OBJECTIVES:To assess the utility of lung magnetic resonance imaging (MRI) for monitoring interstitial lung disease (ILD) in patients with systemic sclerosis (SSc), using high-resolution CT (HRCT) as the reference. Additionally, we explored associations between MRI sequences and common imaging and functional parameters in SSc-ILD evaluation. METHODS:SSc patients with ILD requiring treatment initiation or change underwent lung assessment at baseline and after 6 months, including MRI (T2-weighted, T1 post-contrast, and T2 star sequences), HRCT, lung ultrasound, and pulmonary function tests. Six-month MRI and HRCT were qualitatively evaluated for improvement, stability, or progression. A follow-up HRCT was performed 2 years after enrolment. RESULTS:Fourteen SSc-ILD patients (64.3% female, mean age 48.3 years) were enrolled. MRI showed improvement in 1 patient, stability in 9, and progression in 4; in 2 progressed cases, inflammation decreased while fibrotic features increased. T1 contrast sequence significantly correlated with pleural irregularities on ultrasound (ρ=0.55; p=0.04) and DLCO (ρ=-0.65; p=0.01). MRI and HRCT findings at 6 months were concordant in 64.3% of cases, with fair agreement (weighted κ=0.25). MRI outcomes at 6 months matched HRCT findings at 2 years in 92.8% of patients (13/14). CONCLUSIONS:Lung MRI is a promising adjunctive tool for monitoring SSc-ILD and may provide complementary information to HRCT on early imaging changes, particularly the transition from inflammation to fibrosis. Among MRI sequences, T1 post-contrast best correlated with functional and ultrasound findings, supporting its role in fibrosis assessment.

OBJECTIVE:Mycophenolate mofetil (MMF) use in limited cutaneous systemic sclerosis (lcSSc) is relatively uncommon because of the lower fibrotic burden and the predominance of vascular complications. In vitro observations and clinical data from transplanted patients suggest a protective effect of MMF on endothelial function. Our aim was to evaluate the reasons for prescribing MMF treatment in patients with lcSSc and its impact on the need for escalation of vascular complication-related treatments during follow-up. METHODS:Patients with lcSSc enrolled in the Italian Systemic Sclerosis Progression Investigation registry were retrospectively evaluated. All patients treated with MMF were matched to patients not treated with MMF, which was based on a roll-entry time-dependent propensity score built on demographics, clinical features, and baseline treatment. The escalation of vasoactive or vasodilator treatment up to 60 months was defined as the introduction of iloprost, endothelin receptor antagonists, or phosphodiesterase-5 inhibitors on top of the ongoing treatment, because of uncontrolled or newly diagnosed vascular complications. A hazards Cox model was also adopted to quantify the association of MMF treatment with treatment escalation. RESULTS:A total of 1,435 patients with lcSSc were evaluated, of whom 152 were prescribed MMF (17.1% male; mean age at lcSSc onset 48.7 ± 13.9 years, 54.6% anti-Scl70 positive). The prescription of MMF was more common in men and in anti-Scl70 positive, anticentromere negative patients with interstitial lung disease, myositis, and without a history of digital ulcers. After matching 107 patients with MMF-untreated controls, the overall incidence of vasoactive/vasodilator treatment escalation events related to digital ulcers over a median follow-up of 40.5 months (interquartile range 23.3-60.0) was 0.3 per 100 patient-years in the MMF-treated group and 5.4 per 100 patient-years in the matched control group, with a significant difference in treatment escalation-free survival between the two groups (hazard ratio 0.05, 95% confidence interval 0.01-0.38; P value = 0.004). CONCLUSION:In patients with lcSSc, the introduction of MMF has reduced the need for escalation of vasoactive or vasodilator treatment, suggesting that it may also help to prevent vascular complications, which frequently affect patients with lcSSc.

OBJECTIVES:In clinical practice, standardised reporting of nailfold videocapillaroscopy (NVC) findings is lacking, making the interpretation and comparison of results difficult. We aimed to achieve a national consensus on how to describe NVC findings in routine clinical practice. METHODS:A web-based Delphi consensus study was conducted among members of the Study Group on Capillaroscopy and Microcirculation in Rheumatic Diseases of the Italian Society of Rheumatology (CAPSIR). The study was based on items derived from a previous systematic review and international consensus by the EULAR Study Group on Microcirculation in Rheumatic Diseases (SG_MC/RD). RESULTS:A total of 40 items were proposed during the Delphi process, which was completed by 52 participants from different Italian regions. An agreement was reached on 23 items covering different aspects of the NVC examination: general aspects (2 items), description of the fingers examined (3 items), possible confounding factors (2 items), device description (2 items), image quality (1 item) and details of the NVC examination (13 items). Sixteen of these were considered mandatory for inclusion in the NVC practice report, and 7 were considered optional. CONCLUSIONS:The proposed NVC checklist covers 23 relevant issues in clinical practice, including 16 mandatory items grouped into five categories. This national consensus will improve the reproducibility and generalisability of NVC reporting in daily clinical practice. Furthermore, the outcomes of this NVC consensus process will inform the next European web-based Delphi consensus study, to be conducted among the member countries of the EULAR SG_MC/RD.

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.

Little is known about the relationship between thyroid diseases (TDs) and Idiopathic inflammatory myopathies (IIMs). This study aimed at evaluating the prevalence of TDs in a monocentric cohort of patients with IIMs, exploring possible correlations with clinical phenotype, organ involvement, comorbidities and quality of life (QoL). We retrospectively analysed medical records of patients with IIM according to the EULAR/ACR 2017 criteria, collecting data about demography, IIM subset, disease duration, autoantibody profile, organ involvement and comorbidities. Besides, we registered the occurrence of Hashimoto Thyroiditis (HT), Multinodular goitre (MNG), Grave’s Disease (GD) and Thyroid papillary cancer (TPC). In addition, some different patient reported outcomes were administered, to collect data on QoL. A total of 191 patients were enrolled: 125 (65.4

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases characterised by skeletal muscle inflammation and frequently by involvement of other organs, in particular lung and skin, but also joints, heart and gastrointestinal tract. Although they are rare diseases, the literature on IIMs has been growing rapidly and many studies have been published in order to clarify pathogenesis and to better define diagnosis, clinical manifestations (muscular and extramuscular) and treatment.The purpose of this review article is to summarise the most relevant contributions published over the last year on this topic.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of rare connective tissues diseases that usually share the common feature of immune-mediated muscle or lung injury. Whereas their pathogenesis is widely recognised as multifactorial, the specific triggers initiating their onset remain largely elusive. Factors such as infections, inhalants, or geoclimatic variables are implicated, yet due to the limitations inherent in studies involving small and non-homogeneous cohorts, findings often appear fragmented and inconclusive. This review endeavours to present the most updated evidence regarding the influence of environmental factors in determining the onset of IIM, with the aim of offering insight to optimise the routinary management of affected patients.

ObjectiveBosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the Systemic Sclerosis Progression Investigation (SPRING) registry.MethodsPatients with SSc from the SPRING registry, meeting 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria with data on PAH onset, DU status, BOS exposure, and at least 1 year of follow-up between 2015 and 2020, and having no known PAH at baseline, were included. PAH was diagnosed with right heart catheterization during the follow-up, and its incidence rate (IR) was calculated. Kaplan-Meier curves were determined, and multivariate regression identified PAH risk factors.ResultsAmong 727 eligible patients with SSc, followed for a median of 2.0 years, 54 (7.4%) developed PAH (IR 3.71 per 100 patient-years [PYs]). Patients with DU who were never exposed to BOS had a higher incidence of PAH (IR 4.90 per 100 PYs) compared to those exposed to BOS, whose rates matched those without DU and who were never exposed to BOS. Risk factors independently associated with PAH development included DU (hazard ratio [HR] 1.86), age (HR 1.05), modified Rodnan skin score > 4 (HR 2.07), interstitial lung disease (HR 2.29), and acetylsalicylic acid treatment (HR 1.78).ConclusionIn our cohort, the presence of DU was confirmed as a leading risk factor for PAH development, and BOS use for DU prevention may reduce this risk. Only patients with DU who were not using BOS had an increased PAH incidence.

Digital ulcers (DU) are one of the most frequent manifestations in systemic sclerosis (SSc). The presence of DU seems to be a sentinel sign of internal organ involvement and is related to a poor prognosis of the disease. The aim of this study was to evaluate the prevalence and the relationship of DU with clinical manifestations/variants in a large SSc cohort from the SPRING registry. SSc patients fulfilling the ACR/EULAR 2013 classification criteria without missing data on digital ulcers were enrolled in a cross-sectional study. Logistic regression models were built to test the association between the presence of DU and SSc-related features. Among 1873 eligible SSc patients, the presence of DU was significantly associated with gastrointestinal involvement (OR 1.88, 2.04 and 1.74; p < 0.001) and serum ATA positivity (OR 2.15; p < 0.001), as well as with telangiectasias, sclerodactyly, digital pitting scar, and calcinosis (OR 1.40, p = 0.005; OR 3.43, p < 0.001, OR 9.12, p < 0.001 and OR 2.77, p < 0.001; respectively). In the multivariable regression models, even after adjustment for covariates, ATA positivity (OR 1.76, p = 0.039), puffy fingers (OR 2.82, p < 0.001), and a higher revEUSTAR-AI (OR 6.63, p < 0.001) emerged as risk factors for the presence of DU. Moreover, a low presence of DU was recorded in SSc patients with a history of previous immunosuppressive treatments (OR 0.53, p = 0.032). In our Italian SSc cohort, DUs were significantly associated with the presence of puffy fingers, high revEUSTR-AI, and ATA seropositivity. Noteworthy, immunosuppressive treatments were associated with a low rate of DU, suggesting that they might contribute to the prevention of these harmful manifestations. Key Points • Digital ulcers were significantly associated with the presence of puffy fingers, high disease activity, and anti-Scl70 seropositivity. • Immunosuppressive treatments were associated with a low rate of digital ulcers, suggesting that they might contribute to the prevention of these harmful manifestations.

Background: Hormonal changes in menopause might interact with the presentation of underlying autoimmune diseases, such as systemic sclerosis (SSc). Objectives: Our study aimed to evaluate the association of (1) current menopausal status, (2) early menopause, and (3) disease onset during fertile or post-menopausal age on SSc clinical phenotype in a large SSc cohort from the Italian Systemic sclerosis Progression INvestiGation (SPRING-SIR) registry. Design: Female SSc patients from the SPRING-SIR registry, fulfilling the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 classification criteria, with data on SSc disease onset, menopausal status, and menopausal age, were eligible. SSc onset was categorized as pre-menopausal if SSc onset happened >1 year before menopause or as post-menopausal onset if it occurred >1 year after menopause. An early menopause was defined by a menopausal age <45 years. Methods: Descriptive statistics and regression models were built to test the association between current menopausal status, pre-menopausal disease onset, and early menopause with SSc-related features. Results: At baseline, 1157/1538 (75%) patients were in menopause, 632 (50.4%) had a pre-menopausal SSc onset, and 130 (14.4%) reported an early menopause. Post-menopausal patients had more frequent limited cutaneous SSc, anti-centromere antibody positivity, interstitial lung disease, and gastrointestinal manifestations. Pre-menopausal onset cases showed more frequent diffuse cutaneous involvement and peripheral vasculopathy. Patients with early menopause had more frequent peripheral vasculopathy and interstitial lung disease, being early menopause an independent risk factor for digital ulcers and lower diffusing capacity of the lung for carbon monoxide. Conclusion: Current post-menopausal status and early menopause may impact SSc presentation, being associated with vascular and gastrointestinal manifestations. Menopausal status and age should therefore be thoroughly addressed, aiming at better disease management.

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases characterised by skeletal muscle inflammation and frequently by other organs involvement, in particular lung and skin, but also joints, heart and gastrointestinal tract.Although they are rare diseases, the literature on IIMs has been growing rapidly and many studies have been published in order to clarify the pathogenesis and to better define diagnosis, clinical manifestations (muscular and extra-muscular) and treatment. The purpose of this review is to summarise the most relevant contributions published over the last year on this topic.

Objective Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry. Methods Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included. PAH was diagnosed with right heart catheterization during the follow-up, and its incidence rate (IR) was calculated. Kaplan-Meier curves were determined, and multivariate regression identified PAH risk factors. Results Among 727 eligible patients with SSc, followed for a median of 2.0 years, 54 (7.4%) developed PAH [IR 3.71 per 100 patients/years]. Patients with DU who were never exposed to BOS had a higher incidence of PAH [IR 4.90 per 100 patients/years] compared to those exposed to BOS, whose rates matched those without DU and who were never exposed to BOS. Risk factors independently associated with PAH development included DU (HR 1.85), age (HR 1.05), modified Rodnan Skin Score (mRSS) >4 (HR 2.07), ILD (HR 2.29), and acetylsalicylic acid treatment (HR 1.78). Conclusion In our cohort, DU were confirmed as a leading risk factor for PAH development, and BOS use for DU prevention may reduce this risk. Only patients with DU who were not on BOS had an increased PAH incidence.

Idiopathic inflammatory myopathies are a group of rare, autoimmune, diseases typically involving striate muscle and also variously affecting several other systems or organs, such as joints, skin, lungs, heart and gastrointestinal tract. IIM are mainly characterised by subacute onset and chronic course and are burdened by significant morbidity and mortality. Despite the rarity of these conditions, several efforts have been undertaken in the last years to better understand their pathogenesis, as well as to achieve a more precise classification and to define the optimal therapeutic approach. The aim of this review is to provide an up-to-date digest of the most relevant studies published on this topic over the last year.

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. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.Figure 1

Background:Intravenous iloprost has been widely used for the treatment of systemic sclerosis peripheral vasculopathy. No agreement has been found on the regimen and the dosage of intravenous iloprost in different scleroderma subset conditions. This study aimed to evaluate the modalities of intravenous iloprost administration within a large cohort of systemic sclerosis patients from the SPRING Registry and to identify any associated clinical-demographic, instrumental or therapeutic data. Patients and Methods:Data of systemic sclerosis patients treated with intravenous iloprost for at least 1 year (case group) were retrospectively analyzed, including different timing and duration of intravenous iloprost session, and compared with those of untreated patients (control group). Results:Out of 1895 analyzed patients, 937 (49%) received intravenous iloprost treatment, while 958 (51%) were assigned to the control group. Among cases, about 70% were treated every 4 weeks, 24% with an interval of more than 4 weeks, and only 6% of less than 4 weeks. Most patients receiving the treatment every 4 weeks, or less, underwent infusion cycle for 1 day only, while if it was scheduled with an interval of more than 4 weeks, a total number of 5 consecutive days of infusions was the preferred regimen. The comparison between the two groups revealed that patients treated with intravenous iloprost had a higher frequency of DUs (p < 0.001), pitting scars (p < 0.001), diffuse cutaneous involvement (p < 0.001), interstitial lung disease (p < 0.002), as well as higher rates of anti-topoisomerase I, "late" scleroderma pattern at nailfold videocapillaroscopy. These findings were confirmed by multivariate analysis. Conclusion:Our data provide a picture on the Italian use of intravenous iloprost among systemic sclerosis patients and showed that it was usually employed in patients with a more aggressive spectrum of the disease. The disparity of intravenous iloprost treatment strategies in the different centers suggests the need of a rational therapeutical approach based on the clinical characteristics of different patients' subsets.

Objective:To optimise the organisation of care and encourage the adoption of good clinical practices, the RarERN Path© methodology was designed within ERN ReCONNET. The aim of our work was to report the application of RarERN Path© on systemic sclerosis within the ERN ReCONNET centres, providing a feasible and flexible organisational reference model for optimising the systemic sclerosis care pathway in different countries. Methods:RarERN Path© is a six-phase methodology which enables the creation of a reference organisational model co-designed on the basis of the expertise of different stakeholders. It foresees six phases, ranging from the map of existing patients' care pathways and patients' stories, to the consensus on a common organisational patient care pathways, and its key performance indicators definition. Results:The agreed reference model highlights the importance of having an organisational flow for referrals that foresees how patients may access directly the specialised unit from the different referrals. Specific specialised visits were considered as mandatory to be organised and they included cardiologist, pneumologist, gastroenterologist, psychologist, nephrologist, dermatologist, wound care specialist/nurses and other healthcare professionals (such as nurses, social workers and nutritional counselling). Moreover, specific services related to therapy were highlighted as strongly recommended to be organised, mainly represented by infusion therapy and wound care, as well as occupation therapy and physiotherapy. Conclusion:The organisational model emerged from our investigation emphasises that the organisation of specific services for systemic sclerosis treatment should be organised as a solid support for implementing the existing recommendations on systemic sclerosis management in real life.

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.Female 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.Figure 1Number of comorbidities in our cohort Table 1Distribution 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 fibrillation52,8Icuts31,7

Background: To assess skin involvement in a cohort of patients with systemic sclerosis (SSc) by comparing results obtained from modified Rodnan skin score (mRSS), durometry and ultra-high frequency ultrasound (UHFUS). Methods: SSc patients were enrolled along with healthy controls (HC), assessing disease-specific characteristics. Five regions of interest were investigated in the non-dominant upper limb. Each patient underwent a rheumatological evaluation of the mRSS, dermatological measurement with a durometer, and radiological UHFUS assessment with a 70 MHz probe calculating the mean grayscale value (MGV). Results: Forty-seven SSc patients (87.2% female, mean age 56.4 years) and 15 HC comparable for age and sex were enrolled. Durometry showed a positive correlation with mRSS in most regions of interest (p = 0.025, ρ = 0.34 in mean). When performing UHFUS, SSc patients had a significantly thicker epidermal layer (p < 0.001) and lower epidermal MGV (p = 0.01) than HC in almost all the different regions of interest. Lower values of dermal MGV were found at the distal and intermediate phalanx (p < 0.01). No relationships were found between UHFUS results either with mRSS or durometry. Conclusions: UHFUS is an emergent tool for skin assessment in SSc, showing significant alterations concerning skin thickness and echogenicity when compared with HC. The lack of correlations between UHFUS and both mRSS and durometry suggests that these are not equivalent techniques but may represent complementary methods for a full non-invasive skin evaluation in SSc.

Background 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. Objectives 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. Methods 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. Results 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. Conclusion 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. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests None Declared.