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In 4 decades, Elsa Prochazka completed a range of projects of varying sizes: from furniture design through architecture to urban design projects. If there was a Vienna school of superior contemporary interior design, Elsa Prochazka would be a prototypical representative: Her \"interiors seem to invite tactile participation, demanding the involvement of the body as an agent of the mind\" (Otto Kapfinger). This quote could stand equally for her buildings. Prochazka developed and disseminates her conceptional approach within the framework of her professorships and her international lecturing activities. The book closes the gap in the documentation of significant female Viennese architects.

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.

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.

Background:Patients diagnosed with Systemic Autoimmune Diseases (SAD) face distinct challenges during pregnancy potentially affecting maternal health and neonatal outcomes. Despite the well-known prominence of sleep and mental health disturbances in SAD and the vulnerability of mental health during pregnancy due to physiological, psychological, and hormonal changes, a significant gap persists in comprehending the prevalence, severity, and intricate interrelationships between sleep disturbances, anxiety, and depression symptoms in SAD during gestation.Objectives:To investigate the prevalence of insomnia, anxiety, and depression symptoms in individuals with SAD during pregnancy. Additionally, this study evaluates the correlation between mood, anxiety, and insomnia, providing insights into the complex interrelationships among these factors.Methods:This study assessed consecutive women during pregnancy diagnosed with SADs, including Connective Tissue Diseases (CTD), Inflammatory Arthritis (IA) and Systemic Vasculitis (SV) attending the pregnancy clinic of a tertiary referral center. Patients were evaluated through the Insomnia Severity Index (ISI), the State-Trait Anxiety Inventory (STAI Y1 and Y2), the Difficulties in Emotion Regulation Scale (DERS), the Mood Disorder Questionnaire (MDQ), and the Postpartum Depression Predictors Inventory-Revised (PDPI-R). Frequency analyses identified the prevalence of patients reporting significant symptoms (scores over the cut-point in the referred questionnaires). Non-parametric between group comparisons and correlations were used to evaluate the associations between clinical variables (diagnosis, pharmacological treatment, years between disease onset and pregnancy) and insomnia, mood, and anxiety symptoms with a significance level set at p-value <0.05.Results:Sixty-five patients were enrolled, with a mean age of 34.69 (±4.57) years and a mean disease duration of 9 (±5.10) years. Fifty patients (76.9%) were diagnosed with Connective Tissue Diseases (CTD), 20 (18.5%) with Inflammatory Arthritis (IA), and 3 (4.6%) with Systemic vasculitis (SV). Among the patients, 13 (22.8%) were receiving glucocorticoids (GCs), 33 (56.9%) hydroxychloroquine (HCQ), 9 (15.3%) traditional or biologic immunosuppressants (IS TRAD/BIO), and 29 (49.2%) anticoagulants or antiplatelet agents (ACT/APT). A high percentage of significant symptoms was found with prevalence rates of insomnia at 53.1%, anxiety symptoms at 45.3% (state) and 40.6% (trait), emotional regulation difficulties at 44.6%, significant mood disturbances at 10.8%, and a significant risk of postpartum depression in 6.2% of cases. Between-group comparisons based on diagnostic categories and pharmacological treatments revealed significant differences when patients were categorized according to hydroxychloroquine (HCQ) intake with significantly higher STAI-Y1 and PDPI-R scores in the group not consuming HCQ compared to the group consuming HCQ (p-value <0.05, see Table 1). Significant correlations were found between the STAI and ISI, MDQ and DERS scores (p <0.05, see Table 2).Conclusion:This study sheds light on the underexplored psychological aspects of pregnancy in SAD, emphasizing the need for comprehensive mental health care in this population. Significant correlations between anxiety symptoms and mood, emotion regulation and insomnia suggest the necessity to further explore the role of anxiety as a trigger of mental health complications in this clinical population. The association emerged between the HCQ use and better scores in terms of anxiety symptoms and post-partum depression is intriguing. We could argue that HCQ may maintain better control over the underlying autoimmune condition thus influencing mental health outcomes during pregnancy. These are preliminary interpretations, and further research, including controlled studies and exploration of potential confounding factors, is needed to establish causation and understand the underlying mechanisms.REFERENCES: NIL.Table 2. Correlations between questionnaires total scores and age and disease duration in the overall sampleAcknowledgements: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.

BackgroundSeveral risk factors for hospitalization have been identified among clinical, demographic, and laboratory features in SLE patients. However, less is known about how and how much disease expression in the first year since diagnosis may impact the outcome or predict disease course in the following years.ObjectivesTo define early risk factors for hospitalization in SLE.MethodsObservational, retrospective analysis in a monocentric cohort of SLE patients, regularly followed at our Unit. Demographics, clinical manifestations, hospitalizations over time, SLE flares and treatments at disease onset and during follow-up were collected. In particular, number and type of immunosuppressants (IS), daily and cumulative doses of glucocorticoids (GCs) at o 1 and 5 years of follow-up. Disease activity was evaluated by SLEDAI-2K score at baseline, at 1 and 5 years of follow-up while organ damage was assessed by the SLICC/ACR damage index (SDI). Disease state as modified Lupus Low Disease Activity status (LLDAS) without PGA, was evaluated at one year from SLE diagnosis. T-test and chi square test were used to estimate association between at least one hospitalization within the fifth years of disease and sex, age, and clinical manifestations at disease onset.ResultsAmong 422 Caucasian SLE patients regularly followed in our centre, (87% female, mean age 47±13.2 years, follow-up duration 10.5±9.3 years) 263 (62%) had at least one hospitalization due to SLE disease activity or disease related complications within five years from disease onset. Similar hospitalization incidence rate at five years and at the last visit were found (0.19 and 0.21 patient-years respectively). Characteristics of hospitalized patients at 5 years with respect to non-hospitalized ones are reported in Table 1. Disease onset with nephritis or constitutional symptoms (fever, lymphadenopathies) and not being in LLDAs at one year were significantly associated to hospitalization within five years (p values <0.01). Moreover, number of IS, cumulative GC dose at 1 and 5 years and SDI at 5 years of follow-up resulted significantly associated with hospitalizations (p values<0.01). No correlations were found between age at disease onset, sex, diagnostic delay, other manifestations at disease onset, SDI at 1 year and the risk of hospitalization over time.ConclusionHospitalizations for disease activity and its complications are frequent in SLE patients, not only in the first years of the disease. A more aggressive SLE phenotype and treatments at onset are associated with higher risk for future hospitalizations; on the other hand, being on LLDAS within one year from disease onset seems to be protective. Therefore, our data suggest that disease expression within the first year from diagnosis could be crucial to predict SLE course over time and to stratify patients at lower or higher risk for severe disease.Table 1.Cohort characteristicsOverallHospitalized patients at 5 yearsNon hospitalized patientsP valueF Sex (%)87,487.089.10.5Age at onset (mean, sd)29.1±11.828.7±12.229.9±11.20.3Diagnostic delay (years, mean sd)2.2±4.21.9±0.92.6±1.60.08Renal at onset (%)14.990.49.5<0.01Serositis at onset (%)8.076.423.50.08Constitutional at onset (%)20.675.824.1<0.05Cutaneous at onset (%)39.537.044.50.2Articular at onset (%)55.451.962.40.036No LLDAS at one year (%)30.678.521.4<0.01Cum. GC dose at 1 year (g, mean, sd)2.9±2.43.7±2.61.6±1.4<0.01Cum. GC dose at 5 years (g, mean, sd)9.2±6.310.8±6.56.3±4.7<0.01Number of flares at 5 years (mean, sd)0.5±0.90.7±1.00.2±0.6<0.01Number of IS at 1 year (mean, sd)0.7±0.80.9±0.80.3±0.5<0.01Number of IS at 5 years (mean, sd)1.0±1.21.3±1.30.4±0.7<0.01SLICC-DI at 1 year >0 (%)6.8%11.2%6.7%0.1SLICC-DI at 5 years >0 (%)11.1%21.4%9.1%<0.01REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Health-Related Quality of Life (HRQoL) in patients with autoimmune systemic diseases has a multifactorial origin. In particular, data from the literature show that in patients with Systemic Lupus Erythematosus (SLE) the correlation between HRQoL and disease manifestations is quite controversial. to evaluate the impact of different disease activity patterns on HRQoL and patients' perception of disease activity in a monocentric cohort of patients with SLE. a retrospective analysis of prospectively collected data from adult consecutive patients with a diagnosis of SLE, regularly followed at the Rheumatology Unit of Pisa. For each patient, the following data were collected: demographics, disease duration, clinical and laboratory data, SELENA-SLEDAI for disease activity and SLICC-DI for organ damage, comorbidities and ongoing treatment. Three different disease patterns were defined based on disease activity status during the year before enrollment: long quiescent (LQ) if patients were in remission in each visit; chronically active (CA) if they were not in remission in each visit; relapsing-remitting (RR) if patients presented periods of remission interspersed with periods of disease activity. Only patients with at least two visits in the year before enrollment were included. We excluded patients with a major clinical event/hospitalization not SLE-related during the year before enrolment that could influence their health status. At enrollment, each patient completed the following Patient Reported Outcomes (PROs): SF-36, FACIT-F, Lupus Impact Tracker (LIT), SLAQ, Hospital Anxiety and Depression Scale (HADS). 242 SLE outpatients were enrolled, mainly female (91.7%) and of Caucasian ethnicity (96.7%); mean age 44.2.6 ± 13 years, with a median disease duration of 12 years (IQR 6 – 21). 48.8% had at least one item of organ damage with a median SLICC-DI of 1 (IQR 1-2). Moreover, 15.3% of patients had a concomitant fibromyalgia. At enrollment, median SLEDAI in the entire cohort was 2 (IQR 0-4); the most frequent active disease manifestation was skin involvement (36/242); 12 patients had active arthritis and 11 active renal disease. Almost half of the cohort (51,2%) was on a low dose of glucocorticoids (mean daily dose 4.1 ± 2.9 mg of 6-methylprednisolone); 83.9% were on Hydroxychloroquine and 52.5% on immunosuppressive therapy. We applied the disease pattern definition criteria and we found that the LQ was the most represented pattern in our cohort (63.9% of patients), followed by the RR (22%) and CA (14.1%). No significant differences in SF-36, HADS, LIT and FACIT results were observed between the 3 groups. Only SLAQ scores resulted significantly higher in the RR group compared with the LQ one (p<0.01) (Table 1). our study shows that HRQoL in SLE is not significantly influenced by disease course over time, confirming that disease activity does not represent the major determinant of patients' perception of health status. However, we found that patients' self-evaluation of disease activity is affected by the disease pattern over time. Particularly, it seems that alternating periods of disease activity to periods of remission over time may worsen the patient's evaluation of disease status, even more than a mild chronically active disease. [1]Györi N et al., Lupus Sci Med. 2017 Feb 8;4(1):e000192. NIL. None Declared. Table 1Results of PROs among disease activity pattern groups in the SLE cohort.CALQRRp valuePCS44.54 ± 8.748.22 ± 10.546.7 ± 9.2p=0.16 *p=0.95 §p=1 +MCS43.2 ± 11.944.2 ± 12.542 ± 10.7p=1 *p=1 §p=0.7 +LIT29 ± 18.724.1 ± 20.529.7 ± 24.4p=0.73 *p=1 §p=0.35 +FACIT36 ± 11.639 ± 9.636 ± 11.1p=1 *p=1 §p=0.12 +HADS anxiety7.4 ± 4.56.5 ± 3.96.7 ± 3.4p=1 *p=1 §p=1 +HADS depression5.7 ± 3.75.1 ± 3.65.8 ± 3.7p=1 *p=1 §p=1 +SLAQ11 ± 7.98.6 ± 6.813 ± 9.6p=0.67 *p=0.73 §p<0.01+*CA vs LQ, §CA vs RR, +LQ vs RR

The assessment of cutaneous involvement in Systemic Lupus Erythematosus (SLE), particularly the differential diagnosis between active lesions and damage, might be a challenge for the clinicians. Ultra-high frequency ultrasound (UHFUS), with a sub-millimeter resolution, is a promising tool in the evaluation of superficial structures. In recent years its use has grown in the field of dermatology, where UHFUS finds application in the study of normal anatomy, differentiation of cutaneous lesions (bullous and inflammatory disease), diagnosis of skin malignancies and pre-surgical mapping, increasing diagnostic sensitivity and accuracy in guiding medical and surgical therapies. The aim of the study was to explore a possible role of UHFUS assessment in the evaluation of skin involvement in a monocentric cohort of SLE patients and to evaluate the relationship with patients' perception of their skin disease. Consecutive adult SLE patients (1997 ACR criteria) regularly followed at our Lupus Clinic were prospectively enrolled during a scheduled outpatient visit in presence of skin lesions. Demographical, clinical, laboratory and treatment data were collected at enrolment. Disease activity and organ damage were evaluated with the SELENA-2K and SLICC-DI, respectively. Clinical assessment of skin lesions was done by an experienced rheumatologist using the Cutaneous LE Disease Area and Severity Index (CLASI); at the same time, UHFUS evaluation of skin lesions was performed with a 70 MHz probe by an experienced dermatologist. The Skindex-16 questionnaire was used to assess the impact of the skin involvement on patients' quality of life. We included 70 assessments in 55 SLE patients with skin lesions (5/55 evaluated twice, 5/55 three times). Cutaneous disease subtypes were distributed as follows: acute 37.5%, subacute 12.5%, chronic 50.0%. The characteristics of the cohort are shown in Table 1. The most frequent UHFUS alteration was the presence of power Doppler (51/70, 72.9%), followed by dermal oedema (37/70, 52.9%), dermal inhomogeneity (33/70, 47.1%), follicular plugging (32/70, 45.7%), vascular ectasia (27/70, 38.6%) and thinning of the epidermis (10/70, 14.3%). The CLASI activity score was significantly higher in cutaneous areas with power Doppler signal (p=0.032). Dermal oedema was also found to be associated with higher CLASI activity scores, considered both globally (p<0.001) and at the level of the US-evaluated region (p=0.002), and with higher damage scores of the evaluated area (p=0.034), while vascular ectasia was only associated with higher damage scores of the evaluated area (p=0.036). Skindex-16 symptoms subscale scores were significantly higher in patients with thinning of the epidermis (p=0.01) and dermal inhomogeneity (p=0.049). Regarding the subtype of cutaneous involvement, vascular ectasia was significantly more frequent in chronic cutaneous LE (p=0.029), while no other UHFUS alterations were found to differ between the three groups. Although preliminary, our data seem to suggest UHFUS as a promising tool not only for assessing skin involvement in SLE, but also for monitoring disease activity over time with a view to optimising the management of these patients. NIL. NIL. None Declared. Table 1Characteristics of the cohortN° of patients55Female49 (89.1%)Age1 [years]45 (35-55)Disease duration1 [years]11 (7-19)Ethnicity: Caucasian/ Asian/ African-American51 (92.7%)/ 3 (5.5%)/ 1 (1.8%)Organ involvement [cumulative/ ongoing]Mucocutaneous55 (100%)/ 55 (100%)Haematological20 (36.4%)/ 6 (10.9%)Renal7 (12.7%)/ 2 (3.6%)Articular33 (60.0%)/ 8 (14.5%)Serositis2 (3.6%)/ 0Neuropsychiatric3 (5.5%)/ 0SLEDAI-2K1 *4 (2-6)SLICC-DI1 *0 (0-1)CLASI activity1 *[global/ UHFUS area]5 (3-8)/ 2 (1-3)CLASI damage1 *[global/ UHFUS area]1 (0-5)/ 0 (0-2)Skindex-16 global1 *53.9 (31.3-83.3)Skindex-16 symptoms1 *50.0 (25.0-85.7)Skindex-16 emotions1 *61.9 (28.6-97.6)Skindex-16 functioning1 *33.3 (13.3-86.7)1 Median (IQR)* data on 70 assessments

Background:Anifrolumab (ANI) is a fully human monoclonal antibody against the type I interferon receptor that has recently been approved for the treatment of moderate to severe Systemic Lupus Erythematosus (SLE) as an add-on treatment to standard of care. Data from randomized controlled trials have demonstrated its efficacy and safety, but real-world data are still limited, especially regarding the impact of this new drug on patients’ quality of life (QoL).Objectives:To evaluate the effect of ANI therapy on QoL and disease burden in a multicentric cohort of refractory SLE patients.Methods:Consecutive adult SLE patients (2019 EULAR/ACR criteria) were prospectively enrolled at ANI prescription. Data on demographic features, medical history, previous therapies and SLICC-DI were collected from clinical charts at enrolment. Patients’ assessments were performed at the first ANI infusion and subsequently after one, three and six months of treatment. At each time-point, the clinical evaluation included SLEDAI-2K, SLE-DAS, Physician Global Assessment, number of tender and swollen joints and cutaneous activity and damage assessed using the Cutaneous LE Disease Area and Severity Index (CLASI-A for activity and -D for damage). Patients’ perspective was assessed by self-administration of validated Patient Reported Outcomes (PROs): Lupus Impact Tracker (LIT) to assess the overall impact of SLE on patients’ QoL, Functional Assessment of Chronic Illness Therapy – Fatigue scale (FACIT-F) to measure self-reported fatigue and its impact on daily activities and, in a subgroup of patients with mucocutaneous involvement, Skindex-16 which is a specific PRO to investigate the impact of the skin disease in symptom, emotional and functioning spheres.Results:Twenty-five patients (96% female, 92% Caucasian) with a median age of 45 years (IQR 37-58) and a median disease duration of 11 years (IQR 7.5-21.5) were enrolled. In the whole cohort, 24 patients (96%) had a history of articular involvement, 23 (92%) of mucocutaneous involvement, 16 (64%) of haematological involvement, 7 (28%) of lupus nephritis, 5 (20%) of serositis and 4 (16%) of neuropsychiatric involvement. At baseline, 23 patients (92%) were on concomitant steroid therapy (median prednisone daily dose 7.5 mg, IQR 5-10), 19 (76%) on hydroxychloroquine and 23 (92%) on immunosuppressive treatment (10 methotrexate, 9 mycophenolate mofetil, 3 azathioprine, 1 cyclosporine). Active disease manifestations at the time of ANI prescription were in most cases mucocutaneous (18/25, 72%), followed by articular (10/25, 40%) and haematological (5/25, 20%) involvement.As reported in Table 1, after ANI start, all the disease activity measures showed a progressive improvement over time. A significant correlation was observed between LIT and joint count and Skindex-16 and CLASI-A at baseline (r≥0.464, p≤0.026 for LIT; r≥0.731, p≤0.04 for Skindex-16). During follow-up, LIT and Skindex-16 exhibited progressively and significantly improved scores, while no changes were observed in FACIT-F scores. Notably, Skindex-16 was significantly improved as early as 4 weeks after the first drug infusion (symptoms p=0.028, emotions p=0.03, functioning p=0.05), while LIT reached statistical significance after 12 weeks of treatment (p=0.046), maintaining in both cases the result achieved over time (Figure 1).Conclusion:Our preliminary data show that ANI not only allows a rapid clinical improvement of SLE activity, but also of QoL as shown by the significant amelioration of PROs from the very first months of treatment. Further long-term studies on larger cohorts are needed to confirm and corroborate these results.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.