Explore the vast range of titles available.

Background:Many studies investigating gender-related differences among patients with Connective Tissue Diseases (CTDs) have found a worse Quality of Life (QoL) in female patients. However few studies have investigated gender differences in Idiopathic Inflammatory Myopathies (IIM) with respect to QoL parameters.Objectives:This work aimed at analysing which demographic or clinical factors mostly impacted on the QoL of men with IIMs.Methods:We retrospectively analyzed the clinical charts of male patients with a diagnosis of IIM according to EULAR/ACR 2017 criteria, followed at the Myositis Clinic of our Unit, collecting epidemiological and clinical data. Patients’ QoL was evaluated through the following patient-reported outcomes (PROs): Short-form 36 (SF36), Hospitality Anxiety and Depression Scale (HADS), Functional Assessment of Chronic Disease Therapy-Fatigue (FACIT-F), Health Assessment Questionnaire (HAQ), Patient Global Assessment (PGA). Statistical analysis was performed using the Mann-Whitney test, t test, Chi-square test, and Fisher’s tests, as appropriate; a value of p<0.05 was considered significant.Results:A total of 61 male patients with a mean age of 65.7±14.89 years and a mean disease duration of 8.5 ±6.5 years were enrolled. The graph shows the distribution of clinical subsets. Among organ involvement, the analysis showed a correlation between dysphagia and higher values of HADS-D (p=0.036) and lower values of VT domain of SF36 (p=0.004), between cardiac involvement and higher values of PGA (p=0.004) and lower values of PF (p=0.048), RE and GH items of SF-36 (p<0.04), between sicca syndrome and higher values of HADS-D (p=0.04), FACIT (p<0.001) and items VT (p=0.0015) and BP of SF-36 (p<0.03). Lower scores at MMT8 correlated with higher values of PGA (p=0.07), HAQ-DI (p<0.001) and lower values of items PF, VT and GH of SF36 (p<0.04); higher scores of CDASI-damage correlated with lower values of SF36-VT (p=0.008). Regarding comorbidities, the obesity indirectly correlated with RP and RE items of SF36 (p<0.001), while hyperuricaemia (HU) directly with HAQ-DI (p=0.08) and indirectly with item PF of SF36 (p=0.033). Considering therapies, a correlation was found among higher cumulative doses of steroids and higher values of item SF of SF36 (p=0.038) and between a history of more than 2 immunosuppressant and lower values of RE item (p=0.02) of SF36. Higher scores of PhGA indirectly correlated with FACIT (p=0.002) and item VT of SF36 (p=0.009).Conclusion:Our data showed a higher risk of depression in men with IIM who presented with dysphagia and sicca syndrome. A history of cardiac involvement or obesity seemed to impact on both physical and psychological functioning; on the contrary, muscle involvement, sicca syndrome, cutaneous damage and HU might predominantly interfere with physical domains; moreover, a more complex therapeutic history could compromise the emotional sphere. Of note, higher amounts of steroids seemed to improve the social functioning of patients. Interestingly, rheumatologists seemed to have a better insight in male patients’ perception of fatigue. Further studies are needed to confirm these results; however, they could help clinicians to typify the compromission of QoL in men with IIM, thus allowing focused interventions to improve their management, aiming at optimizing their outcomes.Figure 1.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Idiopathic inflammatory myopathies (IIMs) are rare systemic autoimmune disorders, with a pleiotropic clinical picture, specifically characterized from the inflammatory involvement of striate muscles. As long-term, chronic conditions, IIMs are often associated with comorbidities (CM), often resulting from damage accrual, able to impact on patients’ outcomes.Objectives:The aim of the study was to estimate the prevalence of CM in a monocentric cohort of patients with IIMs, trying to identify those factors most associated with their development and to clarify their weight into the disease’s burden.Methods:We retrospectively analysed medical records of consecutive patients diagnosed with IIM according the EULAR/ACR 2017 criteria, collecting data about demography, clinical characteristics, and quality of life (QoL). QoL was evaluated with Patients Reported Outcomes (PROs): Short-Form 36 Health Survey (SF-36), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT-F), Health Assessment Questionnaire (HAQ), Hospital Anxiety and Depression Scale (HADS). Intergroups comparisons were assessed by using Chi-square, t-test and ANOVA. P values <0.05 were considered significant.Results:A total of 179 patients with a mean age of 66.4±13.7 years and a mean disease duration of 10.0±7.4 years were enrolled; 116 (64.8%) were women. Seventy-nine patients (44.1%) had Dermatomyositis, 71 (39.7%) Polymyositis, 11 (6.2%) Clinically Amyopatic Dermatomyositis, 10 (5.6%) Inclusion Body Myositis, 6 (3.3%) Immune-Mediated Necrotizing Myopathy, 2 (1.1%) Juvenile Dermatomyositis. The aggregation of CM is reported in Figure 1 and their prevalence in Table 1. The most frequent CM were thyroid dysfunctions, hypertension, dyslipidaemia and hyperuricaemia. Twelve patients (6.7%) deceased during the follow-up.Figure 1.Number of comorbidities in our cohortTable 1.Distribution of CM in our cohortComorditiesN%Thyroid dysfunctions9251,4Hypertension8949,7Dyslipidaemia7944,1Hyperuricaemia7340,8Osteoporosis6434,8Fragility fractures2815,6Diabetes mellitus2614,5Obesity2413,4Cataract2011,2Dysthymia1910,6Chronic renal failure1810,1Neoplasms1810,1Fibromyalgia179,5Hypogammaglobulinemia179,5Myocardial infarction116,1MGUS84,5Gout73,9Atrial fibrillation52,8Icuts31,7Female patients had a higher number of CM than males (p=0,008). Both patients’ age and their disease duration directly correlated with CM accrual (respectively p=0,001 and p=0.05).Regarding antibody status, anti-HMGCoA positivity was associated with a greater number of CM (p=0.002).An IIM-related cardiac involvement correlated with a greater number of CM (p=0.04). No CM, nor their combinations in patients, were associated with a higher risk of death.Regarding medications, the number of CM was directly related with intravenous immunoglobulins (IVIg) (p=0.002) and indirectly with cyclosporin A (CyA) (p=0.02). Moreover, those patients who have changed more than two immunosuppressants (IS) have accumulated more CM (p=0.036).PROs’ analysis showed the number of CM inversely correlated with Bodily Pain (p=0.001), Vitality (p=0.016) and Physical Functioning (p= 0.002) domains of SF-36.In the multivariate setting, only the correlation with age, female sex, pharmacological history and SF36 domains were maintained (p=0.001).Conclusion:These data showed more than a half of our cohort had accumulated 4 or more CM. Multi-morbid patients tended to be older and female and seemed to have been treated with a significantly higher number of IS. Moreover, their higher amount of CM compromised QoL, mostly in physical functioning outcomes. Interestingly, they did not seem to be at higher risk of death. Further studies are needed to better clarify these results; however, they might help rheumatologists to improve the assessment of IIM patients, trying to reduce CM aggregation, thus optimizing the disease’s burden.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

To describe different clinical phenotypes of severe flares in a monocentric cohort of SLE patients and to compare treatment and outcomes. Retrospective study of prospectively collected data on 122 severe flares occurred in 110 patients, between 2018 and 2023, and followed up for 12 months after the flare. Baseline characteristics included disease activity assessment by SELENA-SLEDAI and BILAG 2004 scores, demographic and laboratory data. A hierarchical unsupervised segmentation method was applied to cluster flares based on baseline features. Treatments and outcomes according to LLDAS, DORIS Remission and SRI definitions, were compared among clusters at different timepoints. We identified 3 clusters, 2 composed mainly by extra-renal, and one by renal flares. Among non-renal clusters, cluster 1 was characterized by severe constitutional symptoms, serositis and arthritis occurring in younger patients, associated with biomarkers and multiple autoantibodies specificities. Cluster 2 included flares with more BILAG B scores and mainly mucocutaneous and musculoskeletal manifestations, and overlapping antiphospholipid syndrome (APS). Cluster 3 was the renal flares cluster. Cluster 1 and the renal cluster were treated more frequently with glucocorticoid (GC) pulses and mycophenolate mofetil (MMF) and presented higher daily and cumulative GCs doses at 12 months (t12). These two clusters also shared similar percentage of attainment of LLDAS (about 50%) and remission (about 35% both) at t12, compared to 73% of LLDAS and 53% of remission in cluster 2 at t12. We described three different clusters of severe flares in SLE in a real-life setting, identifying a hyper-inflammatory flare phenotype, that shares a comparable proportion of unsatisfying response to treatment as renal flares. Our results may represent a clinical starting point, in the context of precision medicine, for better characterization of severe non-renal disease of SLE, with the final aim of setting up early tailored treatment strategies.

Spondyloarthritis (SpA) are a group of systemic autoimmune diseases, mainly affecting musculoskeletal system, with typical axial or peripheral forms. Recent data from the literature show significant differences in SpA subsets, levels of disease activity, drug efficacy and quality of life between male and female sex. [1, 2] It is well known how disease activity and comorbidities could compromise physical function and quality of life of SpA patients. To describe the clinical characteristics of women with SpA in our single-centre cohort compared to men, in relation to clinical characteristics, disease activity, therapy and comorbidities. Adult patients with a diagnosis of Psoriatic Arthritis (PsA) and Axial Spondyloarthritis (AxSpA) according to CASPAR (ClASsification criteria for Psoriatic Arthritis) and to Assessment of SpondyloArthritis international Society (ASAS) criteria, regularly followed at the SpA clinic of our unit, were consecutively enrolled from April to December 2022. Their epidemiologic, clinic and clinimetric data were collected. Intergroups comparisons were assessed by using Chi-square, t-test and ANOVA. P values <0.05 were considered significant. A total of 200 patients were enrolled, 115 male (57.5%) and 85 female (42.5%) with comparable mean age values (M 56.13±14.48 years, F 56.84±12.22 years; p=0.508) and with a significantly shorter disease duration in women (12.9±10.3 years vs 17.05±12.08 years; p<0.001). No differences in the distribution of PsA or AxSpA based on gender were observed. Six patients had a diagnosis of enteropathic SpA; all of them were women. Taking into account the clinimetric evaluations, female patients show higher values of both Disease Activity Index for Psoriatic Arthrititis DAPSA (15.63 vs 9.40; p=0.003), Leeds Enthesitis Index (LEI) (0.46 vs 0.16; p=0.02) and Spondyloarthritis Research Consortium of Canada (SPARCC) Enthesitis Index (0.7 vs 0.4; p=0.05). The total number of drugs [both Biological disease modifying anti-rheumatic drugs (bDMARDs) and conventional synthetic DMARDs)] taken for disease control was 4.47±2.45 for women and 3.79±2.07 for men (p=0.012); no differences were observed between the 2 groups for any specific drug. Among the comorbidities, none of them was more prevalent in men, while osteoporosis (OP) (p=0,006), mood disorders (0,009), fibromyalgia (FM) (p<0,000015), osteoarthritis (OA) (0,007) and thyroid disease (p<0,000015) were significantly more frequent in women. Women of our cohort show a significantly higher risk of developing an inflammatory bowel disease than men. Female patients have also higher levels of peripheral disease activity, both on joints and enthesis; moreover, they show a significantly lower persistence in drug therapy. Besides, they have a more complex disease, suffering more frequently of OP, OA, FM and thyroid diseases than men. Finally, it is very interesting to note how women tend to develop a much more complex disease, even with a significantly shorter disease duration than male patients. These data confirm how rheumatologists should focus on the assessment of women with SpA, aiming at promoting a closer monitoring of gastrointestinal symptoms, peripheral arthritis and enthesitis and at preventing the development of comorbid conditions, thus optimizing also their quality of life and physical functioning. [1]Rusman T, van Bentum RE, van der Horst-Bruinsma IE. Sex and gender differences in axial spondyloarthritis: myths and truths. Rheumatology (Oxford). 2020 Oct 1;59(Suppl4):iv38-iv46. doi: 10.1093/rheumatology/keaa543. PMID: 33053194; PMCID: PMC7566372. [2]Duruöz MT, Gezer HH, Nas K, Kiliç E, Sargin B, Kasman SA et al, Gender-related differences in disease activity and clinical features in patients with peripheral psoriatic arthritis: A multi-center study. Joint Bone Spine. 2021 Jul;88(4):105177. doi: 10.1016/j.jbspin.2021.105177. Epub 2021 Mar 23. PMID: 33771757. NIL. None Declared.

Backgroundpatients with CTDs have a worse HRQoL compared with the general population and chronic non-rheumatic diseases. However, only few studies have compared HRQoL among CTDs that differ for age at diagnosis, type and severity of organ involvement, disease course.Objectivesto analyze HRQoL and fatigue across different CTDs in a monocentric cohort.Methodsa cross-sectional study that enrolls consecutive adult patients with a diagnosis of Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc) and Idiopathic Inflammatory Myopathies (IIM), regularly followed at the Rheumatology Unit of Pisa. For each patient, demographics, treatment, clinical and laboratory data were collected.At enrollment, each patient completed the SF-36 for HRQoL and the FACIT for fatigue.Resultswe enrolled 490 CTD patients: 255 (52%) SLE, 150 (30.6%) SSc, and 85 (17.3%) IIM. The majority were female (85.7%) and of Caucasian ethnicity (97.5%).At enrollment, patients with SLE were significantly younger (mean age: 44.6 ±12.7 years for SLE, 62.6 ±13.6 for SSc, 65.7 ±12.4 for IIM, p<0.001), with a significantly longer disease duration (14.1 ±10.1 years for SLE, 8.2 ±6.4 for SSc, 5.3 ±5.7 for IIM, p<0.001) compared to the other two groups.The rate of fibromyalgia was highest in SSc (24.7% in SSc, 15.3% in SLE, 11.8% in IIM).Among SLE patients, 79 (31%) presented a mild active disease at enrollment, with skin (35/255) and hematological (32/255) manifestations being the most frequent; only 11 patients (4.3%) presented an active renal disease.Among IIM patients, 67 (78.8%) presented an active disease according to the clinician evaluation: 31 had active myositis and 29 active skin lesions. 33 patients had an Interstitial Lung Disease (ILD).Among SSc patients, 62% presented limited skin involvement; 45 patients (30%) had an ILD and 41 (27.3%) presented a scleroderma heart involvement, while 18 patients had active digital ulcers at enrollment.As far as treatment is concerned, most patients were on Hydroxychloroquine (63.5%); almost half of patients enrolled (45.7%) were on a low dose of glucocorticoids (mean daily dose 2.12 ±2.78 mg of 6-methylprednisolone), and 43.9% were on immunosuppressants.The results of the SF-36 domains are represented in Figure 1.We compared the results of questionnaires across groups.At the univariate analysis, the domains of Physical Function (PF), Role Physical (RP) and Role Emotional (RE) resulted different between groups. In particular, the IIM patients presented the lowest scores compared with the other two groups (Table 1). However, at the multiple linear regression analysis, only the PF domain confirmed to be significantly different across the three groups, after adjusting for age at enrollment and disease duration (β-0.003, [95% CI-0.005, -0.001; p<0.01]).Finally, the FACIT scores were not significantly different between groups.Conclusionin our monocentric cohort of patients with CTDs, there were no clear differences in HRQoL driven by diagnosis, except for the physical function domain which appeared more impaired in myositis patients. HRQoL appears as an independent domain of patients’ health status, only marginally influenced by the clinical characteristics of the disease. This underlines the importance for clinicians managing patients with complex autoimmune diseases to develop common strategies to address and improve HRQoL, parallel to the clinical management of the disease.Table 1.Results of SF-36 and FACIT across CTD patientsIIMSLESScp valuePF55.3±32.175.8±24.567.39±28.4p<0.001 *p<0.01 §p<0.01 +RP40.3±44.264.4±29.350.7±41.9p<0.001 *p=0.12 §p=0.001 +BP63±26.163.8±27.459.3±30.4p=1 *p=1 §p=0.3 +GH43.3±22.149±18.645.4±19.4p=0.07 *p=1 §p=0.25 +VT51.9±22.352.7±21.853.3±20.8p=1 *p=1 §p=1 +SF69.5±2468.3±24.470±24.7p=1 *p=1 §p=0.87 +RE53.3±44.267.5±28.159.3±38.7p<0.01 *p=0.65 §p=0.06 +MH65.2±21.863.2±19.562.4±17.3p=1 *p=0.85 §p=1 +FACIT35.5±10.837.7±10.337.9±9.5p=0.42 *p=0.36 §p=1 +*IIM vs SLE, §IIM vs SSC, +SLE vs SScFigure 1.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

BackgroundSpondyloarthropathies (SpA) are chronic inflammatory arthritis, characterized by both peripheral and axial skeletal involvement and some typical extra-articular marks; they tend to associate with several comorbidities and can increase the risk of an impaired quality of life (QoL). Already published data show SpA expression differs based on gender; in particular, women are at higher risk of a more pronounced peripheral disease activity and show a greater burden of the disease, thus having worse values of Patient Reported Outcomes (PROs). It is well known that a compromission in physical functioning and psychological sphere may compromise productivity of patients and subsequently increase health costs.ObjectivesThe aim of our study was to assess the differences between male and female sex in the prevalence of mood disorders, fatigue and parameters of QoL in a monocentric cohort of patients with SpA.MethodsAdult patients diagnosed with Psoriatic Arthritis (PsA) and Ankylosing Spondylitis (AS) according to the CASPAR (ClASsification criteria for Psoriatic Arthritis) and to Assessment of SpondyloArthritis international Society (ASAS) criteria, regularly followed at the SpA clinic of our unit, were consecutively enrolled from April to December 2022. Epidemiologic, clinic and clinimetric data were collected. Each patient filled in the following Patient Reported Outcomes: Hospital Anxiety and Depression Scale (HADS), FACIT-Fatigue (FACIT-F) and SHORT-FORM 36 (SF-36). Intergroups comparisons were assessed by using Chi-square, t-test and ANOVA. P values <0.05 were considered significant.ResultsA total of 200 patients were enrolled; 85 (42.5%) were women, with a mean age of 56.84±12.22 years and a mean disease duration of 12.91±10.3 years. One hundred and thirty-five patients (67.5%) had a diagnosis of PsA (M/F 79/56), while 59 (29.5%) had a diagnosis of AS (M/F 36/23) and 6 (3%) of Enteropathic Arthritis (M/F 0/6). Women showed worse FACIT-F values than men, (mean 35.20 vs 40.23, p-value 0.002) and a greater impairment of QoL expressed by statistically significant lower values of almost all the domains of SF-36 (exception for Global Health). Moreover, the values of HADS resulted higher in female sex both for the anxiety and depression domains (p-value <0.001 and 0.039 respectively).ConclusionThe analysis of our cohort confirmed that women affected by SpA suffer from anxiety and depression more often than men, develop a higher level of fatigue and have an overall worse QoL in both psychological and physical domains. These data should sensitize rheumatologists in the assessment of this subgroup of patients. The clinician should not only focus on the disease activity evaluation and therapy or in managing of the comorbidities, but also on the psychological and functional status of the patients, developing strategies able to avoid or delay the onset of their impairment in daily life and to optimize with specific therapies, rehabilitation or psychological support their compromission, when already established. This approach could finally improve the social burden and health cost of women with SpA.Figure 1.References[1]Wright GC, Kaine J, Deodhar A. Understanding differences between men and women with axial spondyloarthritis. Semin Arthritis Rheum. 2020 Aug;50(4):687-694. doi: 10.1016/j.semarthrit.2020.05.005. Epub 2020 May 25. PMID: 32521322.[2]Swinnen TW, Westhovens R, Dankaerts W, de Vlam K. Widespread pain in axial spondyloarthritis: clinical importance and gender differences. Arthritis Res Ther. 2018 Jul 27;20(1):156. doi: 10.1186/s13075-018-1626-8. PMID: 30053895; PMCID: PMC6062924.AcknowledgementsTo all the dear collegues I work with.Disclosure of InterestsNone Declared.

Subcutaneous immunoglobulins (SCIg) therapy is effective in patients with Idiopathic Inflammatory Myopathies (IIMs), especially in severe and refractory forms of the disease; however, its use is burdened by high costs. Although it is now known that the dosage to be used for the immunomodulatory effect is 2 g/kg per month, there are no standardised tapering or discontinuation schemes to date. The aim of the study was to assess whether there are differences in terms of clinical outcomes and patient Quality of Life (QoL) between two different SCIg treatment regimens, involving discontinuation of therapy after the first six months or continuation of the drug for a further six months. Within the cohort of IIM patients (2017 EULAR/ACR criteria) followed at our Myositis Clinic, we selected those who were treated with SCIg and, dividing them into two groups according to whether they discontinued therapy after the first six months, we performed a retrospective analysis on data prospectively collected. For each patient, demographic and clinical data (age, sex, disease subset and duration, organ involvement, comorbidities, treatment) were collected from medical charts. The International Myositis Assessment & Clinical Studies Group Disease Activity Core Set Measures (IMACS-CSMs) [Physician Global Activity (PhGA), Patient Global Activity (PGA), 8-items Manual Muscle Testing (MMT8), Health Assessment Questionnaire (HAQ), serological muscle enzymes values] were used to assess the disease activity at baseline and to evaluate the clinical outcomes after six and twelve months from the start of SCIg therapy. Patients' perspective was evaluated also by administration of Patient Reported Outcomes (PROs) not included in the IMACS-CSMs: Short-Form 36 Items Health Survey (SF-36), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT-F), Hospital Anxiety and Depression Scale (HADS). We then compared the delta of IMACS-CSMs and PROs between the 12-month (12m) and 6-month (6m) assessments to detect any significant changes in patients' health status following the discontinuation of SCIg therapy. We included 18 patients (12 dermatomyositis, 6 polymyositis; 61.1% female) with a mean age at the beginning of SCIg therapy of 63.8±14.8 years. Of these, 10 (55.6%) discontinued SCIg therapy after 6 months of treatment while 8 (44.4%) continued it for at least one year. We found no differences between the two groups in terms of disease activity and patients' QoL at baseline. At 12 months evaluation, there were no statistically significant differences in PhGA (2.9±1.7 vs 2.5±2.0), MMT8 (67.9±8.0 vs 75.1±6.1) and serum values of muscle enzymes (CPK, LDH, aldolase) between those who had discontinued SCIg after the first 6 months and those who continued the treatment. Patients' QoL was also found not to differ between the two groups, as no statistically significant differences emerged between the scores of HAQ, all SF-36 domains, FACIT-F and HADS anxiety and depression subscales. The delta 12m-6m of QoL assessment parameters showed no differences between the two groups, while there was a worsening of mean PhGA (1.5±1.4 vs -0.5±1.8, p=0.026) and MMT8 (-5.9±6.6 vs 3.3±2.8, p=0.010) in those who discontinued therapy compared with those who continued SCIg. Although preliminary and with the limitations inherent to the retrospective nature of the study, these data seem to suggest that there are no significant differences in outcomes assessed by IMACS-CSMs and in patient's perception of the disease between different treatment regimens concerning the discontinuation of SCIg. If our findings will be confirmed by future studies on larger cohort of IIM patients, although it appears that there may be a slightly deterioration in muscle function, discontinuing SCIg therapy after the first six months of treatment could reduce health costs, without the risk of significantly compromising patients' quality of care. NIL. NIL. None Declared.

Background:C-Reactive Protein (CRP) elevation in SLE is considered as strongly suggestive of infection. In clinical practice, however, it is not uncommon to observe an increase of inflammatory biomarkers related to SLE disease activity, namely to arthritis and serositis.Objectives:To characterize severe SLE flares with hyper-inflammatory stigmata and to compare them to severe, no-hyper-inflammatory flares.Methods:This is a retrospective analysis of prospectively collected data about severe disease flares occurred in the last five years in a monocentric cohort of SLE inpatients fulfilling 2019 ACR/EULAR classification criteria. In all cases concomitant infections, hematological or oncological conditions were excluded.The following data were collected from clinical charts: demographics, comorbidities, disease duration, cumulative organ involvement and damage accrual (SLICC-DI), disease activity (SLEDAI 2K, BILAG 2004), immunological profile, laboratory, imaging, histology, and treatment data at the time of the flare (T0), treatment and disease status at 3-, 6- and 12-months of follow-up.Hyper-inflammatory (HI) flares were defined by CRP serum levels ≥ 5 mg/dL and/or circulating ferritin levels ≥ 500 µg/L and were compared with flares without HI characteristics (no-HI), hospitalized in the same period in our Centre.Chi-square and t-tests were used for univariate analysis and a logistic regression model was used for multivariate analysis.Results:Among 122 severe flares hospitalized in the study period, based on the previously defined criteria, 22 flares, in 21 patients, were HI flares while 100, in 89 patients, were no-HI flares. No patient was found to belong to both groups. Patient and flare characteristics are summarized in Table 1.HI flares presented more frequently fever, pericarditis, arthritis and large vessels vasculitis and less frequently renal involvement, according to SLEDAI definitions. Lymphadenopathies and splenomegaly were also significantly associated with HI flares. According to the BILAG index, patients in the HI group presented more frequently a BILAG A score in the constitutional, cardiopulmonary, and hematological domains.As for laboratory data HI flares showed significantly higher levels of all the inflammatory biomarkers and higher C3 complement levels. Moreover, patients in the HI group presented more frequently a Lupus anticoagulant (LAC) positivity.At the multivariate analysis, cardiopulmonary, constitutional, and hematological involvement, splenomegaly, LAC+ and higher levels of fibrinogen confirmed to be independently associated with the HI phenotype (Table 2).Having a HI flare resulted significantly associated with an overall higher number of diagnostic procedures performed and to a longer duration of hospitalization.With exception of colchicine, no other significant differences were found with respect to therapeutic choices (Table 3); achievement of treatment targets at the different time-points and damage accrual at 12 months was also similar in the 2 subgroups.Conclusion:We identified a subgroup of SLE flares that defines a “hyper-inflammatory” phenotype whose severity seems to be mainly driven by marked constitutional symptoms. Interestingly, HI flares were associated with an underlying large vessels vasculitis in 3 cases.HI flares may represent a challenge for rheumatologists, particularly for the difficulty in differential diagnosis with infections and hematological conditions, leading to a higher number of diagnostic procedures and a longer duration of hospitalization. In view of the association with large vessel vasculitis, the clinician should consider performing a PET-CT scan in patients with HI phenotype to properly establish immunosuppressive therapy.This work suggests that severe SLE flares may present with proteiform and unexpected clinical characteristics. Further studies are needed to better outline clinical phenotypes, from “bedside to bench”, looking for new biomarkers and tailored treatment strategies.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:the relationship between disease activity and Health-Related Quality of Life (HRQoL) in Systemic Lupus Erythematosus (SLE) is controversial. Data in the literature show that disease activity and different aspects of HRQoL may follow distinct trajectories over time.Objectives:to evaluate the change of HRQoL, between two consecutive evaluations, in a monocentric cohort of SLE patients.Methods:this is a retrospective study of prospectively collected data of consecutive outpatients with SLE. For each patient, the following data were collected: demographics, laboratory and clinical data, SELENA-SLEDAI for disease activity and SLICC-DI for organ damage, comorbidities and ongoing treatment. At each visit, patients completed the following Patient Reported Outcomes (PROs): SF-36, FACIT-Fatigue, Lupus Impact Tracker (LIT), SLAQ, Hospital Anxiety and Depression Scale (HADS).For each patient two consecutive evaluations (T1 and T2), performed at a time interval ranging from 6 to 18 months between each other, were analysed.Comparisons between T1 and T2 were performed through the paired Wilcoxon test for continuous variables and the chi-square test for categorical ones. The difference in scores obtained in each questionnaire between the two observations (T2-T1 = Delta value) was calculated to determine how they evolved over time. The Spearman correlation test was used to explore the correlations of delta values between questionnaires and clinical variables to evaluate the congruence in the changes observed over time.Results:205 SLE patients were enrolled, mainly female (91.7%); mean age at enrolment was 44.8±12.5 years, with a mean disease duration of 13.5±10 years. Overall, patients enrolled presented a low disease activity (mean SLEDAI 2.03±2.2) and organ damage (mean SLICC-DI 0.72±1.23), without a significant difference between the two consecutive evaluations. The majority of patients were in remission in both the timepoints (T1 70.2% - T2 76.9%, p=0.2), while 31 (15.12%) presented an active disease at T1, with cutaneous (13.3%) and haematological (8.8%) being the most frequent active manifestations. 28 patients (14%) had concomitant fibromyalgia. 104 patients (57.5%) were on a low dose of glucocorticoids (GC) (mean 2.48 mg/daily of 6-methylprednisolone), 76 (42%) were on immunosuppressive therapy and 164 (90.6%) on hydroxychloroquine. 27 patients (14.9%) were on treatment with belimumab.There was no significant difference in the results of PROs between the two consecutive evaluations in the entire cohort. We then analysed the subgroup of 31 patients with active disease at T1 (Table 1). They presented a significant reduction of the SLEDAI at T2 (T1 5 [4-6] - T2 2 [0-4], p<0.001). Concordantly, we found a significant increase of patients in remission (T1 0 - T2 62.1%, p<0.001), a reduction in the GC daily dose (T1 4 [4-8] - T2 4 [2-4] mg/daily, p<0.01) and an increase of patients on belimumab (T1 19.4% - T2 50%, p<0.05).Within this subgroup of active patients, some of the PROs results showed a significant improvement between the two timepoints. In particular, they showed a significant improvement in fatigue (FACIT, p<0.05), disease burden (LIT, <0.05) and some of the SF-36 domains (MCS p<0.05; PF p=0.001; RE p<0.01).Importantly, variations over time (Delta values) of the scores of SLAQ, FACIT, LIT, and some SF-36 domains (PF, RP, BP, SF, RE) resulted significantly correlated and congruent with variations of the SLEDAI score between the two evaluations (Table 2).Conclusion:our study demonstrates that, in a large cohort of SLE patients, with an overall stable and well-controlled disease, PROs tend to remain stable over time. However, among patients with active disease at enrolment, we observed that the improvement of disease activity, in the short term, can have a positive impact mainly on the physical aspects of HRQoL and disease burden. On the contrary, mental health seems to be an independent domain, maybe influenced by different factors. Therefore, understanding the trajectories of change in HRQoL outcomes over time may help to develop tailored interventions for the management of SLE patients.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

BackgroundGlucocorticoid (GC) dose minimization and, if possible, complete withdrawal is one of the main targets in the daily management of Systemic Lupus Erythematosus (SLE).Objectivesto describe frequency and clinical characteristics of SLE patients in GC-free remission in a real-life setting and to identify predictive factors for achieving GC-free remission.Methodsthis is a retrospective analysis of prospectively collected data from a monocentric SLE cohort. The following variables were retrieved: demographic data, cumulative organ involvement; at last observation: disease activity (SLEDAI-2K score), ongoing therapy, disease state (remission defined according to the 2021 DORIS criteria) and organ damage (SDI score). Disease state at 1 year from disease onset was also recorded.Resultsfrom our cohort, a total of 390 SLE patients had at least 1 year of follow-up and complete clinical data to be included in the analysis; of these, 142 (36.4%) were in GC-free remission at the last evaluation, and 44 (11.3%) were GC-free for 5 years (Table 1). The mean follow-up duration was 10.5 years (min 1-max 41).No significant differences were found with regard to age at disease onset, disease duration and organ involvement between the GC-free remission group (GC-) and the other SLE patients under GC treatment (GC+). Patients GC- were less frequently taking immunosuppressants (IS) (28.9%, vs 43.5% p<0.01) or biological drugs (6.3% vs 20.2% p<0.01) and were more frequently under HCQ treatment (85.2% vs 75.8%, p=0.03) at last observation.Being GC- at last observation was associated with a significantly lower organ damage with respect to GC+ (mean SDI 0.7 vs 1.5, p<0.01); significant differences regarded cardiovascular (CV) events (4.9% vs 12.1%, p=0.01) and osteoporosis (OP) (12.7% vs 27.0%, p<0.01) were also found. Overall, 212 patients were in remission after 1 year from disease onset (78.2% in GC- and 57.2% in the GC+) and this condition resulted significantly associated with GC-free remission at last evaluation (p<0.01).At multivariate analysis, being in remission at 1 year resulted an independent predictor of GC-free remission (OR=2.06, p=0.02); the multivariate analysis also confirmed that GC- patients were less likely on IS or biological treatment at last evaluation (OR 0.46 p<0,01 and OR 0.20 p<0.01, respectively).Conclusionthese data suggest that GC-free remission is an achievable goal in SLE patients with today’s drugs, and in our cohort GC-free remission is also a IS-free remission in most of patients. Our study also confirms that GC withdrawal has important advantages in term of organ-damage sparing.Of note, the early achievement of remission during the disease history is associated with a good probability of GC withdrawal over time.Table 1.characteristics of the cohortGC-N=142 (40.4%)GC+ N=248 (59.6%)P valueFemale (%)123 (86.6)218 (87.9)0.71Age at disease onset, years128.7±11.829.8±12.20.44Cumulative dose of GC, grams114.7±13.323.8±24.7<0.01Disease duration, years116.9±8.616.6±10.30.72Remission after 1 year from disease onset (%)*97 (78.2)115 (57.2)<0.01Renal involvement (%)61 (42.9)114 (45.7)0.55Joint involvement (%)100 (70.4)190 (76.6)0.06Skin involvement (%)82 (57.7)162 (65.3)0.11Haematological involvement (%)84 (59.1)157 (63.3)0.23Serositis (%)24 (16.9)61 (24.5)0.05Neuropsychiatric involvement (%)10 (7.0)29 (11.7)0.12HCQ (%)121 (85.2)188 (75.8)0.03IS (%)41 (28.9)108 (43.5)<0.01Biologicals (%)9 (6.3)50 (20.2)<0.01SLICC-Damage Index10.7±1.11.5±2.0<0.01CV event (%)7 (4.9)30 (12.1)0.01OP (%)18 (12.7)67 (27.0)<0.011 Mean±standard deviation; *Data on 325 patientsREFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.