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Objectives: To investigate the risk and predictors of severity and mortality of COVID-19 infection in French patients with Connective Tissue Diseases (CTDs).

Methods: Using the French nationwide claims and hospitalization database, we assembled a nation-wide exhaustive cohort of adult CTD patients with rheumatoid arthritis, systemic lupus, Sjögren's disease, inflammatory myopathies, systemic sclerosis. We analyzed the rates of hospitalization, severe inpatient stays (intensive care unit [ICU] admissions or in-hospital mortality), and in-hospital mortality due to COVID-19 from January 1 to December 31, 2020.

Results: The study included 329,276 CTD patients (75.5% female, mean age 65.2 ± 15.3 years). Among these, 3389 (1.03%) were hospitalized with COVID-19 infection, 973 (0.29%) required admission to ICU, and 713 (0.22%) died. Patients who were hospitalized, had severe inpatient stays, or died were predominantly male, older and with comorbidities (p<0.0001 for all). The risk of hospitalization, severe inpatient stay, and death was significantly higher in patients treated with glucocorticoids, leflunomide, sulfasalazine, mycophenolate derivatives, and rituximab (p<0.05 for all). TNF inhibitors were associated with reduced hospitalization and severe inpatient stays (p<0.05 for all) and methotrexate use was associated with decreased mortality (p<0.01).

Conclusion: In CTD patients with COVID-19, use of glucocorticoids, rituximab, and certain immunosuppressants was associated with severity and mortality, while TNF inhibitors and methotrexate were protective. These findings can guide clinical and public health decisions for this highly vulnerable group.

Introduction: The continuum in Still's disease has never been addressed in Afro-descendant (AD) populations. The aim of this study was to compare the features of Still's disease between children and adults in the AD population of French West Indies (FWI).

Methods: Retrospective longitudinal study from January 2000- 2022. We included children and adults with systemic juvenile idiopathic arthritis and Still's disease. Clinical data were obtained from computerized hospital archives, registries of clinicians and the national registry for rare diseases. The main outcome was similarity in cardinal and non-cardinal symptoms.

Results: Fifty-eight patients were included (57% adults). Sex distribution between children and adults was significantly different (Female respectively 36% vs 70.6%, p=0.03). Diagnostic criteria overlapped in most cases (80%), regardless of age. The children had significantly more typical skin rashes (100% vs 29.4%, p<0.001), coronary artery dilation (16% vs 0%, p=0.03), and macrophage activation syndrome (52% vs 9%, p<0.001). The adults had significantly more inflammatory polyarthalgia without arthritis (91% vs 32% p<0.001) and pulmonary involvement (51.5% vs 4% p<0.001). The phenotypes were 86% systemic (43% monophasic, 43% polycyclic) and 14% chronic polyarticular. No difference was found in the number of relapses, use of biologics and mortality.

Conclusions: There is a rationale for considering Still's disease as a single entity in our AD population, focusing on the cardinal symptoms, but particular attention should be paid to the non-cardinal symptoms depending on the age of onset.

Objectives: Lung ultrasound (LUS) has been proposed as a useful tool for the assessment of interstitial lung disease (ILD) in connective tissue diseases. However, there are no studies investigating the significance of pleural irregularities (PI) on LUS in primary Sjögren's disease (SjD) patients. The aim of this study was to explore the role of PI for the assessment of SjD-related lung involvement.

Methods: All primary SjD patients who had undergone a chest CT scan in the lasts 2 months from the start of the study were enrolled, including both SjD patients with known ILD and SjD patients without known lung involvement who underwent a chest CT due to clinical indications other than ILD screening. LUS was performed for all patients and PI total and partial scores were assigned from 0 (normal) to 2 (major changes). Based on CT scans results SjD patients were divided into 5 groups: normal CT scan, non SjD-related lung abnormalities, SjD-related non-ILD lung abnormalities, established ILD, newly diagnosed ILD.

Results: nineteen SjD patients with established ILD and 42 without known lung involvement who had undergone a CT scan were included. Among the latter, CT allowed the diagnosis of 4 new ILD cases. Both total and postero-inferior PI scores were comparable between established ILD and newly diagnosed ILD patients and significantly higher compared to patients with normal CT scan and SjD related non-ILD lung abnormalities. The AUC for ILD diagnosis was significantly higher for the PI postero-inferior score compared to the PI total score. A cut-off score of 15 for the PI postero-inferior score resulted in a sensitivity of 86.6% and specificity of 84.2% for SjD-ILD diagnosis. Both PI total and postero-inferior scores strongly correlated with HRCT Warrick score (r= 0.809 and r= 0.854). The correlation between PFT and both total and postero-inferior PI scores was higher than that observed between PFT and the Warrick HRCT score.

Conclusions: PI may represent a valid tool for the assessment of lung involvement in SjD, particularly for the screening of ILD. PI limited to postero-inferior lung fields seem to maintain good diagnostic accuracy, allowing to save time in clinical practice.

Periodontal disease (PD) and rheumatoid arthritis (RA) are both inflammatory diseases affecting the tooth and joint, with local inflammation associated with bone loss. Bacterial infections by oral bacteria are involved in periodontal inflammation, and the best known to be associated with PD is Porphyromonas gingivalis (Pg). A large body of recent data suggests a strong involvement of this specific bacteria, Pg, in PD outcomes, but also in RA. The aim of this review is to discuss the association between PD and Pg, RA and its mechanisms, and to determine whether targeting Pg bacteria could improve RA. Numerous epidemiological studies have already confirmed the association between PD and Pg, as well as between PD and RA, which is mainly associated with a common genetic background, the shared epitope. The involvement of Pg in pathogenesis of RA is supported by the induction of gingival citrullinated proteins and therefore of anti-citrullinated proteins antibodies, which constitute the most specific biomarker of RA. The prevalence of Pg in RA is still controversial, but studies should include patients with preclinical and early RA. Finally, recent data confirmed that targeting Pg is highly effective in improving RA.

Objectives Gender impact on phenotypical expression of Behçet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD. Methods Data were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behçet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout disease course, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated. Results In total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean ± SD BODI (1.92 ± 2.09) at the last follow-up, compared to female patients (1.25 ± 1.87) (p<0.0001). Uveitis (p<0.0001) and vascular involvement (p=0.0076) were significantly more frequent among males whereas female patients were significantly overrepresented in arthralgia (p<0.0001), arthritis (p=0.00025), isolated headache (p<0.0001), central nervous system (CNS) involvement (p=0.040), and gastrointestinal involvement (p=0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (p=0.617). Four variables were associated with the development of major organ involvement: male gender (OR=2.104, p=0.001), current treatment with biologic agents (OR=2.257, p=0.0003), origin from endemic countries (OR=2.661, p=0.0009), and disease duration (OR=1.002, p=0.024). Conclusion BD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression.