Systemic sclerosis (SSc) is a connective tissue disease characterized by initial microvascular damage, immune system activation and progressive fibrosis with insufficiency of internal organs. Gastrointestinal (GI) involvement is characterized by atrophy of the smooth muscle and small bowel hypomotility, mainly resulting from an autonomic nerve dysfunction. These modifications significantly affect gut transit and nutrient absorption, thus leading to malnutrition deficit induced by malabsorption. Nutritional deficit induced by malabsorption might also lead to bone alterations. This study aims to evaluate the relationship between malnutrition and bone status. Thirty-six postmenopausal female patients fulfilling the ACR 2013 criteria for SSc underwent dual-energy X-ray absorptiometry scan (DXA) to detect quantitative lumbar spine bone mineral density (BMD) and trabecular bone score (TBS) analysis to detect bone quality. Data from DXA also allow to assess body composition and provide several quantitative parameters, including free fat mass index (FFMI) that identifies the patient with malnutrition (values <15 kg/m2 in women and 17 kg/m2 in men), according to the ESPEN criteria. Body mass index (BMI) was calculated for all SSc patients and every patient completed a diary reporting GI symptoms. Two groups of SSc patients with or without diagnosed malnutrition according to FFMI parameter were identified. Malnourished SSc patients showed significantly lower weight (p = 0.01) and BMI (p = 0.001), as well as lower serum levels of hemoglobin (p = 0.009), albumin (p = 0.002), PTH (p = 0.02) and 25OH-vitamin D (p = 0.008). DXA analysis showed significantly lower lumbar L1-L4 T-score (p = 0.009) and BMD values (p = 0.029) in malnourished SSc patients. Consistently, TBS values were significantly lower in malnourished patients (p = 0.008) and correlated with BMD (at any site) and serum albumin levels (p = 0.02). In addition, FFMI positively correlated with bone parameters as well as with symptoms of intestinal impairment in malnourished SSc patients. Finally, GI symptoms significantly correlated with BMD but not with TBS. This pilot study shows that in malnourished SSc patients (2015 ESPEN criteria: FFMI<15 kg/m2), an altered bone status significantly correlates with GI involvement, in terms of symptoms being mainly due to intestinal involvement together with the presence of selected serum biomarkers of malnutrition.

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BackgroundTMJ is involved in about 50% of JIA cases, often bilateral and asymptomatic in up to 71% of cases1. Adult patients with JIA have been shown to have, compared to healthy individuals, higher rate of dysfunction and anatomical abnormalities2. Clinical examination has been shown to have high specificity but low sensitivity in revealing TMJ inflammation3. To date MRI is the gold standard to assess TMJ involvement 4ObjectivesTo investigate the prevalence of TMJ involvement in young adults with JIA and young adults with non-JIA inflammatory rheumatisms.MethodsPatients were recruited prospectively in 2 clinical centers. Inclusion criteria were: patients <35 years diagnosed with JIA who had undergone transition from the pediatric to the adult rheumatologist, and patients diagnosed with non-JIA inflammatory arthropathies. All patients were assessed for joint count, clinical examination for TMJs (tenderness to palpation, swelling, signs of damage such as joint crepitations, lateral deviation, retrognathia and decreased mouth opening), evaluation of global disease activity with composite indexes and underwent MRI of the TMJs to detect inflammation (bone marrow edema, effusion, synovial thickening) or damage (condylar flattening, erosions, disk abnormalities); MRIs with either inflammation or damage were considered pathological. Demographic and clinical characteristics were described using frequency or median and interquartile range (IQR), depending on the distribution of the variable. Differences between groups were analyzed using the Mann-Whitney U test and the χ2 test when appropriate. The significance was set at p-value ≤ 0.05.Results19 patients were included in the JIA group and 8 patients in the non-JIA group. Patients’ demographic and disease characteristics were reported in Table 1.Abstract AB1007 Table 1 JIAn 19 Non-JIAn 8 Sex F, n (%)16 (84.2%)4 (50%) Age (ys) at diagnosis, median (IQR)8 (4-12.5)23 (20.5-25.5) Current age (ys), median (IQR)22.5 (20.2 – 27.1)29.5 (28.1-32.5) Disease duration (ys), median (IQR)16 (13-17)6 (5.8-8.5) Global disease activity, n (%)Remission12 (63.1%)4 (50%)Low1 (5.3%)2 (25%)Moderate5 (26.3%)1 (12.5%)High1 (5.3%)1 (12.5%) TMJs tenderness, n (%)4 (21.1%)2 (25%) TMJs swelling, n (%)0 (0%)0 (0%) TMJ damage, n (%)13 (68.4%)4 (50%)MRI results are collected in Table 2.Abstract AB1007 Table 2 JIATMJs n 38 Non-JIATMJs n 16 Inflammation on MRI, n (%)14 (36.8%)5 (31.2%) Joint damage on MRI, n (%)26 (68.4%)11 (68.8%) Pathological MRI, n (%)26 (68.4%)11 (68.8%) TMJ involvement, n patients (%)Monolateral2 (10.5%)1 (12.5%)Bilateral12 (63.2%)5 (62.5%)There are no statistically significant differences between groups for the presence of inflammation on MRI, while damage (in particular, disk abnormalities) is more likely in JIA rather than non-JIA patients (p= 0.02). Joint effusion is more likely to be mild rather than moderate/severe in JIA patients (p = 0.04).ConclusionIt was found that it is more likely to find damage on MRI in patients of both groups rather than inflammation. Both groups show ...

Background:Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by specific vascular and obstetric manifestations and by antiphospholipid antibodies (aPL) positivity [1].To date, little is known regarding nailfold videocapillaroscopy (NVC) alterations in APS patients and in asymptomatic aPL-carriers, non-specific abnormalities being the most frequently reported [2,3,4].Objectives:To retrospectively analyze NVC alterations in APS patients and in asymptomatic aPL-carriers and to correlate NCV alterations with both clinical manifestations and serum aPL profile.Methods:Thirty-five aPL positive patients having received at least one NCV investigation (mean age 47 years, range 16-81, 31 female and 4 male) were retrospectively included in the study. For each patient complete medical history was collected with a particular attention to past vascular thrombosis and pregnancy morbidity. Patients were classified as affected by APS according to the updated Sapporo classification criteria [5]. Lupus anticoagulant (LAC), IgM and IgG anti-cardiolipin antibodies (ACL) and IgM and IgG anti-Beta2 Glycoprotein 1 (anti-B2GP1) were assessed in each patient according to the recommended procedures [5]. NCV parameters were analyzed in each patient, with a particular interest to hemorrhages or nailfold bed-parallel hemosiderin deposits (“comb-like”hemorrhages) presence [2,6]. Statistical analysis was performed by parametric and non-parametric tests.Results:Seventeen patients (mean age 49 years, range 16-81 years) were asymptomatic aPL-carriers and 18 (mean age 46 years, range 26-71 years) were affected by APS. Within APS patients, 16 had a history of vascular thrombosis and 2 had pregnancy morbidity; in 6 patients APS was secondary to other autoimmune rheumatologic conditions (3 to Systemic Lupus Erythematosus, 2 to vasculitides and 1 to Mixed Connective Tissue Disease).Among the total number of aPL-carriers and APS patients six patients showed a normal NVC pattern, 24 patients had non-specific NVC abmormalities and 5 patients had a “scleroderma-like” pattern. Interestingly, NCV microhemorrhages were significantly more frequent in APS patients than in asymptomatic aPL-carriers, both in score and in absolute (p=0.05 andp=0.04, respectively). Particularly, in APS patients “comb-like”hemorrhages had a statistically significant higher prevalence than isolated hemorrhages (p=0.03). Dilated capillaries score was significantly higher in APS patients than in asymptomatic aPL-carriers (p=0.01).Not any statistically significant difference was observed regarding other capillary parameters (score of giant capillaries, loss of capillaries, or anormal shpaes, i.e. angiogenesis). Not any statistical correlation was observed between NVC parameters and different aPL profile.Conclusion:The study shows that the total number of microhemorrhages and in particular the“comb-like”subtype, are significantly the most frequent specific abnormalities in APL patients when compared to asymptomatic aPL carriers. The presence of the “scleroderma like” NVC pattern may suggest a concomitant overlap syndrome. Not any correlation was found between aPL profile and other NVC parameters. Further studies need to develop a more specific APS NVC pattern for APS patients.References:[1]Tektonidou MG, et al RMD Open 2019; 5(1);[2]Cutolo M, Elsevier 2010, pp141-143;[3]Candela M, et al.1998:444-9;[4]Aslanidis S, et al. Clin Exp Rheumatol 2011, 29:307-9;[5]Miyakis S, et al. J Thromb Haemost 2006, 4:295–306;[6]Cutolo M, et al Best Pract Res Clin Rheumatol 2008, 22:1093-108Disclosure of Interests: :Giorgia Ferrari: None declared, Sabrina Paolino: None declared, Alberto Sulli Grant/research support from: Laboratori Baldacci, Carmen Pizzorni: None declared, Greta Pacini: None declared, Emanuele Gotelli: None declared, Adriano Lercara: None declared, Vanessa Smith Grant/research support from: The affiliated company received grants from Research Foundation - Flanders (FWO), Belgian Fund for Scientific Research in Rheumatic diseases (FWRO), Boehringer Ingelheim Pharma GmbH & Co and Janssen-Cilag NV, Consultant of: Boehringer-Ingelheim Pharma GmbH & Co, Speakers bureau: Actelion Pharmaceuticals Ltd, Boehringer-Ingelheim Pharma GmbH & Co and UCB Biopharma Sprl, Maurizio Cutolo Grant/research support from: Bristol-Myers Squibb, Actelion, Celgene, Consultant of: Bristol-Myers Squibb, Speakers bureau: Sigma-Alpha