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Patients with familial mediterranean fever (FMF) often present with musculoskeletal involvement typical of spondyloarthropathy (SpA) or ankylosing spondylitis (AS), posing a diagnostic challenge for medical practitioners and leading to the description of the FMF-related SpA/AS clinical spectrum. Currently, the available data focuses on SpA diagnosis in patients with known FMF, while the contrary is rarely reported in the medical literature. We describe an unusual case of concomitant FMF diagnosis in a patient with an eight-year long history of typical, human leukocyte antigen-B27 positive AS on adalimumab treatment, who presented with recurrent febrile attacks and abdominal pain. The laboratory work-up revealed high titres of serum amyloid A while genetic testing was positive for the pathogenic M694V heterozygous variant in the MEFV gene. The patient was promptly treated with colchicine, showing complete remission of clinical symptoms and normalisation of inflammatory markers to date. We also performed a review of the available literature elaborating on the interrelationship of AS and FMF in terms of pathogenesis and clinical characteristics. Our case highlights the need for reporting of similar cases and further explores the association of AS and FMF as distinct clinical entities or as constituents of the same disease continuum model., Competing Interests: Declarations. Conflict of interest: The authors declare no competing interests. Ethical approval: This manuscript is compliant with ethical standards. Consent to participate: The patient provided informed consent to participate for the purpose of publication of this case report. Consent for publication: The patient has provided written consent for publication of this case report., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Systemic lupus erythematosus (SLE) is characterised by increased cardiovascular morbidity and mortality risk. We aimed to examine the prevalence of traditional cardiovascular risk factors and their control in an international survey of patients with systemic lupus erythematosus., Methods: In this multicentre, cross-sectional study, cardiovascular risk factor data from medical files of adult patients (aged ≥18) with SLE followed between Jan 1, 2015, and Jan 1, 2020, were collected from 24 countries, across five continents. We assessed the prevalence and target attainment of cardiovascular risk factors and examined potential differences by country income level and antiphospholipid syndrome coexistence. We used the Systemic Coronary Risk Evaluation algorithm for cardiovascular risk estimation, and the European Society of Cardiology guidelines for assessing cardiovascular risk factor target attainment. People with lived experience were not involved in the research or writing process., Findings: 3401 patients with SLE were included in the study. The median age was 43·0 years (IQR 33-54), 3047 (89·7%) of 3396 patients were women, 349 (10.3%) were men, and 1629 (48·1%) of 3390 were White. 556 (20·7%) of 2681 patients had concomitant antiphospholipid syndrome. We found a high cardiovascular risk factor prevalence (hypertension 1210 [35·6%] of 3398 patients, obesity 751 [23·7%] of 3169 patients, and hyperlipidaemia 650 [19·8%] of 3279 patients), and suboptimal control of modifiable cardiovascular risk factors (blood pressure [target of <130/80 mm Hg], BMI, and lipids) in the entire SLE group. Higher prevalence of cardiovascular risk factors but a better blood pressure (target of <130/80 mm Hg; 54·9% [1170 of 2132 patients] vs 46·8% [519 of 1109 patients]; p<0·0001), and lipid control (75·0% [895 of 1194 patients] vs 51·4% [386 of 751 patients], p<0·0001 for high-density lipoprotein [HDL]; 66·4% [769 of 1158 patients] vs 60·8% [453 of 745 patients], p=0·013 for non-HDL; 80·9% [1017 of 1257 patients] vs 61·4% [486 of 792 patients], p<0·0001 for triglycerides]) was observed in patients from high-income versus those from middle-income countries. Patients with SLE with antiphospholipid syndrome had a higher prevalence of modifiable cardiovascular risk factors, and significantly lower attainment of BMI and lipid targets (for low-density lipoprotein and non-HDL) than patients with SLE without antiphospholipid syndrome., Interpretation: High prevalence and inadequate cardiovascular risk factor control were observed in a large multicentre and multiethnic SLE cohort, especially among patients from middle-income compared with high-income countries and among those with coexistent antiphospholipid syndrome. Increased awareness of cardiovascular disease risk in SLE, especially in the above subgroups, is urgently warranted., Funding: None., Competing Interests: Declaration of interests AGS has received speaker fees from Merck and Schering-Plough, Bristol Myers Squibb, UCB, Pfizer, Novartis, Lilly and Women's College Hospital, Toronto, ON, Canada. AMK has received speaker fees from Boehringer Ingelheim and Sanofi; has participated on advisory boards for Pfizer, Gilead, and Boehringer Ingelheim; and has received congress sponsorship from Pfizer, Celgene, UCB, Mylan, and Roche. GJP-E has received grants from Janssen; consulting fees from GSK, AstraZeneca, Janssen, Novartis, and Bago; speakers fees from GSK, Werfen, Janssen, AstraZeneca, and Novartis; support for attending meetings and travel from GSK, AstraZeneca, and Boehringer Ingelheim; and for participation on a data safety monitoring board or advisory board from RemeGen, AstraZeneca, and Janssen. GAK has received consulting fees from Janssen and Scipher; and for participation on a data safety monitoring board or advisory board from Janssen. MFU-G has received grant support from Janssen and Pfizer; has been a speaker for GSK and AstraZeneca; and has been a member of advisory boards for AstraZeneca and Ferrer. NC-C has received grants from Roche and UCB. EH has received consulting fees and meeting fees from Johnson & Johnson, Boehringer Ingelheim, Bayer, GSK, Roche-Chugai, and Sanofi-Genzyme; speaking fees from Johnson & Johnson, GSK, and Roche-Chugai; and research funding from Commonwealth Serum Laboratories Behring, GSK, Roche-Chugai, and Johnson & Johnson. NP has received grants from Gilead Sciences Hellas and the European Centre for Disease Prevention and Control. OAM has received speaker's fees or payment for advisory boards from AbbVie, APSEN, AstraZeneca, Boehringer Ingelheim, Celltrion, GSK, and Janssen. MS has received research grants and consulting fees, and has participated as a speaker for: AbbVie, Bristol Myers Squibb, GSK, Janssen, Lilly, Pfizer, Roche, and AstraZeneca. ACSM has received speaker fees from GSK and AstraZeneca. All other authors and SURF-SLE and APS Collaborators declare no competing interests., (Copyright © 2024 Elsevier Ltd. 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Vaccination against Sars-CoV-2 has been proven to significantly reduce COVID-19 morbidity and mortality and is therefore recommended for the general population, and especially for seniors with impaired immunity. However, it is currently postulated that COVID-19 vaccines could rarely induce autoimmune diseases in previously healthy individuals. We report a case of new-onset anti-melanoma differentiation-associated protein 5 (anti-MDA5) antibody-positive dermatomyositis in a patient presenting with rash and fever following the third dose of COVID-19 vaccine. The laboratory testing revealed high titres of anti-MDA-5 antibody and chest computed tomography showed micronodular lesions and ground glass opacities bilaterally. The patient was promptly treated with corticosteroids, methotrexate, and azathioprine, and was later started on rituximab due to dermatomyositis rash exacerbation along with newly formed, diffuse skin ulcers. Our case highlights the potential immunogenicity of COVID-19 vaccines and the need for further reporting of rare rheumatic syndromes possibly related to COVID-19 disease and vaccination., Competing Interests: The authors declare no conflict of interest., (© 2024 The Mediterranean Journal of Rheumatology (MJR).)

Objectives: We aimed to perform a bibliometric analysis of original research articles on Behçet's syndrome (BS) published over the last 20 years prior to the COVID-19 pandemic, and to systematically describe their characteristics and citation records., Methods: The PubMed database was searched for any article published on BS between 2000 and 2019. We identified all original research articles and categorised them by country of origin and type of research, i.e., clinical, translational and basic. Each article's impact was assessed using the individual citation numbers from Google Scholar search engine; we also calculated the median annual citation rates (ACRs), both per country and research type., Results: Of a total of 2,381 retrieved original articles from 51 countries, the majority reported on clinical (52.6%), followed by translational (46.0%) and basic research (1.4%). Turkey had the highest number of publications (39% of articles) followed by four countries (Korea, China, Japan, Italy) where BS is also relatively prevalent. However, regarding median ACRs, France was first, followed by the United Kingdom, Germany and Collaboration. Although the number of articles has almost doubled between 2010-2019 versus 2000-2009, median ACRs across either clinical or translational research had a downwards trend., Conclusions: Researchers from countries where BS is prevalent are more productive, albeit their work is of lower impact compared to countries with generally higher research budgets. A considerable increase of original research articles on BS is observed over time but further funding may be warranted for a parallel increase in the respective scientific impact.

Cariprazine (CAR) is an antipsychotic drug for the treatment of schizophrenia (SCZ) and bipolar disorder (BD), and it acts as a partial agonist on the dopamine receptors (DR), D2, and D3. Although many single nucleotide polymorphisms (SNPs) in genes coding for these receptors are known to influence response to antipsychotics, to date, no study on CAR pharmacogenetics exists. In this pilot study, we investigated the relationship between SNPs in DRD2 (rs1800497 and rs6277) and DRD3 (rs6280), and response to CAR treatment, evaluated by the psychometric Brief Psychiatric Rating Scale (BPRS), in a cohort of Caucasian patients. We found a significant association between DRD2 rs1800497 and rs6277 and response to CAR treatment. When genotypes were combined into an arbitrary score, the receiver operating characteristic curve analysis showed that using a cut-off value of -2.5 the response to CAR treatment could be predicted with a positive likelihood ratio of 8.0. Our study report, for the first time, a correlation between SNPs in DRD2 and response to CAR treatment. After confirmation in a larger cohort of patients, our results could open the way for the identification of new tools for the provision of response to CAR treatment., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 De Pieri, Ferrari, Marino, Traber, Bolla and Cosentino.)

Background: Cardiovascular disease (CVD) is the foremost cause of morbidity and deaths in antiphospholipid syndrome (APS), driven by thrombo-inflammation and atherothrombosis mechanisms. Metabolic syndrome (MetS) is a proinflammatory and prothrombotic state characterized by increased CVD risk. We aimed to evaluate the prevalence of MetS in APS patients compared to rheumatoid arthritis (RA) and diabetes mellitus (DM) and its associations with clinical and laboratory patient characteristics and vascular ultrasound (US) markers of subclinical atherosclerosis., Methods: We included 414 patients in our study: 138 patients with APS (median age: 44.9 years, females 70%) and matched 1:1 for age and sex RA and DM subjects. Three sets of criteria were used for MetS diagnosis: Joint Interim Statement (JIS), International Diabetes Federation (IDF) and modified National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATPIII). The demographic, clinical and laboratory characteristics of all participants were recorded and carotid and femoral US was performed in patients with APS. Multivariate regression models were applied., Results: Prevalence of MetS was 23.9%, 23.2%, 20.3% (based on JIS, IDF, modified NCEP-ATPIII criteria, respectively) in APS versus 17.4%, 17.4%, 13% in RA (p=0.181, p=0.231, p=0.106, respectively), and 44.2%, 44.2%, 40.6% in DM patients. In multivariate analysis, patients with systemic lupus erythematosus- related APS had an approximately 2.5-fold higher risk of MetS versus RA patients. MetS in APS was independently associated with arterial thrombosis (Odds ratio 3.5, p=0.030). Odds ratio for MetS was 1.16 for each one unit increase in C-reactive protein levels according to JIS and IDF criteria, and 1.49 and 1.47 for each one unit increase in uric acid levels using the IDF and modified NCEP-ATPIII models, respectively. APS patients with atherosclerotic carotid plaques had 4 to 6.5-fold increased risk of MetS. Odds for MetS were decreased by 26% with an increase in physical activity by one hour per week., Conclusions: MetS is present in approximately one-fourth of APS patients at a comparable prevalence to that observed in patients with RA. MetS in APS is associated with arterial thrombosis, cardiovascular risk biomarkers, physical activity, and subclinical atherosclerosis, supporting its role in cardiovascular risk stratification and management in APS., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Bolla, Tentolouris, Sfikakis and Tektonidou.)

Background: Crisis Resolution Home Treatment (CRHT) is an alternative to inpatient treatment for acute psychiatric crises management. However, evidence on CRHT effectiveness is still limited. In the Canton of Ticino (Southern Switzerland), in 2016 the regional public psychiatric hospital replaced one acute ward with a CRHT. The current study was designed within this evaluation setting to assess the effectiveness of CRHT compared to standard inpatient treatment., Methods: CRHT was offered to patients aged 18 to 65 with an acute psychiatric crisis that would have required hospitalization. We used a natural experiment based on geography, where intervention and control groups were formed according to the place of residence. Primary endpoints were reduction of psychiatric symptoms at discharge measured using the Health of the Nation Outcome Scales, treatment duration in days, and rate and length of readmissions during a two-year follow-up period after discharge. Safety during the treatment period was measured with the number of serious adverse events (suicide/suicide attempts, major self-harm episodes, acute alcohol/drug intoxications, aggressions to caregivers or family members). We used linear, log-linear and logistic regression models with propensity scores for the main analysis., Results: We enrolled 321 patients; 67 were excluded because the treatment period was too short and 17 because they were transferred before the end of the treatment. Two hundred thirty-seven patients were available for data analysis, 93 in the intervention group and 144 in the control group. No serious adverse event was observed during the treatment period in both groups. Reduction of psychiatric symptoms at discharge (p-value = 0.359), readmission rates (p-value = 0.563) and length of readmissions (p-value = 0.770) during the two-year follow-up period did not differ significantly between the two groups. Treatment duration was significantly higher in the treatment group (+ 29.6% on average, p-value = 0.002)., Conclusions: CRHT was comparable to standard hospitalization in terms of psychiatric symptoms reduction, readmission rates and length of readmissions, but it was also characterized by a longer first treatment period. However, observational evidence following the study indicated that CRHT duration constantly lowered over time since its introduction in 2016 and became comparable to hospitalization, showing therefore to be an effective alternative also in terms of treatment length., Trial Registration: ISRCTN38472626 (17/11/2020, retrospectively registered)., (© 2022. The Author(s).)

Introduction: Cariprazine is a third-generation antipsychotic, approved for the treatment of schizophrenia and bipolar disorder and used off-label for schizoaffective disorder and treatment-resistant depression. Cariprazine is a partial agonist at dopamine receptors D2 and D3 and serotonin receptor 5HT1A and an antagonist at serotonin receptors 5HT2B and 5HT2A. It is metabolized by CYP3A4 in desmetyl-cariprazine and didesmethyl-cariprazine, both active metabolites with a half-life of 1-2 days and 2-3 weeks, respectively., Case Report: Here we show the cases of 3 outpatients diagnosed with bipolar I disorder (two patients) and schizoaffective disorder (one patients) and characterized by low adherence to treatment, satisfactory cognitive and personal functioning and average disease severity to whom we administered cariprazine as a monotherapy, on a two-times a week schedule (i.e., every 72-96 h). We evaluated response to treatment and disease remission according to conventional definitions, using rating scales BPRS, PANSS and BDI-II. Two-times a week treatment was set either after a disease relapse (one patient), after a sustained remission obtained with daily administration of cariprazine (one patient) or since our first evaluation (one patient). After 4 weeks of treatment all three patients satisfied criteria for response to treatment and remission, a result that was sustained for 8 (in one patients) and 12 months (in other two patients) and still ongoing., Discussion: Reported results support our hypothesis that long half-lives of cariprazine and its metabolites provide an adequate therapeutic response with a two-times a week administration. In selected patients, cariprazine administered as a "oral long-acting" seems effective in treating acute episodes of illness and in sustaining remission, combining advantages of oral and long-acting injectable antipsychotics concerning therapeutic alliance., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Dyrmishi, De Pieri, Ferrari, Traber, Preve, De Peri and Bolla.)