tbuckley/cabot-search huggingface dataset:
AB1494 CLINICAL ASSOCIATIONS OF ANTI-RO52 ANTIBODIES DETECTED IN 150 PATIENTS WITH SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASES FROM A SINGLE REFERRAL CENTRE
Authors: M. Renuncio-García, D. Prieto-Peña, C. Secada-Gomez, J. Irure-Ventura, M. López Hoyos, R. Blanco
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Anti-SSA/Ro autoantibodies (aAbs) can target Ro60 and Ro52 antigens. The presence of anti-Ro60 aAbs has been widely described in patients with systemic autoimmune rheumatic diseases (SARDs). Nevertheless, the clinical implication of anti-Ro52 aAbs for the diagnosis and management of SARDs is still unclear. <h3>Objectives:</h3> To assess the possible clinical associations of anti-Ro52 antibodies in patients with SARDs. <h3>Methods:</h3> Between November 2017 and November 2023, a total of 150 patients in our hospital tested positive for anti-Ro52 aAbs, and their clinical features were collected. Patients were divided into 2 groups: 1) isolated anti-Ro52 + 2) antiRo52+ with other aAbs. A comparative study between groups was performed. <h3>Results:</h3> 150 patients (115 women/35 men; mean age 62.47±13.73 years) with anti-Ro52+ aAbs were identified. Final diagnosis were: anti-synthetase/overlap myositis (n=42), undifferentiated connective tissue disease (UCTD) (n=18), Sjögren's syndrome (n=18), other systemic inflammatory diseases (n=16), scleroderma (n=11), interstitial pneumonia with autoimmune features (IPAF) (n=10), systemic lupus erythematosus (n=5) and dermatomyositis (n=5). In 25 (16.66%) patients the diagnosis of inflammatory diseases was finally ruled out. 102 patients were classified in the isolated Ro52+ group and 48 in the Ro52+ with other aAbs. Isolated Ro52+ aAbs were more frequently associated with malignancy (p=0.04), while Raynaud's phenomenon was more commonly found in patients with anti-Ro52+ and other aAbs+ (p=0.02) (Table 1). Differences in diagnoses between both groups as well as the specificities combined with anti-Ro52 are shown (Figure 1). <h3>Conclusion:</h3> Anti-Ro52 aAbs determination has clinical implications in the diagnosis of SARDs. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0219 UNDIFFERENTIATED ARTHRITIS: HOW DOES IT PROGRESS?
Authors: O. Jomaa, S. Abdellatif, M. Brahem, M. Ardhaoui, H. Hachfi, M. Younes
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Undifferentiated arthritis is a frequently occurring clinical presentation with a variable outcome. While some forms of undifferentiated arthritis will spontaneously remit, other forms will progress to chronic arthritis. <h3>Objectives:</h3> The aim of our study was to investigate the different clinical, biological, and evolutionary characteristic of patients presenting an undifferentiated arthritis. <h3>Methods:</h3> This is a retrospective descriptive study of the records of patients labelled with undifferentiated arthritis over a period of 15 years (2005-2019) and followed up in a rheumatology department. Epidemiological, clinical, para-clinical and evolutionary data were collected and analyzed. <h3>Results:</h3> Seventy patients were included; 53 women and 17 men with a sex ratio of 1.9. The mean age was 50 years [19-84]. The mean age of onset of symptoms was 43 years [12-82], and the mean time to consultation was 22 months, with extremes ranging from 1 day to 20 years. The most common comorbidities, in decreasing order of frequency, were diabetes (15.7%), hypertension (11.4%), dyslipidemia (10%) and osteoporosis (8.7%). Four patients (5.7%) had a family history of chronic inflammatory rheumatism. Oligoarthritis was the most common mode of onset (47.1%). Monoarticular and polyarticular onset were noted in 27.1% and 25.7% of patients respectively. Extra-articular manifestations were present in 35.7% of patients, dominated by ophthalmological signs (dry eye syndrome) in 30% of cases, skin signs were noted in 8.6% of patients, and renal involvement in only one case. The most common laboratory abnormalities were anemia (45.7%), hyperleukocytosis (27.1%) and lymphopenia (2.9%). A biological inflammatory syndrome was observed in the majority of patients (82.9%). On immunological tests, 5 patients had positive anti-cyclic citrullinated peptide antibodies, 6 had positive rheumatoid factor antibodies and 8 patients had positive antinuclear antibodies. Radiological damage was present in 18.6% of patients, mainly geodes (10%) and joint space narrowing (11.4%). The course was marked by progression to rheumatoid arthritis in 8 cases (11.4%), spondyloarthritis in 2 cases (2.9%), connective tissue disease in 2 cases (2.9%), and rheumatic fever in one case. However, the arthritis remained undifferentiated in the majority of cases (81.5%). <h3>Conclusion:</h3> Undifferentiated arthritis is a moving entity, requiring rigorous monitoring to confirm the diagnosis and introduce early, targeted treatment. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0261 INTERSTITIAL LUNG DISEASE ASSOCIATED WITH INFLAMMATORY MYOSITIS: FOLLOW-UP, TREATMENT AND MORTALITY
Authors: E. Cimen Gunes, E. Tekgoz, S. Colak, M. Çinar, S. Yilmaz
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Inflammatory myositis is a connective tissue disorder (CTD) primarily characterized by muscle inflammation, with potential involvement of extramuscular manifestations such as the skin, lungs, heart, and gastrointestinal tract. <h3>Objectives:</h3> To assess the follow-up, treatment, and factors contributing to mortality in patients with ILD associated with inflammatory myositis. <h3>Methods:</h3> This study included patients with ILD associated with CTD, who were enrolled in the GUR-ILD database from January 2016 to June 2023. The demographic and clinical data of the patient group diagnosed with inflammatory myositis were retrospectively analyzed and recorded from the GUR-ILD registry. <h3>Results:</h3> A total of 190 patients were included in the GUR-ILD cohort. In this cohort group, 9 (4.7%) patients were diagnosed with inflammatory myositis. The median age of the patients (4 males/5 females) with inflammatory myositis was 64.4 (47.0-79.0) years, and disease duration was 15 (1-202) months. Four patients were diagnosed with inflammatory myositis before ILD, while 5 patients were diagnosed with inflammatory myositis and ILD concurrently. The presenting complaint in all patients was cough and dyspnea. In the evaluation of pulmonary function tests (PFTs), forced vital capacity (FVC) was found to be below 70 ml in 3 patients. Computed tomography (CT) findings revealed nonspecific interstitial pneumonia (NSIP) in 8 patients (88.9%) and usual interstitial pneumonia (UIP) in one patient (11.1%). The prevalence of ILD findings on CT images was 20% or less in 6 (66.7%) patients and above 20% in 3 (33.3%) patients at the time of diagnosis. All patients received at least one immunosuppressive (IS) treatment for ILD. Three patients were treated with azathioprine, 2 with rituximab and cyclosporine, 5 with mycophenolate mofetil, one with tocilizumab and one with intravenous immunoglobulin. During the follow-up, nintedanib was added as antifibrotic therapy for one patient. In addition to IS treatment, all patients frequently received prednisolone at a dosage ranging from 7.5 to 30 mg per day. Jo-1 antibody was positive in 4 patients, MDA-5 antibody was positive in 2 patients, while PM-SCL and SRP antibodies were positive in one patient each. During the follow-up period, 3 patients died. Among them, 2 patients with positive MDA-5 antibodies died due to disease activation and respiratory failure resulting from PCP (pneumocystis carinii pneumonia) infection (Table 1). <h3>Conclusion:</h3> While the utilization of IS therapies at different stages enhances our treatment approach, it's crucial to note that MDA-5 antibody positivity remains a significant factor contributing to swift and progressive lung involvement, leading to increased mortality. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0254 EXPLORING GENDER DIFFERENCES IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOSITIS: DATA FROM A MONOCENTRIC ITALIAN COHORT
Authors: E. Cela, E. Capparelli, A. D’antonio, E. Greco, S. Modica, A. Bergamini, B. Kroegler, M. S. Chimenti, P. Triggianese
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Idiopathic Inflammatory Myositis (IIM) is a rare autoimmune systemic disease characterized by a wide spectrum of clinical manifestations mainly involving skin and muscles. Few data are available on literature exploring the gender differences in IIM patients. <h3>Objectives:</h3> To analyze the gender difference in IIM patients in clinical and autoantibody profiles as well as treatment strategies. <h3>Methods:</h3> We performed a cross sectional observational study including patients who fulfilled ACR/EULAR 2017 criteria for Dermatomyositis (DM), Polymyositis (PM), and Antisynthetasis Syndrome (ASS) (time frame Jan 2018-December 2023), referring to the Reference Center of "Tor Vergata" University Hospital in Rome (Italy). Demographic data, clinical manifestations, and treatment approaches were recorded. Additionally, an assessment of the antibody profile, encompassing Myositis Specific Antibodies (MSA) and Myositis Associated Antibodies (MAA), was conducted. Continuous variables were compared with T test, categorical variables by using Chi-square or Fisher's exact test. P&lt;0.05 values were considered statistically significant. <h3>Results:</h3> The study cohort included 51 patients (68.8% F). Median age at disease onset and diagnostic delay were similar in males and females, while disease duration was significantly longer in males (p 0.018) (Table 1). The diagnosis of DM and PM was equally distributed, while ASS was slightly prevalent in men. Accordingly, lung involvement occurred more frequently in males than females (Figure 1). No significant differences emerged between the groups according to comorbidities (Table 1). Joint involvement as well as upper limb muscle weakness affected more frequently female patients (p&lt;0.05, Figure 1). A similar prevalence of ANA titer ≥ 1:160 resulted among males and females as well as for the distribution of MSA and MAA. Dual positivity of MSA was observed in one male (anti-Mi-2/anti-Pl7) and two females (anti-MDA5/anti-NXP2 and anti-MDA5/anti-EJ antibodies). Mycophenolate Mofetil (p&lt;0.001) and Rituximab (p&lt;0.05) were administrated with a higher prevalence in male patients than females. <h3>Conclusion:</h3> Our study documents for the first time gender differences in IIM, highlighting a different distribution of lung and joint involvement being prevalent mainly in males and females, respectively. Differences in clinical phenotypes, in the absence of a gender-related prevalence of specific autoantibodies, might affect treatment strategies. Our findings support the idea for a gender orientated approach which is crucial to improve decision making process in clinical practice. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0253 MULTIMODAL ULTRASOUND OF MUSCLE: A PROMISING BIOMARKER FOR IMMUNE-MEDIATED NECROTIZING MYOPATHY
Authors: J. Zhu, Y. Xin
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Immune-mediated necrotizing myopathy (IMNM) [1] is characterized by acute or subacute onset, symmetric distribution, and proximal muscle weakness, with extremely high muscle enzyme serum levels and muscle histopathology showing necrosis or regeneration. Early diagnosis and monitoring disease activity are essential in the clinical management of patients with IMNM. Muscle ultrasound may offer a promising and cost-effective evaluation tool. <h3>Objectives:</h3> To compare muscle ultrasound parameters (including conventional ultrasound and shear wave elastography) in patients with IMNM and healthy controls and analyze the correlation with disease activity. <h3>Methods:</h3> 15 patients with IMNM were recruited prospectively from the rheumatology outpatient clinic, and 20 healthy control cohorts were matched based on age and sex. They all underwent muscle ultrasound of the bilateral right rectus femoris (RF) in axial and longitudinal scans and ultrasound parameters were measured. Spearman's test was used to identify the correlations between ultrasound and disease activity measures. <h3>Results:</h3> The mean age at diagnosis of patients with IMNM was 50.73 (±9.83) years, with a median disease duration of 13 months, of which 66.67% were females. The control participants were close in age and sex. By ultrasound, patients with IMNM had an increased echogenicity of RF compared with healthy controls [mean pixel intensity of 69.88±4.21 in IMNM vs 66.18±2.33 in controls (<i>P</i> = 0.006)]. No significant differences between IMNM and healthy controls were found in fascia thickness, muscle thickness and power Doppler score (all <i>P</i> &gt; 0.05). Shear wave elastography (SWE) measurements (shear wave speed and shear wave dispersion slope) exhibits good repeatability and stability on longitudinal section. The results shows that shear wave speed (1.89 vs 1.97 m/s, <i>P</i> = 0.028) and dispersion slope (8.85 vs. 10.54 [m/s]/kHz, <i>P</i> = 0.002) of IMNM patients were lower than that of the controls (Figure 1). As shown in Table 1, there was a significant correlation between the two SWE parameters and the serum enzyme indices and MMT. A negative correlation trend for an association between SWE and PGA VAS and MDAAT did not reach statistical significance (<i>P</i> &gt; 0.05). <h3>Conclusion:</h3> Multimodal ultrasound of muscle may be a valuable imaging method for early detection of muscle involvement and evaluation of IMNM disease activity. <h3>REFERENCES:</h3> [1] Allenbach Y, Benveniste O, Stenzel W, Boyer O. Immune-mediated necrotizing myopathy: clinical features and pathogenesis. Nat Rev Rheumatol. 2020,16(12):689-701. <h3>Acknowledgements:</h3> This study was supported by the National Natural Science Foundation of China (No. 82071930). <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0279 A HIGH PROPORTION OF NORWEGIAN WOMEN WITH JUVENILE IDIOPATHIC ARTHRITIS (JIA) ARE BREASTFEEDING
Authors: T. Birkeland, Å. Størdal, C. Götestam Skorpen, M. Wallenius
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Juvenile idiopathic arthritis (JIA) represents challenges for women during pregnancy and breastfeeding. Limited research exists on breastfeeding practices among women with JIA. To our knowledge, there are no publications so far investigating the proportion of breastfeeding in mothers with JIA, and if breastfeeding affects disease related factors. <h3>Objectives:</h3> This study aimed to explore the proportion of women with JIA breastfeeding at 6 weeks, 6 months and 12 months postpartum, as well as examining demographic and disease related factors, and the use of medications, comparing the breastfeeding and the non-breastfeeding groups. <h3>Methods:</h3> Data from the Norwegian nationwide quality register RevNatus, collecting data about women with inflammatory rheumatic diseases in relation to pregnancy, were analyzed. The data were collected from clinical documentation and self-reported material during visits at the outpatient clinic. All women with JIA with a live birth and attending at least the control 6 weeks postpartum, were included in the study. <h3>Results:</h3> Among 304 live births in 227 women, 85,5% were breastfed at 6 weeks, 70% at 6 months and 30% at 12 months postpartum. Breastfeeding women had higher educational level, longer pregnancy duration, a lower prevalence of C-sections, lower scores for VAS pain, VAS fatigue and VAS total, and used less conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) compared to women not breastfeeding. 14 women did not specify their breastfeeding status. <h3>Conclusion:</h3> In the present study, we observed a high proportion of women with JIA breastfeeding at 6 weeks and 6 months postpartum. Based on our findings, women with JIA should be encouraged by health professionals to breastfeed. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
AB0205 HYPERURICEMIA’S ASSOCIATION WITH LEFT ATRIUM DIAMETER AND RELATIVE WALL THICKNESS IN INFLAMMATORY RHEUMATIC DISEASES
Authors: A. L. Guajardo-Aldaco, M. F. Elizondo-Benitez, D. A. Galarza-Delgado, I. J. Colunga-Pedraza, J. R. Azpiri-Lopez, J. A. Cardenas-de la Garza, R. I. Arvizu-Rivera, V. González-González
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> An increase in left atrial diameter (LAD) and a greater relative wall thickness (RWT) of the left ventricle (LV) has been associated with a greater risk of suffering cardiovascular disease (CVD). It has been shown that hyperuricemia can lead to an increase in LAD and greater RWT in healthy adults, but its effect in patients with inflammatory rheumatic disease has not been established. <h3>Objectives:</h3> Compare the LAD and RWT in patients diagnosed with inflammatory rheumatic disease with and without hyperuricemia. <h3>Methods:</h3> A cross-sectional study was carried out, including patients aged 40 to 75 years who met the classification criteria for ACR/EULAR 2010 for Rheumatoid Arthritis (RA) and the Classification Criteria for Psoriatic Arthritis (CASPAR) 2006 for Psoriatic Arthritis. Patients with a history of cardiovascular events, overlap syndromes, or pregnancy were excluded. Biochemical profiles were performed to calculate serum uric acid (SUA) levels. Hyperuricemia was considered as a SUA level &gt; 6.8 mg/dL. Transthoracic echocardiograms were performed by a board-certified cardiologist, blinded to clinical information. The distribution was evaluated using the Kolmogorov-Smirnov test, comparisons were made with the Chi-square test, Fisher's exact test, Student's T-test, or u-Mann Whitney test, depending on the case. A <i>p</i>-value &lt; 0.05 was taken as statistically significant. <h3>Results:</h3> Our study included 32 patients, mainly men (62.5%). The average age was 53.75 (±7.76). The most frequent diagnosis was PsA with a frequency of 65.6%. It was found that patients with hyperuricemia showed larger LAD than patients without hyperuricemia [4.05 vs. 3.45, p=0.003]. Patients with hyperuricemia showed higher RWT compared to patients without hyperuricemia [0.47 vs. 0.39, p=0.000]. Patients with hyperuricemia also had higher prevalences of hypertension [9 (64.3) vs. 8 (44.4); p=0.021] with no significant differences in prevalence for other comorbidities. (Table 1) <h3>Conclusion:</h3> In our cohort, LAD and left ventricular RWT were elevated in patients with inflammatory rheumatic disease and hyperuricemia. Complete cardiovascular evaluations are recommended in patients with autoimmune disease and hyperuricemia. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>
POS0366 B-CELLS DEPLETION AFTER TREATMENT WITH RITUXIMAB PREDICTS RELAPSE OF IgG4-RELATED DISEASE
Authors: M. Lanzillotta, G. A. Ramirez, R. Milani, L. Dagna, E. Della Torre
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> B-cell depletion therapy with rituximab is effective in most patients with IgG4-related disease (IgG4-RD) but requires repeated cycles to prevent disease flares leading to prolonged immuno-suppression posing patients at high rik of infectious complications. <h3>Objectives:</h3> We here aimed to assess B cells after rituximab to predict relapse of IgG4-RD and guide retreatment. <h3>Methods:</h3> Patients with active IgG4-RD included in this retrospective study fulfilled the ACR/EULAR Classification Criteria. Total CD19+ B cells, plasmablasts, naïve and memory B cells were measured on peripheral blood by flow-cytometry at baseline and six months after rituximab. All patients were treated with two 1gr infusions of rituximab 15 days apart and monitored for 48 months. Disease response was assessed using the IgG4-RD Responder Index <h3>Results:</h3> Thirty-three patients were included. Six months after rituximab, disease response was observed in all patients. Complete depletion of CD19+ B cells, plasmablasts, naïve and memory B-cell depletion was achieved in 30%, 55%, 39%, and 42% of cases, respectively. Twenty-three relapses (70%) were observed at a median time of 24 months after rituximab. Relapse rate was significantly higher in patients who failed to achieve complete depletion of CD19+ cells (60% vs 17%, p=0.02), naïve B cells (54% vs 15%, p=0.01), or memory B cells (50% vs 16%, p=0.03) six months after rituximab. The median relapse free survival time was shorter in patients who failed to achieve complete depletion of CD19+ cells (19 vs 38 months, p=0.02), naïve B cells (16 vs 38 months, p=0.01), or memory B cells (19 vs 38 months, p=0.03) six months after rituximab (Figure 1). <h3>Conclusion:</h3> The degree of B-cell depletion six months after rituximab may predict disease flare and may instruct on the pacing of B-cell depletion therapy in IgG4-RD. <h3>REFERENCES:</h3> [1] Lanzillotta M, BMJ 2020 Advances in the management of IgG4-related disease. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>