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Background Both cardiovascular and complement-mediated disorders might lead to microvascular damages in anti-neutrophil cytoplasm autoantibodies (ANCA)-associated vasculitides (AAV). We aimed at investigating, for the first time, subclinical microvascular abnormalities with non-invasive techniques in AAV patients by analyzing both retinal and nailfold capillary changes. Retinal plexi were investigated using optical coherence tomography angiography (OCT-A), while nailfold capillary changes by video-capillaroscopy (NVC). Potential correlations between microvessels’ abnormalities and disease damage were also explored. Methods An observational study was conducted on consecutive patients who met the inclusion criteria of defined diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA), age ≥ 18 ≤ 75 yrs, and no ophthalmological disorders. Disease activity was assessed by Birmingham Vasculitis Activity Score (BVAS), damage by Vasculitis Damage Index (VDI), and poorer prognosis by the Five Factor Score (FFS). Quantitative analysis of vessel density (VD) was performed by OCT-A in both superficial and deep capillary plexi. Figures and detailed analysis from NVC were performed for all subjects in the study. Results Included AAV patients (n = 23) were compared with 20 age/sex-matched healthy controls (HC). Retinal VD in superficial whole and parafoveal plexi resulted significantly decreased in AAV compared to HC (P = 0.02 and P = 0.01, respectively). Furthermore, deep whole and parafoveal vessel density was strongly reduced in AAV than HC (P ≤ 0.0001 for both). In AAV patients, significant inverse correlations occurred between VDI and OCTA-VD in both superficial (parafoveal, P = 0.03) and deep plexi (whole, P = 0.003, and parafoveal P = 0.02). Non-specific NVC pattern abnormalities occurred in 82% of AAV patients with a similar prevalence (75%) in HC. In AAV, common abnormalities were edema and tortuosity in a comparable distribution with HC. Correlations between NVC changes and OCT-A abnormalities have not been described. Conclusion Subclinical microvascular retinal changes occur in patients with AAV and correlate with the disease-related damage. In this context, the OCT-A can represent a useful tool in the early detection of vascular damage. AAV patients present microvascular abnormalities at NVC, whose clinical relevance requires further studies.

Background: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.Objectives:To analyze the gender difference in IIM patients in clinical and autoantibody profiles as well as treatment strategies.Methods: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<0.05 values were considered statistically significant.Results: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<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<0.001) and Rituximab (p<0.05) were administrated with a higher prevalence in male patients than females.Conclusion: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.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Primary antiphospholipid syndrome (pAPS) is a rare systemic autoimmune disease characterized by arterial, venous, or microvascular thrombosis, pregnancy morbidity, or no thrombotic manifestations in patients with persistent antiphospholipid antibodies (aPL). Migraine headaches are one of the commonest complaints in pAPS patients. Evidence reports non-specific capillary abnormalities in Nailfold capillaroscopy (NVC) from pAPS patients, and few previous investigations had shown debated abnormalities of blood flow at NVC study in patients with migraine without APS.Objectives:Aims of this prospective study involving pAPS patients were to evaluate NVC abnormalities and their potential correlations with headache-related disability.Methods:We performed a prospective study including patients fulfilling the following inclusion criteria: 1.a defined diagnosis of pAPS according to 2023 ACR/EULAR APS classification criteria; 2. age>18 y.o.; 3. no other connective tissue disease; 4. clinical diagnosis of migraine, assessed by an expert Neurologist. All patients referred to APS Reference Center at Rheumatology Unit of Tor Vergata University (Rome, Italy). Patients underwent clinical and laboratory assessment, NVC, and migraine headache tests including HIT-6 and MIDAS questionnaires (assessing headache-related disability) and PSQI test evaluating sleep quality. A moderate-severe level of disability was defined as MIDAS score ≥11, HIT-6 score ≥50, while a PSQI score ≥5 indicated a poor sleep quality. We also included healthy controls (HC). Continuous variables were compared with T test, categorical variables by using Chi-square or Fisher’s exact test. Linear logistic regression analysis was used to evaluate the adjusted risk of headache disability related to NVC variables. P<0.05 values were significant.Results:The study cohort included 30 patients affected by pAPS and migraine, and 28 age and sex-matched HC Table 1). Thrombotic events as well as antiphospholipid antibodies (aPL) occurred only in pAPS group (Table 1). Raynaud phenomenon showed a trend to be more frequent in pAPS than in HC (Table 1). Patients with pAPS and migraine had microhemorrhages and tortuosity at NVC more frequently than HC (p=0.02 and p<0.05, respectively). Moreover, patients with pAPS showed a greater level of headache-related disability, as described by abnormal values of MIDAS and HIT-6 score than HC, while PSQI was similar between patients and HC (Table 1). Among pAPS patients, no differences in MIDAS, HIT-6, and PSQI scores occurred in accordance with the type of aPL positivity neither with the concomitant treatments (not shown). Linear logistic regression showed that a HIT-6 score ≥50 significantly correlated with slow capillary flow at NVC studies (OR 7.7, p= 0.03) in pAPS patients.Conclusion:Headache-related disability could be relevant in pAPS patients in whom NVC abnormalities, mainly microhemorrhages and tortuosity, appear to be present in a higher percentage than in controls. Slow capillary flow, documented at NVC, might be strongly associated with headache-disability in pAPS patients.Table 1. Clinical, laboratory features, NVC findings, and headache scores from the study cohort.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Inflammatory Idiopathic Myositis (IIM) represents a specific group of rare rheumatologic diseases mainly characterized by skin and musculoskeletal involvement. However, interstitial lung disease (ILD) constitutes a key disease target in IIM, strongly affecting outcome and treatments.Objectives:We aimed at evaluating distribution and pattern of ILD in a selected cohort of IIM patients. Potential association between ILD and both the disease phenotype and the autoimmunity profile have been explored.Methods:We performed a cross sectional observational study including patients who fulfilled 2017 ACR/EULAR 2017 criteria for Dermatomyositis (DM), Polymyositis (PM), and Antisynthetasis Syndrome (ASS) (time frame June 2023-December 2023) referring to the Reference Centre of “Tor Vergata” University Hospital in Rome (Italy). Demographical data, including clinical and laboratory records, were collected. Concomitant ILD was defined based on chest computed tomography (chest CT) images: included patients were divided in two groups according to the presence of ILD. Continuous variables were compared with T test, categorical variables by using Chi-square or Fisher’s exact test. P<0.05 values were considered statistically significant.Results:The study cohort included 51 patients (68.6% women). Patients’ characteristics were summarized in Table 1. Patients with ILD were 51% (n=26; Group 1) while patients without were 49% (n=25 Group 2). Patients from Group 1 were older than those from group 2 at IIM diagnosis (p=0.04). ASS diagnosis resulted prevalent in Group 1 than Group 2 (p=0.01), while DM was significantly more frequent in Group 2 p=0.001). Patients in Group 1 showed a higher prevalence of dyspnoea (p<0.0001), arthralgias p=0.006), and arthritis (p=0.04) than those in Group 2. A significantly higher prevalence of Gottron papules and periorbital oedema has been registered in Group 2 than Group 1 (p<0.05). A diagnosis of malignancy occurred mainly in patients from Group 1 (p=0.02). Autoantibody profile resulted similar between two groups, excepted for anti-Jo1 patients who were mainly present in Group 1 (p=0.01) (Graphic 1).Conclusion:Our real-life data support the relevant association between concomitant ILD and specific IIM clinical pattern resulting mainly associated with joint involvement and IIM complicated by malignancy. Autoantibody profile might help in IIM patients’ stratification by potentially predicting ILD. Focusing on lung involvement in IIM represents a key tool to improve disease management and treatment strategies.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:In systemic sclerosis (SSc), primary heart involvement (pSHI) is a major cause of related death. In most cases, pSHI is subclinical especially in early stages of disease. pSHI is related to both fibrosis and coronary lesions potentially leading to chronic heart failure (CHF). Coronary computed tomography angiography (CCTA) is a non-invasive imaging modality with high sensitivity for the detection of coronary lesions. The early diagnosis of cardiac involvement in SSc is a key challenge for the improvement of both damage and burden of the disease.Objectives:The study aimed at exploring for the first time the potential role of CCTA in the early detection of subclinical cardiac involvement in SSc patients.Methods:A prospective cohort study included patients fulfilling the following inclusion criteria: 1. a defined diagnosis of SSc according to 2013 ACR/EULAR classification criteria; 2. age>18 y.o.; 3. no history of cardiovascular disease (including severe hypertension and CHF); 4. consent to study. All included subjects referred to SSc Reference Center of the Rheumatology Unit (“Tor Vergata”, Rome, Italy) between March 2023 and December 2023. Patients underwent clinical and laboratory evaluation for traditional cardiovascular risk factors and SSc assessment, nailfold video capillaroscopy (NVC), a color doppler trans-thoracic echocardiogram, and CCTA. Detailed analysis from NVC was performed by an expert Rheumatologist. The CT protocol foresaw a low dose basal acquisition on thoracic volume to evaluate pulmonary interstitium, followed by a cardio-synchronized CT angiography acquisition to assess coronary arteries.Results:The study cohort included 13 patients (92.3% women) with a mean age of 68.6 ± 7.9 y.o. Demographic and clinical data were reported in Table 1. The limited cutaneous SSc (lcSSc) represented 61.5% of the cohort while the remaining was the diffuse cutaneous (dcSSc). In accordance with CCTA findings, patients were divided in non-significant coronary abnormalities (group 1) and luminal severe stenosis (group 2). Group 2 included 30.7% of the cohort (n=4) and required subsequent coronary interventions in all cases. The dcSSc phenotype was prevalent in group 2 than in group 1 (p<0.05) as well as the concomitant interstitial lung disease (ILD, p=0.02). Patients from group 2 showed active-late NVC pattern in a higher percentage than those from group 1 (p=0.03).Conclusion:Preliminary findings from our pilot proof-of-concept study for the first time provide evidence of severe coronary lesions in asymptomatic SSc patients. These vascular changes revealed by CCTA appear to be associated with dcSSc phenotype and concomitant ILD and could precede CHF. CCTA findings might improve SSc management by an early detection of subclinical damage and a tailored risk stratification for the disease outcome.Table 1.Demographic, clinical, and laboratory data from patients with systemic sclerosis (SSc) with non-significant coronary abnormalities (group 1) and luminal severe stenosis (group 2) detected by using coronary computed tomography angiography.Group 1N=9Group 2N=4Diffuse cutaneous, N (%)2 (22.2)3 (75)Age, mean ± SD70.6±5.668.6±7.9Smoking habits, N (%)4 (44.4)2 (50)Body mass index, mean ± SD24±524.1±4.6Cholesterol HDL, mean ± SD61.9±15.760.2±15.2Cholesterol total, mean ± SD200.2±46205±47.9Brain natriuretic peptide, mean ± SD106.1±134.597.7±136.9Disease duration, mean ± SD23.9±15.326.9±16.1Raynaud phenomenon, N (%)9 (100)4 (100)Interstitial lung disease, N (%)2 (22.2)3 (75)Pulmonary arterial hypertension, N (%)3 (33.3)1 (25)Figure 1.A representative scan of coronary computed tomography angiography from a female patient with systemic sclerosis shows a significant stenosis resulting in positive remodelling of the proximal segment of the left anterior descending artery.REFERENCES: NIL. Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Mixed connective tissue disease (MCTD) is a rare complex syndrome with features of different autoimmune connective tissue diseases (CTDs) in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In MCTD, immune-system abnormalities and, thus, fibrosis may involve skin and other internal organs, including lungs, also leading to diffuse microangiopathy. Lung involvement in terms of interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are key manifestations strongly affecting morbidity and mortality in MCTD.Objectives:MCTD patients’ phenotypic pattern were analyzed according to the presence of concomitant lung involvement to explore potential association with microvascular damage.Methods:We performed a cross sectional observational study including patients with a defined diagnosis of MCTD, referring to the Reference Centre of “Tor Vergata” University Hospital in Rome (Italy). Recorded data included clinical and laboratory findings. Nail fold videocapillaroscopy (NVC) patterns and trans-thoracic echocardiographic PAPs value (PAPs>25 mmHg defined PAH) were analyzed. Concomitant ILD was defined based on chest computed tomography (chest CT) features: patients were thus divided in accordance with the presence of ILD. Continuous variables were compared with T test, categorical variables by using Chi-square or Fisher’s exact test. P<0.05 values were considered statistically significant.Results:The study cohort included 24 patients (95.8% women). Patients’ characteristics were shown in [Table 1]. Patients with ILD were 37.5% (n=9; Group 1) while patients without were 63.5% (n=15 Group 2). Disease duration was greater in Group 1 than in Group 2 (p=0.02). PAH has been revealed in a significantly higher percentage in Group 1 than Group 2 (P<0.01). Patients from Group 1 also exhibited a higher prevalence of telangiectasias, digital ulcers, pitting scars, and serositis than patients from Group 2 (p<0.02 for all the comparisons) along with a NVC late pattern (p<0.01) [Graphic 1]. A higher prevalence of cardiovascular and other respiratory comorbidities was observed in Group 1 than Group 2 (p<0.001 and p<0.05, respectively). A joint involvement showed a trend to be more frequent in Group 2. Patients from Group 1 showed a higher prevalence of both RoSSA-60 and -52 autoantibodies than those from Group 1 (p<0.01). Azathioprine resulted administered more frequently in Group 1 than in Group 2 (p<0.01).Conclusion:Our study documented in MCTD patients complicated with ILD a disease phenotype characterized by a more severe microvascular damage while confirming the role of specific autoantibodies profile in lung disease.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Telomeres are specific regions of repetitive nucleotide sequences that protect chromosome ends and preserve genetic information. In each cell division, telomeres shorten, leading finally to apoptosis and cell cycle arrest when reaching a critical point. The telomere-associated protein (telomeric repeat-binding factor 1 [TERF1]) is essential for the maintenance of telomeres, acting as an inhibitor of telomerase, and its levels correlate with telomere length (TL).Shortened telomeres have been demonstrated in Rheumatoid Arthritis (RA). Furthermore, they are a recognized risk factor for idiopathic pulmonary fibrosis. Few data are present in the literature regarding TL in RA-associated interstitial lung disease (RA-ILD), while no data are present regarding TERF1 in RA and RA-ILD.Objectives:We aimed to evaluate the TL and TERF1 expression levels in RA-ILD.Methods:TL and TERF1 expression levels were evaluated in patients with RA-ILD compared to age-matched RA patients without lung involvement (RA-non-ILD) and healthy controls. Genomic DNA and total RNA were isolated from peripheral blood mononuclear cells. Relative TL was measured using real-time quantitative polymerase chain reaction (qPCR) assay, which quantifies a ratio of telomeric repeat copy signal and a reference single-copy gene (human beta globin) signal. Expression analysis of TERF1 was performed by qPCR assay. T-test was used to compare mean TL and TERF1 expression data among the different phenotypic groups. RA patients were divided according to whether their TL fell within or above the first quartile of the cohort. A multivariate logistic regression analysis was used to correct the p-value for sex, age and disease duration.Results:Eighty-nine RA patients were included (mean age 63.6 ± 13.8 years, median disease duration 9 [IQR 8.4-11.6] years): 42 RA-ILD and 47 RA-non-ILD. 21 age- and sex-matched controls were collected. RA-ILD patients were older and with minor disease duration than RA-non-ILD patients. They exhibited higher disease activity, CRP levels, positivity for RF and ACPA, and were more treated with bDMARDs than RA-non-ILD patients (Table 1). TL in all patients was significantly shorter compared to controls (p = 0.0016). RA-ILD patients presented significantly shorter TL compared to controls (p = 0.00001) and compared to RA-non-ILD (p = 0.0006) (Figure 1A). In the multivariate regression analysis, TL was reduced in RA-ILD compared with RA-non-ILD when adjusted for sex, age and disease duration (p<0.001). After patients stratification according to their TL, it is observed that the prevalence of ILD was significantly higher in patients with short vs normal-length telomeres (82.6% vs 34.8% p=0.00008). In RA-ILD, TL correlated negatively with disease duration (p= 0.007 r= -0.408). TERF1 expression levels were reduced in RA compared with controls (p= 2.17E-17). Both RA-ILD patients and RA-non-ILD patients exhibited reduced TERF1 expression levels than controls (p= 3.37E-10 and p= 2.78E-10, respectively. Figure 1B). TERF1 levels correlated positively with TL (p= 0.004 r= 0.328).Conclusion:Telomere shortening is a feature of immunosenescence and it has been linked to more severe articular disease. Shorter TL and reduced TERF1 expression levels characterize RA. In particular, RA-ILD patients displayed higher disease activity, negative prognostic factors, received more biologic treatments, and exhibited shorten TL than RA-non-ILD patients suggesting that TL might represent a hallmark of a more aggressive disease with lung involvement.Table 1.Figure 1.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:ANCA-associated vasculitides (AAVs) are characterized by small blood vessel inflammation resulting in organ damage. Evidence suggests that positivity to ANCA antibodies could affect clinical phenotype. Pulmonary involvement is frequent and variable among AAV manifestations. Radiological findings, mainly computed tomography (CT) images, represent key tools for the detection of lung involvement, including interstitial lung disease (ILD), nodules, tracheobronchial inflammation, and alveolar hemorrhage. Lung diseases are a relevant factor for the overall outcome and the treatment-related damage in AAV. As previously documented, subclinical microvascular retinal changes occur in AAV patients as a disease-related damage thus correlating with the burden of the disease. Microvascular retinal changes might correlate with ANCA positivity and lung involvement in AAV patients.Objectives:In this study, we aimed to explore potential associations between retinal changes and ANCA positivity in AAV patients. In addition, retinal abnormalities have been analyzed in accordance with lung diseases.Methods:An observational study was conducted on consecutive patients who met the following inclusion criteria: i. a defined diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA); ii. age ≥ 18 ≤ 75 yrs; iii. no ophthalmological disorders. Retinal changes have been documented by Optical coherence tomography angiography (OCT-A) that registered a quantitative analysis of vessel density (VD) in both superficial and deep capillary plexi. Lung diseases was defined as the presence of ILD, nodules, tracheobronchial inflammation, and alveolar hemorrhage by using CT scans. Continuous variables were compared with T test, categorical variables by using Chi-square or Fisher’s exact test. P<0.05 values were considered statistically significant.Results:A total of 25 AAV patients were included. 14 patients (56%) were ANCA positive (79% p-ANCA and 21% c-ANCA). Of the whole cohort, 15 patients (60%) had lung involvement, with ANCA positivity in 80% of them (75% p-ANCA). Data from the study cohort were reported in Table 1. A longer diagnostic delay was observed in ANCA-negative patients than in ANCA-positive (96.9 ± 88 months vs 25 ±36 months, P=0.01). At the OCT-A study, ANCA-negative patients showed a significantly lower foveal vascular density, documented in both the superficial (SFD) and the deep (DFD) scans, compared to ANCA-positive patients (p= 0.007 and p= 0.009, respectively). Among patients with lung involvement, ILD was documented in 33.3% of patients, tracheobronchial inflammation in 33.3%, nodules in 26.7%, and alveolar hemorrhage in a single case. Patients with lung involvement had a lower diagnostic delay than patients without (23 ±37.72 months vs 80.54 ± 80.1 months, p=0.04). Moreover, AAV patients without lung involvement had lower values of both SFD and DFD than patients with lung involvement (p < 0.0001 for both).Conclusion:This is the first OCT-A study in AAV patients highlighting differences in retinal microvascular network between ANCA-negative and ANCA-positive patients as well as potential correlation with concomitant lung diseases. Our preliminary findings support the idea that the presence of ANCA and/or lung involvement in AAV could reduce the diagnostic delay by increasing the index of suspicion. Therefore, the early diagnosis of AAV vasculitis could improve the therapeutic management leading to a reduced retinal vessels damage.REFERENCES: NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

BackgroundBoth cardiovascular and complement-mediated disorders might lead to microvascular damage in anti-neutrophil cytoplasm autoantibodies (ANCA)-associated vasculitides (AAV). No evidence of subclinical microvascular retinal abnormalities neither their potential correlation with capillaroscopy anomalies has been reported in eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA).ObjectivesWe aimed at investigating subclinical microvascular abnormalities by analyzing retinal and nailfold capillary changes in an AAV cohort. Retinal plexi were investigated using optical coherence tomography angiography (OCT-A), while nailfold capillary changes with videocapillaroscopy (NVC). Potential correlations between OCT-A abnormalities and both disease damage and periungual capillaries change were also explored.MethodsA monocentric observational study was conducted on consecutive AAV. Main inclusion criteria were a defined diagnosis of EGPA/GPA/MPA in accordance with International Criteria, age ≥18 ≤ 75 yrs, intraocular pressure (IOP) <21 mmHg by Goldmann, a best-corrected-visual-acuity (BCVA) ≥ 0,5 logMAR, and no ophthalmological and/or systemic disorders or treatment with known retinal involvement. Disease activity was assessed by Birmingham Vasculitis Activity Score (BVAS), damage by Vasculitis Damage Index (VDI), and poorer prognosis by the Five Factor Score (FFS). Moreover, OSDI and MIDAS questionnaires have been administered. Quantitative analysis of vessel density (VD) was performed by OCT-A in the superficial capillary plexi (SCP) and deep capillary plexi (DCP) for all subjects. For completeness, a structural analysis of retinal thickness was performed by OCT-scans. Figures and detailed analysis from NVC were performed for all AAV patients.ResultsFrom 47 consecutive AAV patients referring to the Rheumatologic Clinic, 23 consecutive patients who met the inclusion criteria were included and compared with 20 age/sex-matched healthy subjects (HC) (Table 1). In AAV, BVAS correlated directly with VDI (P=0.001) and FFS (P=0.01) while it was inversely related with disease duration (P=0.01). A total of 46 eyes from AAV patients were analyzed. Retinal VD in superficial whole (SWD) and parafoveal (SPFD) plexi were significantly decreased in AAV compared to HC (P=0.02 and P=0.01, respectively, Figure 1A-B). Furthermore, deep whole (DWD) and parafoveal vessel density (DPFD) were strongly reduced in AAV than HC (P ≤0.0001 for both, Figure 1C-D). In AAV patients, significant inverse correlations emerged between VDI and OCTA-VD in both the superficial (parafoveal, P=0.03) and the deep plexi (whole, P=0.003, and parafoveal P=0.02). The retinal thickness measured by OCT-scans was similar between AAV patients and HC.At NVC examination we found non-specific pattern abnormalities in 82% of AAV patients. Common abnormalities were pericapillary edema (73%), tortuosity (65%), while rare cases of ectasias and hemorrhages resulted (0.8%). No meandering capillaries nor empty dermal papillae were observed. Correlations between NVC changes and OCT-A abnormalities were not described.ConclusionSubclinical microvascular retinal changes occur in patients with AAV and correlate with the disease-related damage. In this context, the OCT-A can represent a useful tool in the early detection of vascular damage. AAV patients present microvascular abnormalities at NVC, whose clinical relevance requires further studies.Table 1.AAV (n=23)GPA, N/%5/22EGPA, N/%9/39MPA, N/%9/39Age at the study (yrs, mean ± SD)60.9 ± 8.7Disease duration (yrs, mean ± SD)9.6 ± 9.1ENT, N/%15/65Kidney, N/%4/17Heart, N/%4/17Lung, N/%21/91Skin, N/%6/26Joint, N/%9/39Peripheral Nervous System, N/%13/57ANCA positivity, N/%16/69.6BVAS (mean ± SD)3.3 ± 2.5VDI (mean ± SD)4.5 ± 1.5FFS (mean ± SD)0.3 ± 0.4Figure 1.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

BackgroundIdiopathic inflammatory myopathies (IMM) include a heterogeneous group of rare autoimmune diseases with a large spectrum of muscular and systemic manifestations, mainly involving skin and lung [1,2]. Myositis specific antibodies (MSA) and myositis associated antibodies (MAA) have been described in IMM patients potentially correlating to disease outcome [3]. As well documented, autoimmune diseases present with a clear gender bias with a greater prevalence among women. To our knowledge, no studies has explored the potential gender difference in IIM patients.ObjectivesThe aim of the study was to explore differences in disease features, clinical outcome, antibody profile, and treatments in IMM patients according to the gender.MethodsIn an observational study, we included patients with a defined IIM diagnosis who were referred to 3rd level Rheumatology Unit “Tor Vergata” University Hospital in Rome (Italy) for the past 5 yrs (to Dec 2022). Inclusion criteria were i. a defined diagnosis of dermatomyositis (DM), polymyositis (PM), and anti-synthetase syndrome (ASS), in accordance with the 2017 EULAR/ACR criteria, ii. age ≥ 18 y.o., iii. availability of medical records and consent to study. Data comprised: disease duration and diagnostic delay, clinical phenotype, treatments, and autoantibodies including anti-nuclear antibodies (ANA), MSAs (anti -MDA5, -NXP2, -SAE, Mi2, -TIF1, anti-tRNA synthetase, -Jo1, -PL7, -EJ), and MAAs (anti-PM/Scl, -Ro52, -Ku, U1RNP).ResultsThe study cohort comprised 31 patients who met the inclusion criteria, with a similar gender distribution [n= 17 (54.8%) females and n=14 (45.2%) males]. The median age at symptoms onset was similar in both groups (59±13.3 vs 58.6±12.6 yrs) while males experienced slightly longer diagnostic delay (10.7±14.4 vs 8.7±9.6 months) and disease duration (30±29 vs 21±20 months) than females (P <0.05 for both). No significant difference in the distribution of IIM occurred between the two groups with a half of patients affecting mostly by DM (F 76.5% vs M 50%). However, both PM (F 6.5% vs M 21.4%) and ASS (F 6.5% vs M 28.6%) were moderately prevalent in males. Skin involvement occurred similarly in both groups while lung disease occurred about twofold in males (57.1%) than females (29.4%). Most patients in the cohort showed ANA titre≥ 1:160, with a comparable rate in females and males (64.7% vs 64.3%), and a positivity for at least one MSA and MAA. A double positivity of MSA occurred in 6.5% of the cohort, all females (MDA5/anti NXP2 and MDA5/EJ). The whole cohort had undergone steroids as 1st line therapy: as steroid-sparing agents, the main difference on treatments occurred for the mycophenolate mofetil which resulted significantly more administered in males (57.14%) than females (6%, P 0.002). Furthermore, among patients with lung involvement (n=13), the need to treat the progression of interstitial lung disease, by using the antifibrotic agent (nintetanib), resulted only in males (15%).ConclusionOur preliminary findings suggest that IMM can present a gender difference in disease outcome by showing a longer diagnostic delay and a higher respiratory involvement in male patients. These data might highlight a possible gender-oriented approach in accordance with a different disease profile and treatment strategies but require further investigations in a larger cohort.References[1]Lundberg, I. E. et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann. Rheum. Dis. 76, 1955–1964 (2017).[2]Lilleker, J. B. et al. The EuroMyositis registry: an international collaborative tool to facilitate myositis research. Ann. Rheum. Dis. 77, 30–39 (2018).[3]Halilu, F. & Christopher-Stine, L. Myositis-specific antibodies: Overview and clinical utilization. Rheumatol. Immunol. Res. 3, 1–10 (2022).Acknowledgements:NIL.Disclosure of InterestsNone Declared.