serum levels are not elevated in all patients; it has also been described in other respiratory system diseases (bronchiectasis, asthma, sarcoidosis), pancreatic diseases (chronic pancreatitis), liver diseases (cirrhosis), or in relation to other autoimmune diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), or inflammatory myopathies.Objectives:To determine the prevalence and clinical significance of IgG4 positivity in the blood of patients with an initial suspicion of systemic autoimmune disease.Methods:A single-center cross-sectional study was conducted, in which all results from the electronic medical records of patients with elevated IgG4 (>135 mg/dL) from a single center and requested by various hospital departments from January 2010 to August 2022 were analyzed. Demographic data were collected, as well as final diagnoses, including those with IgG4-RD.Results:A total of 182 patients with serological elevation of IgG4 were reviewed (Table 1). Of the patients analyzed, only 13 met the diagnostic criteria for IgG4-RD according to the Umehara-Okazaki 2011 criteria, and 7 met the ACR/EULAR 2019 criteria. Among the remaining patients, the majority (45.1%) presented with respiratory pathology, mostly comprising patients with COPD (13.19%), bronchiectasis (8.79%), or asthma (5.5%). An elevation of IgG4 was also observed in other systemic autoimmune diseases (20.33%), being more frequent in patients diagnosed with SLE (3.3%), RA (2.2%), or EGPA (2.75%); or patients diagnosed with some malignancy (10.99%), most frequently in pulmonary (3.3%) or pancreatic and biliary tract cancers (2.2). Finally, elevation of this immunoglobulin was also observed in patients with digestive pathology (9.34%), especially in patients with pancreatitis (6.04).Table 1. Variables Collected from Patients with Elevated IgG4Conclusion:The study reflects that an elevation of IgG4 is not exclusive to IgG4-related disease (IgG4-RD), also appearing in various respiratory pathologies (COPD, bronchiectasis, asthma), autoimmune diseases (SLE and RA), and neoplasms. These data suggest that high levels of IgG4 could indicate a more generalized immunological reaction and would not be useful as a screening tool for IgG4-RD.REFERENCES:[1] Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb 9;366(6):539-51.[2] Iaccarino L, Talarico R, Scirè CA, Amoura Z, Burmester G, Doria A, et al. IgG4-related diseases: state of the art on clinical practice guidelines. RMD Open. 2019 Jan 19;4(Suppl 1):e000787.[3] Wallace ZS, Naden RP, Chari S, Choi H, Della-Torre E, Dicaire JF, et al; The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol. 2020 Jan;72(1):7-19.Acknowledgements:NIL.Disclosure of Interests:None declared.

BackgroundBelimumab efficacy and safety has been studied through randomized controlled trials in Systemic Lupus Erythematosus (SLE) patients even in cases with kidney involvement. However, more evidence is needed not only from long-term trials but also from real world conditions especially in Lupus Nephritis (LN) patients.ObjectivesTo analyze the effectiveness and safety of Belimumab in SLE patients with data from a Real-World cohort.MethodsA single center observational study was performed including SLE patients who had initiated treatment with Belimumab from September 2017 to January 2023. Demographic, clinical, laboratory, effectiveness and safety variables were collected. Effectiveness was evaluated according to changes from the baseline in SLEDAI-2K and disease activity markers (proteinuria, complement consumption and/or Anti DNAds). Safety data was collected including any adverse event (AE) due to any cause. AE was considered serious (SAE) if it was life-threatening or result in hospitalization, disability or in death.ResultsOverall, 15 patients were included in the study, whom baseline characteristics are exposed in the Table 1. Nine patients were still receiving the drug with a mean drug survival of 15.6 months. Belimumab allowed steroid tapering in all cases, but treatment was discontinued just in 1 patient (7%). Treatment also improved disease activity markers in all (100%) patients. Belimumab was well tolerated, and the AE reported were infection (14 events) and malaise in 1 patient. In 11 cases, infection was mild (9 upper respiratory tract infection, 1 urinary tract infection and 1 gastroenteritis). 3 severe infections were registered (1 pneumonia, 1 pyelonephritis and 1 meningitis).Regarding LN patients, treatment exposure achieved was 10.85 patients/year. Renal Biopsy demonstrated class III in a patient (20%) and class IV in 4 patients (80%). Mean proteinuria at baseline was 6.66g/24h. In 3 cases, Belimumab was started in the first 6 months after LN diagnosis was established. In 4 cases (80%), Belimumab addition allowed significant reduction of proteinuria and corticosteroids. In 2 out 5 (40%) treatment was discontinued, one case due to an insufficient response and in the other, to a SAE (Cryptococcus neoformans meningitis).ConclusionBelimumab maintained an acceptable safety profile and an adequate effectiveness. Intravenous and subcutaneous formulations showed similar performance. Belimumab addition resulted in reduction in proteinuria and corticosteroid use.Table 1.BASELINE CHARACTERISTICSCharacteristicAll SLE patientsn=15 (100%)No Lupus NephritisN=10 (37%)Lupus Nephritisn=5(51%)Age – years (ds)36.4 (11.4)30.5 (6.9)48.2 (9.2)Female sex – number (%)14 (%)10 (100%)4 (%)Race – number (%)WhiteHispanicAsian13 (86.7%)1 (6.67%)1 (6.67%)10 (100%)0 (0%)0 (0%)3 (60%)1 (20%)1 (20%)Formulation use at baseline – number (%)IntravenousSubcutaneous5 (33.3%)10 (66.7%)3 (30%)7 (70%)(40%)3 (60%)Disease features – number (%)Neuropsiquiatric involvementHistory of Lupus NephritisArthritisCutaneousMucosal UlcersHematologicalSerositis3 (20%)5 (33.3%)10 (66.7%)7 (46.7%)4 (26.7%)13 (86.7%)2 (13.3)2 (20%)0 (0%)7 (70)4 (40%)2 (20%)8 (80%)1 (10%)1 (20%)5 (100%)3 (60%)3 (60%)2 (40%)5 (100%)1 (20%)Steroid treatment - NumberMean glucocorticoid dosage – mg of prednisone or equivalent15 (100%)24.4 (18.03)10 (100%)15.2 (7.6)5 (100%)35.4 (20.62)Concomitant medication– number (%)AntimalarialMethotrexateAzathioprineMycophenolateCyclophosphamide14 (93.3%)5 (33.3%)4 (26.7%)5 (33.3%)1 (6.67%)9 (90%)5 (50%)4 (40%)0 (0%)0(0%)5 (100%)0 (0%)0 (%)5 (100%)1 (20%)SLEDAI – mean total score15.7 (5.4)14.6 (6.01)18 (4.2)SLE immunological testsANA positivityComplement component 3Complement component 4Anti-double strained DNA positivityAnti-double strained DNA titters15 (100%)15 (100%)15 (100%)15 (100%)204.08 (220.6)10 (100%)10 (100%)10 (100%)10 (100%)59.2 (29.6)5 (100%)5 (100%)5 (100%)5 (100%)431.2 (206.75)REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Background:Systemic sclerosis (SSc) is an autoimmune disease of unknown etiology, characterized by fibrosis in the skin, vascular walls, and certain organs such as the lungs, digestive tract, heart, or kidneys. The disease is classically divided into limited (lcSSc) or diffuse (dcSSc), according to, among other manifestations, the extent of cutaneous involvement. Within lcSSc, the CREST syndrome is described, which includes calcinosis, Raynaud’s phenomenon, esophageal involvement, sclerodactyly, and telangiectasia. The most characteristic laboratory finding is the presence of antinuclear antibodies (ANA) in over 95%, with anticentromere antibodies (ACA) being among the most common, appearing in 30-80% of lcSSc and less than 5% of dcSSc. Antibodies in SSc are markers of clinical profiles, and although they are not activity markers, they are related to the prognosis of the disease.Objectives:To determine the prevalence and clinical significance of ACA positivity in patients with suspected systemic autoimmune disease, as well as to describe and analyze the epidemiological variables and clinical manifestations of those patients who meet SSc criteria.Methods:A single-center cross-sectional study was performed, including all patients with positive anticentromere antibodies (ACA) through indirect immunofluorescence (IIF) from 2010 to March 2023. The relationship between the presence of antibodies and the diagnosis of SSc according to ACR/EULAR 2013 criteria was analyzed. In addition, demographic data, clinical diagnoses, and clinical manifestations of patients with SSc were collected, based on the responsible physician’s criteria or complementary tests. Skin, pulmonary, digestive, cardiac and renal manifestations, presence of Raynaud’s phenomenon, and other manifestations such as neoplasms, acute myocardial infarctions (AMI), strokes, or deep vein thrombosis (DVT) were included; and their prevalence was analyzed. lcSSc was defined as skin hardening in acral areas, distal to elbows and knees, and on the face; and dcSSc as thickening of the skin on the trunk or proximal limb region.Results:A total of 393 patients with positive ACA on IIF were reviewed, with 58 (15%) meeting SSc criteria. Of these 58, 50 were diagnosed with lcSSc (86%), 7 with SSc sine scleroderma (12%), and only 1 with dcSSc (2%). Among lcSSc cases, 7 met CREST syndrome criteria, 4 had overlap syndrome with SSc and Sjögren’s syndrome, and 1 was diagnosed with Reynolds syndrome (Primary Biliary Cirrhosis + SSc). Table 1 shows demographic variables collected and diagnostic classifications.Among the different clinical manifestations, Raynaud’s phenomenon was present in 100% of patients, of which 41 (71%) had pathological capillaroscopy. Cutaneous manifestations were observed in 66% of patients, followed by digestive manifestations in 53% of cases. Renal involvement was noted in 12%, and cardiac involvement in 10%. Table 2 provides a detailed classification of each domain of manifestations.Table 1. Demographic data and diagnosis of patients with ACA +Table 2. Clinical manifestations SSc patientsConclusion:Fifteen percent of patients with positive ACA met SSc criteria, with 86% classified as lcSSc. Among SSc patients, the majority were women aged 18 to 65 years. The most frequent clinical manifestations were Raynaud’s phenomenon (100%), followed by cutaneous manifestations (66%), especially sclerodactyly, telangiectasia, and calcifications. Digestive manifestations (53%), particularly dysphagia and gastroesophageal reflux; and pulmonary manifestations (17%), with pulmonary hypertension standing out, were also notable for their frequency.REFERENCES:[1] Calderon LM, Pope JE. Scleroderma epidemiology update. Curr Opin Rheumatol 2021;33;122-127.[2] Allanore Y, Simms R, et al. Nature review Primer: Systemic Sclerosis. Nat Rev 2015;1:1-21.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Rheumatisms are the second most frequent reason for consultation in Europe [1]. In Spain, the bulk of prevalence studies exploit public health data [2-3], despite the fact that our health system is mixed. Spanish private health spending reaches 26.7% of the total and is booming [4]. However, there is a fundamental lack of studies focused on private care, most of which are local [5-6].Objectives:To characterise the activity performed by rheumatologists in private medicine throughout Spanish geography.Methods:Members of SERPA working group were summoned to participate by email. They were proposed to carry out a detailed study of the diagnoses and diagnostic-therapeutic procedures carried out in usual practise during 4 consecutive weeks, chosen between November 2022 and January 2023. A shared spreadsheet was developed with predefined diagnostics. All diagnoses made were integrated, so that in the presence of several diagnoses in the same patient, 1 point was added to each corresponding category. Approval was obtained from the EC.Results:Out of 274 rheumatologists working in the private area in Spain [7], a total of 21 rheumatologists from 15 different medical centers have participated. During the month of follow-up, a total of 5,214 diagnoses and 431 therapeutic procedures were obtained. The resuts of the most frequent diagnostics are as follows: OP in first place, followed by OA and STP. However, when we unified the diagnoses by type of disease, the I&SCTD took the first place, followed by OP and OA, and then other diagnoses such as STP and CSS were less common (Figures 1 and 2).Regarding to the procedures, all rheumatologists (21) have performed a total of 287 infiltrations, 7 of them carried out 171 ultrasounds and another 7 carried out 38 capillaroscopies, only 1 performed DEXAs. In our case, the I&STCD stood out over the STP and CSS, contrary to what was expected when comparing our findings with those of the general population from EPISER-2016 and 2000 registries [2-3], where the proportion is inverse. It could be explained because consultations for second opinions or seeking a closer monitoring than the one given in the public system are common in private care.Having used a sampling for convenience, external validity biases are possible. However, we consider that the vast geographical and healthcare environment ubiquity of participating experts provides the analysis of a high representativeness.Conclusion:The practice of rheumatology in the private sector addresses the entire broad spectrum of rheumatic diseases. It is essential to incorporate data from private clinical activity that allows us to specify its contribution to Spanish rheumatology and to offer a more reliable perspective of the impact of rheumatic diseases in our society.REFERENCES:[1] Woolf AD, Zeidler H, Haglund U, Carr AJ, Chaussade S, Cucinotta D, Veale DJ, Martin-Mola E. Musculoskeletal pain in Europe: its impact and a comparison of population and medical perceptions of treatment in eight European countries. Ann Rheum Dis. 2004;63(4):342-7.[2] Seoane-Mato D, Sánchez-Piedra C, Silva-Fernández L, Sivera F, Blanco FJ, Pérez Ruiz F, et al. Prevalencia de enfermedades reumáticas en población adulta en España (estudio EPISER 2016). Objetivos y metodología. Reumatol Clin. 2019;15(2):90-6.[3] Carmona L, Gabriel R, Ballina J, Laffon A. Proyecto EPISER 2000: prevalencia de enfermedades reumáticas en la población española. Rev Esp Reumatol. 2001;28(1):18-25.[4] Instituto para el Desarrollo e Integración de la Sanidad. Sanidad privada aportando valor. Análisis de situación 2023.[5] Nieto González JC. Reumatología privada ambulatoria, estudio descriptivo en la Comunidad de Madrid. Reumatol Clin. 2019;15(5):e10-3.[6] Alegre C. La Reumatología privada en Cataluña. Reumatol Clin. 2019;15(3):170-2.[7] Yoldi Muñoz B, Gómez Centeno A, Moreno Muelas JV. Estado de la reumatología privada en España. Reumatol Clin. 2017;13(6):313-7.Acknowledgements:NIL.Disclosure of Interests:None declared.

IgG4 immunoglobulin-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory process with unclear etiology and pathophysiology. Can affect multiple organs that encompass common pathophysiological, serological and clinical characteristics. To describe the heterogeneity of the clinical presentation, evolution, and treatment of patients diagnosed with IgG4-RD and compare the performance of the last two IgG4-RD classification and diagnostic criteria. Single-center retrospective study in which patients with a possible diagnosis of IgG4-RD from various hospital departments are studied from January 2010 to August 2022. Some exclusion criteria were applied (patients with clinical manifestations attributable to other diseases were excluded) and to those who remained with a suspected diagnosis of IgG4-RD, the Umehara-Okazaki 2011 and ACR/EULAR 2019 criteria were applied. 182 patients with elevated IgG4 and/or a suspected diagnosis of IgG4-RD were collected. Finally, after exclusion criteria, 22 possible patients with IgG4-RD remained (Table 1). The diagnostic criteria proposed by Umehara and Okazaki in 2011 are applied to these patients, including a total of 13 patients, with a mean age of 60 years, 57% women; 5 being classified with definitive disease, 3 as probable disease and 5 as possible disease. Finally, we applied the ACR/EULAR 2019 classification criteria to those patients too, establishing diagnosis in 7 patients. The mean age was 57 years, 71% were women with a mean follow-up of 5.3 years; 85.71% of the patients had elevated IgG4, with mean levels of 176.3 mg/dL. Retroperitoneal fibrosis and aortitis was the most prevalent presentation in both groups (2011 and 2019 criteria) with 38.5% and 28.6% respectively. IgG4-RD is a recently described entity that is very heterogeneous in terms of its clinical, analytical and histopathological presentation. According to our series, clinical heterogeneity is the rule, being the retroperitoneal fibrosis and aortitis the most frequent. There are differences when we use the different criteria. ACR/EULAR 2019 classification criteria are stricter criteria that allows us to classify patients more accurately. In our series 6 patients fulfilled Umehara-Okazaki criteria but not ACR/EULAR due to giving more importance to histopathology, clinical manifestations and the need to reach a minimum score in which, for example, only IgG4 levels are not enough. Therefore, on many occasions, a multidisciplinary approach with experienced teams is necessary. [1]Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol. 2020 Jan;72(1):7-19. [2]Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012 Feb;22(1):21-30. NIL. None Declared. [Display omitted] Table 1High diagnostic IgG4-RD group, Umehara and Okazaki 2011 and ACR/EULAR 2019 IgG4-RD criteria.High diagnostic suspition of IgG4-RDn=22Umehara-Okazaki criteria 2011n=13ACR/EULAR criteria 2019n=7Age, median (IQR), years62 (34-87)60 (34-82)57 (34-79)Sex, female (%)54,5461,5471,42Follow-up, median (IQR), years5,18 (1-28)4,4 (1-9)5,3 (1-9)Death, n (%)9,097,690IgG4 level, median (IQR), mg/dL152 (32,6- >194)163,71 (56,6 ->194)176,3 (109- >194)Elevated IgG4, n (%)63,6384,6285,71Normal IgG4, n (%)13,637,690No evaluated IgG4, n (%)22,727,6914,29CRP, median (IQR), mg/dL3,33 (0-10,7)3 (0,1 -10,7)6 (0,39 -7,8)ESR, median (IQR), mm/h32,89 (7-120)42 (10 -120)48,4 (16-120)Available biopsy at IgG4 involvement site (%)40,9169,23100Clinical phenotypesPancreatohepatobiliary, n (%)18,1815,3814,28Retroperitoneum and aorta, n (%)40,9138,4628,57Head and neck limited, n (%)4,557,690Mickulicz and systemic, n (%)4,557,6914,28Undefined phenotype, n (%)31,8230,7742,86Initial corticotherapy, n (%)90,9184,6185,71

Background:Immunotherapy in cancer is a therapeutic strategy based on stimulating the immune system to recognize and destroy tumor cells. Among immunotherapy, therapies targeting molecular processes occurring in tumor cells stand out. Although these therapies have been a significant advance in the fight against cancer, they can also trigger various immune-mediated adverse effects, some of rheumatological nature.Objectives:To describe the rheumatological manifestations in cancer patients receiving targeted therapies.Methods:Multicenter, retrospective, and descriptive study, including patients derivated from oncology related to immunotherapy treatment between January 2020 and December 2023. Demographic and clinical variables were collected. An immune-mediated effect was considered severe if it endangered the patient’s life and/or led to hospitalization, disability, or death. Statistical analysis was performed using R4.3.2.Results:56 patients were included. The majority were men (60.7%), with an average age of 65.56 ± 12.56 years. The most frequent comorbidities were hypertension (47.4%), dyslipidemia (44.7%), and type 2 diabetes (28.9%). The immunotherapy treatments received were PD-1 inhibitors: Pembrolizumab (48.2%) and Nivolumab (41.1%) and PDL-1 inhibitors: Atezolizumab (7.1%) and Durvalumab (3.6%). The most common types of neoplasia were lung (51.8%), followed by melanoma (16.1%), renal (10.7%), and digestive tract (7.14%). Other less frequent tumor types included bladder (3.6%), ear-nose-throat area (3.6%), hematological (3.6%), mesothelioma (1.79%), and cervix and lung (1.79%). The most common reasons for referral were arthralgias (77.8%), arthritis (55.6%), and suspected autoimmune disease (37%). After follow-up, the most common diagnosis was “inflammatory arthralgia/polyarthritis” in 51.2% of patients. Other newly diagnosed pathologies were: Sjögren (n=4), RA (n=4), PsA (n=2), lupus-like (n=2), PMR (n=1), myositis (n=1), antisynthetase syndrome (n=1), gout (n=1), scleroderma (n=1), and Behçet’s (n=1). There were 3 patients with a previous diagnosis of Rheumatoid Arthritis and 2 with Systemic Lupus Erythematosus who had a flare, one of them severe, after the start of immunotherapy. In 96.2% of cases, the diagnoses were attributed to treatment with targeted therapies. The average time between tumor diagnosis and symptom onset was 114.45 ± 147.72 weeks, while the time between treatment initiation and symptom onset was 25.35 ± 31.32 weeks (Figure 1). Regarding the treatment received, 65.5% received corticosteroids as indicated by Rheumatology, with an average prednisone dose in the last 6 months of 5.86 ± 7.18 mg. 27.3% received infiltrations, 34.5% DMARDs (23.6% methotrexate and 10.9% hydroxychloroquine), and only 2 patients (3.6%) biologics. In 7 patients (12.7%), immunotherapy had to be withdrawn. 48% died with an average time from the start of immunotherapy to death of 153.14 ± 96.61 weeks.Conclusion:Most rheumatological manifestations are derived from treatment with PD-1 inhibitors, with symptoms appearing early after treatment initiation. A high percentage of patients respond to corticosteroids, although some require DMARDs, biologics, or even discontinuation of immunotherapy due to severe manifestations.Figure 1.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.