Chiara Cardelli

Little is known about the relationship between thyroid diseases (TDs) and Idiopathic inflammatory myopathies (IIMs). This study aimed at evaluating the pre

Background: Interstitial lung disease (ILD) is the most frequent extra-muscular manifestation in patients with idiopathic inflammatory myopathies (IIMs). Although high-resolution chest tomography (HRCT) represents the gold standard for the evaluation of ILD, lung ultrasound (LUS) might be a useful tool for its assessment. The aim of our study was to evaluate the number and distribution of pleural irregularities (PIs) identified by lung US in a cohort of patients with IIMs and to find possible correlations with clinical, serological and HRCT data to verify the potential usefulness of lung US for the study of ILD in patients with IIM. Patients and methods: Fifty-three patients with IIM according to EULAR/ACR classification criteria were enrolled. All patients underwent a clinical evaluation with measurement of disease activity and myositis-specific autoantibodies, pulmonary function tests, HRCT evaluated with the Warrick score, and lung US for the measurement of PIs. Results: The number of PIs was higher in patients with myositis-specific autoantibodies, particularly those with anti-synthetase autoantibodies (p < 0.001) and in patients with high-grade dyspnea (p < 0.03). A negative correlation was identified between PIs and pulmonary function tests, particularly TLC (r = −0.74; p < 0.001) and DLCO (r = −0.56; p < 0.001). Interestingly, PI score was higher in patients with ILD identified with HRCT (p = 0.015) and a positive correlation between PIs and Warrick score (r = 0.542; p < 0.001) was also found. Conclusions: The study of PIs with lung US represents a promising diagnostic tool for the bedside evaluation of patients with IIMs. This can possibly allow for a reduction in unnecessary HRCTs, reducing the exposition of patients to ionizing radiations, optimizing resources and reducing the costs of patients’ management.

Review; Authors: G. Cascarano, D. Zucchi, C. Cardelli, M. Oliva, G. Capozzo, A. Elia, E. Silvagni, E. Elefante, A. Bortoluzzi, C. Tani

Objectives: To describe different clinical phenotypes of severe flares in a monocentric cohort of SLE patients and to compare treatment and outcomes. Material and methods: 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. Results: 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 hyper-inflammatory 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. Conclusions: 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.

Objective: To analyze the efficacy and safety of anifrolumab, a monoclonal antibody targeting the type I interferon receptor subunit 1, as a therapeutic option in patients with refractory cutaneous dermatomyositis (DM). Methods: Patients with DM presenting with cutaneous involvement refractory to different immunosuppressive treatments were enrolled. The Cutaneous Dermatomyositis Disease Area and Severity Index activity (CDASI-A) was used to evaluate the evolution of skin involvement. Manual muscle test-8 (MMT-8) and patient global assessment (PtGA) scores were collected, to evaluate both muscle involvement and patients’ quality of life. We registered the changes in glucocorticoid (GC) daily dose, to explore the possible GC-sparing effect of the drug. Results: We enrolled 4 patients (50% female, mean age 60 years). At enrollment, they showed an active cutaneous disease (mean CDASI-A 27/100), mean MMT-8 of 67.5/80, mean daily dose of GC of 10 mg of prednisone, and mean PtGA value of 7.5/10. After treatment, all these variables improved, with mean CDASI-A decreasing to 8.75/100, MMT-8 increasing to 71.3/80, daily GC dose decreasing to 4 mg, and PtGA decreasing to 1.5/10. Conclusion: The data we collected showed a significant reduction in CDASI-A values. Further, our results showed an improvement in muscle involvement and in patients’ perception of disease burden. Finally, a GC-sparing effect and a good global safety profile of anifrolumab were observed, thus confirming anifrolumab as a new valid therapeutic option in patients with refractory cutaneous DM.

Objective: To identify different clusters of SLE patients in remission based on patient-reported outcomes (PROs) and clinical factors that predict cluster membership. Methods: A cross-sectional monocentric study of adult consecutive SLE outpatients in stable clinical remission. Clinical and laboratory data were collected. At enrolment, each patient completed PROs. A hierarchical clustering analysis based on PROs exploring fatigue (Functional Assessment of Chronic Illness Therapy), disease impact (Lupus Impact Tracker), mental and physical health (mental component summary and physical component summary of the Short Form 36) was performed. Logistic regression assessed predictors of cluster membership, adjusting for demographic and clinical variables. Results: 195 SLE patients were enrolled. Two distinct clusters emerged: a ‘low symptom burden (LSB)’ cluster, including 81/195 (41.5%) patients, and a ‘high symptom burden (HSB)’ cluster, including 114/195 (58.5%) patients. The HSB was characterised by worse scores in all PROs compared with the LSB. The HSB cluster exhibited older age at diagnosis (33.23±11.97 vs 27.75±11.57; p=0.002), less frequent previous renal involvement (33.3% vs 65.4%; p<0.001), more frequent previous neuropsychiatric lupus (17.2% vs 1.3%; p=0.001), more fibromyalgia (26.3% vs 5.3%; p=0.001) and ongoing glucocorticoid use (58.8% vs 37.0%; p=0.004). Fibromyalgia (OR 7.27, 95% CI 2.27 to 29.15, p=0.002) and glucocorticoid treatment (OR 3.05, 95% CI 1.43 to 6.77, p=0.005) were independently associated with higher likelihood of HSB membership; renal involvement was associated with LSB membership (OR 0.23, 95% CI 0.10 to 0.51, p≤0.001). Conclusions: Among SLE patients in remission, two distinct health-related quality of life phenotypes can be identified: an HSB and an LSB. Fibromyalgia and glucocorticoid treatment emerged as independent predictors of HSB. These findings underline the need to optimise disease management and align treatment goals with patient priorities.

PV238 / #532 Poster Topic: AS23 - SLE-Diagnosis, Manifestations, & Outcomes Background/Purpose: With the new millennium major changes have occurred in the management of lupus, these encompass the optimization of the use of old drugs, the identification of therapeutic targets such as LLDAS and remission and the introduction of new drugs such as biologics. The aim of the present study was to analyze how the new treatment paradigm impacted on the management of SLE over time in the real-life setting. Methods: This is a retrospective observational study on SLE patients regularly followed at our unit and with a follow-up of at least 1 year; patients were divided in 2 groups: group 1 including patients diagnosed between 1980 and 2000 and group 2 those diagnosed after 2000. Demographic characteristics, clinical manifestations at disease onset and cumulative organ involvement, disease activity, organ damage and disease state over time and treatments received were compared. Results: 428 SLE patients were included in the study, 377 females (88%), 143 patients in Group 1 (33.4%) and 285 (66.6%) in group 2. Presenting symptoms were similar between the 2 groups, while over the entire disease course we found a higher frequency of renal involvement (54% vs 40%) and neuropsychiatric involvement (17% vs 6%) in patients diagnosed before 2000. As far as therapy is concerned, almost all patients received GC during their disease course although in group 2 we found 11 patients who did never take GC (4%, p=0.02). Longitudinal analysis of the GC cumulative dose showed similar doses during the first year of the disease; however, at 5 and 10 years after diagnosis group 1 received significantly higher dosages (median 8.7 g vs 7.6 g and 15.35 g vs 14 g respectively, p<0.001 in both analyses). Interestingly, a higher percentage of patients was GC-free at 5 years from diagnosis in the group diagnosed after 2000 (29.4% vs 6.6%). 394 patients received HCQ during the disease course (93.5%), a higher proportion of patients in group 2 although not statistically different (95% vs 90%). The proportion of patients who received at least 1 IS drug within the first year of follow-up was significantly higher in patients diagnosed after 2000 (60.7% vs 39%, p<0.001) Overall, 129 patients received at least on biological drug (30%), with a significantly higher proportion in group 2 (34% vs 22%, p=0.01). In group 2, 22 (8%) patients received a biological treatment within the first year since diagnosis. At 1 year from diagnosis remission resulted more frequently achieved in patients diagnosed after 2000 (57% vs 46% respectively, p=0.03). Overall 9.7% of patients developed at least 1 item of the SLICC/DI within the first year from diagnosis (median SDI=1, range 1-3); 17.7% within 5 years (median SDI= 1, range 1-5) and 32.6% within 10 years (median SDI=1, range 1-6). The median damage score over time was similarly very low in both groups. The occurrence of renal damage (RD), cardiovascular events (CVE) and osteoporotic fractures did not show statistically significant differences between the 2 groups. Conclusions: The study highlights how the clinical management of SLE patients has changed in recent years by incorporating the new treatment paradigm based on the early use of immunosuppressive drugs and steroid-sparing strategies, resulting in more effective disease control.