Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group’s cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The “ex-novo” occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies’ positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.

Objective To describe the cohort of patients with inflammatory rheumatic diseases (IRD) hospitalized due to SARS-CoV-2 infection in our hospital and to determine the increased risk of severe coronavirus disease regarding no IRD patients. Methods Retrospective single-center observational study of patients with IRD actively monitored in the Department of Rheumatology who were hospitalized due to COVID- 19. Results 41 (1,8%) out of 2,315 patients admitted due to severe SARS-CoV-2 pneumonia suffered from an IRD. The admission Odds ratio (OR) for IRD patients was 1.87 against the general population, and it was higher in patients with Sjögren’s syndrome, vasculitis and systemic lupus erythematosus. Twenty-seven patients were receiving treatment for IRD with corticosteroids, 23 with conventional DMARDs, 12 with biologics (7 rituximab, 4 anti-TNF and 1 abatacept) and 1 with JAK inhibitors. Ten deaths were registered among patients with IRD. A higher hospitalization rate and a higher number of deaths were observed in patients treated with rituximab (OR=12.8) but not in patients treated with anti-TNF (OR=0.9). Conclusion Patients with IRD, especially autoimmune diseases and patients treated with rituximab, may be at higher risk of severe pneumonia due to SARS-Cov 2, compared to the general population. More studies are needed to analyze this association further in order to help managing these patients during the pandemic.

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. MethodsWe conducted a multicentre, international, retrospective cohort study. Results149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. ConclusionThe clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Background Recently there have been studies showing the presence of inflammatory joint activity in patients with systemic lupus erythematosous (SLE) without musculoskeletal symptoms. The predictive value of these findings remains unknown. On the other hand, there is a proportion of healthy patients where synovitis can be demonstrated as a result of recent mechanical overexertion. Objectives The aim of this study is to compare the sonographic findings of SLE patients without joint symptoms with healthy people. Methods An ultrasonographic study (USS) was performed, using a linear probe for soft tissues (5-12 MHz), to 15 patients newly diagnosed with SLE and 44 healthy subjects matched for age and sex. The areas studied were: back of the wrist, 2nd and 3rd metacarpophalangeal joint (MCP) and 2nd and 3rd flexor tendon. The studies were performed in the nondominant hand. We determined the presence of synovitis, tenosynovitis and tendinosis and measured the thickness of the flexor tendons. Dichotomous variables were analyzed using the chi-square test and numerical variables through the Student t test. Results Controls were matched with SLE patients of the same sex and age with a maximum difference of ± 3 years. SLE patients came from a cohort previously used in another study with no less than 5 years follow-up. Controls were healthy volunteer patients from the osteoporosis clinics or patients with localized non-inflammatory diseases outside the field of USS. Carpal sinovitis was seen in 33,3% of SLE patients and 27,2% of controls (p<0,05); metacarpophalangeal (MCP) synovitis was seen in 33,3% of SLE patients and 13,6% of controls (p<0,05); carpal tenosynovitis was seen in 40% of SLE patients and 13,6% of controls (p<0,05). The height of the synovial capsule of the carpal joint was 3,67±0,21 in SLE patients and 2,99±0,30 in the control group (p<0,001); Thickness of the extensor sheath was 2,99±0,42 in SLE patients and 2,49±0,36 in the control group (p<0,05); height of the capsule of the 2nd MCP joint was 3,19±0,27 in SLE patients and 3,04±0,19 in the control group (p>0,05); height of the capsule of the 3rd MCP joint was 2,91±0,52 in SLE patients and 2,54±0,32 in the control group. In order to compare MCP capsules height we assay a corection using the lenght of the major axis of the joint. The fixed height of the 2nd MCP was 0,461±0,121 and 0,406±0,098 (SLE/controls) (p<0,001); the fixed height of the 3rd MCP was 0,445±0,200 and 0,3642±0,117 (p<0,001). Conclusions Our results suggest that the population of SLE patients without joint symptoms present sonographic findings suggestive of joint inflammation and tendon involvement that can not be explained by the acceptable changes that can be found in disease-free population, however, interpretation of their evolutionary significance has not been clarified. The role of time since diagnosis in the development of synovitis or tendinosis, or corticoids in the development of tendinosis may be revealed with studies with larger amounts of patients. Disclosure of Interest None D...

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