BACKGROUND:Behçet's disease (BD) is a multisystem inflammatory disorder primarily affecting mucocutaneous tissues. Central nervous system (CNS) involvement, Neuro-BD (NBD), is a potentially severe manifestation of BD with a variable prevalence (1-59%). AIMS:We aimed to characterize BD patients with CNS involvement and to identify predictors of this clinical subtype. METHODS:We performed a single-centre observational retrospective study using data from patients fulfilling the 2013 International Criteria for BD registered at the Rheumatic Diseases Portuguese Registry (Reuma.pt) between October 2014 and May 2023. NBD was defined according to International Consensus Recommendation Criteria for NBD diagnosis. Demographic, clinical and treatment data were collected. RESULTS:We included 157 patients with BD, 116 (74%) of them were females and 127 (81%) Caucasian with a mean±SD age at diagnosis of 32.0±12.2 years and the median (IQR) follow-up was 11.0 (11.8) years. NBD was diagnosed in 24 (15%) patients, with two (1%) cases identified at disease onset. The mean age at NBD diagnosis was 31.6±8.7 years, occurring 4.7 (1.0) years after BD onset. Among NBD patients, 15 (63%) had parenchymal, eight (33%) non-parenchymal and one (4%) mixed CNS involvement. Compared to patients without NBD, those with NBD were more likely to be non-Caucasian (27% vs 17%, p=0.026), have genital ulcers at disease onset (68% vs 34%, p=0.002) and present with ocular manifestations during the disease course (58% vs 35%, p=0.028). NBD patients more frequently received systemic glucocorticoids (100% vs 78%, p=0.008) and conventional synthetic disease-modifying anti-rheumatic drugs (86% vs 62%, p=0.035), particularly cyclophosphamide (48% vs 1%, p<0.001). Mortality was also significantly higher among NBD (13% vs 2%, p=0.026). On multivariable analysis, genital ulcers at presentation (OR 4.50, 1.68-12.06) and ocular involvement during the disease course (OR 2.69, 1.01-6.76) were independent predictors of CNS involvement. CONCLUSION:In summary, our cohort showed a 15% prevalence of CNS involvement, with parenchymal affection being the predominant form. Genital ulcers at onset and ocular manifestations emerged as independent predictors of NBD, serving as potential clinical markers for identifying high-risk patients. The high mortality rate observed among these patients underscores the severity of NBD and the critical need for prompt recognition.

Existing guidelines for systemic lupus erythematosus (SLE) predominantly focus on common and major organ involvements. An international taskforce involving experts from three SLE expert groups (ie, the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases, the Systemic Lupus Erythematosus International Collaborating Clinics group, and the European Lupus Society) was established. A total of 119 participants contributed to the development of consensus therapeutic strategies for 24 rare SLE manifestations, using a multistep process. For SLE enteritis and pancreatitis, experts recommended hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil. Rare lung conditions such as pneumonitis were also managed with cyclophosphamide if severe or with mycophenolate mofetil if not severe. SLE for myocarditis with hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil, are recommended based on severity. For CNS manifestations, hydroxychloroquine, glucocorticoids, and cyclophosphamide or mycophenolate mofetil were common choices for treatment. For rare skin manifestations, the preferred strategy was a combination of hydroxychloroquine and glucocorticoids with anifrolumab or mycophenolate mofetil. This expert-based consensus provides a valuable framework for guiding therapeutic decisions where the available recommendations might be insufficient or inapplicable.

OBJECTIVES:To compare proliferative (PLN) and membranous (MLN) lupus nephritis (LN) regarding clinical and laboratory presentation and long-term outcomes, and to investigate predictors of progression to chronic kidney disease (CKD). METHODS:Multicentre observational study, with retrospective analysis of a prospective cohort, using data from the Rheumatic Diseases Portuguese Registry - Reuma.pt. Patients with biopsy-proven PLN, MLN and mixed LN were included. Cox regression survival analysis was used to investigate predictors of CKD. RESULTS:A total of 260 patients were included. Median follow-up was 8 years (IQR 11; minimum 1, maximum 35 years). MLN patients presented with significantly lower serum creatinine [0.70 (IQR 0.20; minimum 0.50, maximum 1.30) mg/dl vs 0.80 (IQR 0.31; minimum 0.26, maximum 2.60) in PLN, P = 0.003]. Proteinuria levels did not differ between groups (P = 0.641). Levels of complement were reduced in PLN but nearly normal in MLN patients, and there were fewer patients with positive anti-dsDNA antibodies in the MLN group (P < 0.001). One year after the beginning of treatment, 62% of the patients achieved EULAR/ERA-EDTA complete response, with a further 5% achieving partial response. Patients with lower proteinuria at diagnosis were more likely to achieve a complete renal response at one year; however, proteinuria at diagnosis or at one year did not predict long-term CKD. Estimated glomerular filtration rate (eGFR) ≤75 mL/min/1.73 m2 at one year was the strongest predictor of progression to CKD (HR 23 [95% CI 8-62], P < 0.001). Other possible predictors included the use of azathioprine for induction of remission, older age at diagnosis and male sex. CONCLUSION:Proteinuria levels did not predict LN histologic class in our cohort. eGFR cutoff of 75 mL/min/1.73 m2 after one year of treatment was strongly predictive of progression to CKD.

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.

Objectives To compare the prevalence of anxiety and depression in patients with GCA with that in the general population, using the Hospital Anxiety and Depression Scale (HADS), and to identify independent predictors of these psychiatric manifestations in patients with GCA.Methods We conducted a cross-sectional study including all patients diagnosed with GCA followed during 1 year in a vasculitis outpatient clinic. The HADS and 36-item Short Form (SF-36) questionnaires were prospectively collected. Patients' HADS results were compared with an age- and gender-matched control group. HADS anxiety (HADS-A) and HADS depression (HADS-D) scores between 8 and 10 defined possible anxiety and depression and >= 11 defined probable anxiety and depression, respectively.Results We included 72 patients and 288 controls. Compared with controls, patients with GCA had a statistically significant higher prevalence of HADS-A >= 8 (48.6% vs 26.4%), HADS-A >= 11 (30.6% vs 12.2%) and HADS-D >= 11 (33.3% vs 18.1%). GCA was an independent predictor of HADS-A >= 8 [odds ratio (OR) 3.3 (95% CI 1.9, 5.9)], HADS-A >= 11 [OR 3.8 (95% CI 2.0, 7.4)] and HADS-D >= 11 [OR 2.6 (95% CI 1.4, 4.7)]. Among patients with GCA, a negative correlation was observed between HADS-A/D and SF-36 mental health scores (r = -0.780 and r = -0.742, respectively). Glucocorticoid therapy was a predictor of HADS-A >= 8 [OR 10.4 (95% CI 1.2, 94.2)] and older age of HADS-D >= 8 [OR 1.2 (95% CI 1.1, 1.3)] and HADS-D >= 11 [OR 1.1 (95% CI 1.0, 1.2)].Conclusions Compared with the general population, patients with GCA have a higher prevalence of anxiety and depression and GCA is an independent predictor of these symptoms. Glucocorticoid treatment and older age are predictors of anxiety and depression, respectively, in patients with GCA. What does this mean for patients?Giant cell arteritis (GCA) is a condition that causes inflammation of the arteries, most frequently in the head and neck. It affects predominantly the elderly and can potentially lead to stroke and blindness. Both GCA-related symptoms and treatment with glucocorticoids can impact mental health. We compared the prevalence of anxiety and depression in individuals with GCA with that of the general population using the Hospital Anxiety and Depression Scale (HADS). We found that GCA was an important contributor to a higher prevalence of anxiety and depression in this group compared with the general population. We also explored which aspects contributed the most to anxiety and depression in people with GCA. We found that treatment with glucocorticoids was a predictor for anxiety and older age was a predictor for both anxiety and depression. Moreover, we described a negative correlation between anxiety/depression scores and quality of life among people with GCA, which suggests an impact of mental health on overall well-being. These findings highlight the burden of anxiety and depression in GCA, emphasizing the need for awareness and physician attention to mental health in this population in order to improve overall care and elevate the quality of life for individuals with GCA.

(1) Background: Patients with systemic lupus erythematous (SLE) experience profound effects on health-related quality of life (HRQoL) that cannot be explained by objective indicators of mortality and morbidity. This study aimed to adapt the SLE Quality of Life (SLEQoL) questionnaire to the European Portuguese population and to assess its reliability and validity for patients with SLE. (2) Methods: Two independent translators translated the original version of the SLEQoL questionnaire into Portuguese. A back-translated version was produced. The Portuguese version of the questionnaire was reviewed and tested for validity and reliability. Cronbach’s alpha and the internal validity index were calculated to verify the internal reliability and validity of the content. Rheumatologists filled out the SLE Disease Activity Score (SLE-DAS) and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index SLICC/ACR-DI questionnaires. (3) Results: This study involved 180 patients, of which 93.8% were females. The results indicated very high internal consistency reliability (α = 0.949), low correlations between the SLEQoL and the SLE-DAS, a correlation between all SLEQoL dimensions and all SF-36 dimensions (except for “response to treatment” and “self-image”), and good correlation scores with both the EQ-5D-5L index and VAS. (4) Conclusion: The Portuguese version of the SLEQoL questionnaire is valid and reliable for the measurement of HRQoL in SLE patients.

A 25-year-old woman with a recent diagnosis of SSc (anti-topo 1), expressed by progressive scleroderma, oesophageal dysmotility, serositis and RP, presented with bilateral shoulder pain and limited mobilization. The complaints prompted an US scan, which showed no synovitis or tendinopathy. However, intra-articular calcium deposits stood out, adhering to the hyaline cartilage of both humerus heads and moving with it on dynamic exploration (Fig. 1). Deposits on the glenoid labrum were also documented. There were no signs of skin calcinosis , and peripheral joint US and radiographs were normal. Secondary causes of calcium deposition disease were excluded. Calcinosis is a frequent manifestation of SSc and is caused by the deposition of insoluble calcium in soft tissues, especially in the skin and the s.c. tissue [1]. Of interest, to the best of our knowledge, there are no previous reports of isolated IA calcinosis. Calcium crystal arthropathies are mainly...

Seixas, Maria Inês MD; Marques, Tiago MD; Ferreira, Cristina MD; Romeu, José Carlos MD; Macieira, Carla MD Author Information

Non-infectious uveitis (NIU) is a group of sight-threatening diseases that generates significant burden for the healthcare systems due to its adverse outcomes, irreversible structural complications in the eye with loss of visual function, limited clinical expertise and low-grade evidence for best practice. The usefulness of multidisciplinary care, specifically close collaboration between Rheumatologists and Ophthalmologists in NIU, has been emphasized in the literature. In this paper, the assessment tools and protocols used in our clinic are depicted and an overview of our activity with a brief description of the patients included in our registry, between 2018 and 2020 is provided. The cohort of 290 patients assessed in our NIU clinic, their demographics, sources of referral, details about immunosuppression treatment, and internal and external collaborations is described. This experience-based manuscript aims to describe the general functioning of our multidisciplinary NIU clinic, highlighting the benefits and drawbacks of multidisciplinary team management in patients with NIU, ultimately initiating a dialogue on what an NIU clinic should be and providing information for newly NIU clinics start-up. In conclusion, establishing a standardized and multidisciplinary clinic in NIU allows to systematically observe and follow-up this infrequent disease at a tertiary hospital level, thus improving quality of care delivery and research avenues.

The coronavirus disease 2019 (COVID-19) pandemic has come with many challenges for healthcare providers and patients alike. In addition to the direct burden it has placed on societies and health systems, it had a significant impact in the care of patients with chronic diseases, as healthcare resources were deployed to fight the crisis, and major travel and social restrictions were adopted. In the field of rheumatology, this has required notable efforts from departments and clinicians to adapt to the novel status quo and assure the follow-up of patients with rheumatic and musculoskeletal diseases. In the present viewpoint, we provide a practical approach to tackle this reality. Key measures include setting up preventive team management strategies, optimising communication with patients and reorganising patient care in all its dimensions. We then anticipate the nuances of rheumatology practice as restrictive measures are progressively lifted, while an effective vaccine is still pending. This includes the need to reimpose the same strategy as further waves unfold. Finally, we look ahead and address the lessons we can incorporate into post-COVID-19 rheumatology.

Background Lupus Nephritis (LN) is one of the most severe manifestations of Systemic Lupus Erythematosus (SLE). We aimed to compare proliferative (PLN), membranous (MLN) and mixed LN regarding clinical and laboratory presentation, and serologic profiles. Previous work suggested that these groups differ in autoantibody profile and complement levels, but those reports mainly originate from single-centres. Methods Multicentre observational study, with retrospective analysis of a prospective cohort, using data from the Portuguese registry of rheumatic diseases–Reuma.pt. Patients with biopsy-proven PLN, MLN and mixed LN were included. The first renal biopsy showing one of these classes was considered, for each patient. Groups were compared using Pearson's Chi-Square for categorical variables and One-Way ANOVA or Kruskal-Wallis for numerical variables. Results 232 patients were included (87% females; 88.5% White Europeans). Median follow-up was 7 years (IQR 10.75; maximum 35 years). As seen in table1, the level of proteinuria did not differ between groups; however, MLN patients presented with significantly lower creatinine. Levels of complement were reduced in PLN and mixed LN but were normal in MLN patients, and this difference was statistically significant. Groups also differed regarding the proportion of positivity for anti-dsDNA (higher in PLN) and anti-RNP antibodies (higher in MLN). There was a lower SLEDAI in MLN, probably linked with the lower prevalence of anti-dsDNA antibodies and complement consumption in this group. Conclusions Our results support previous findings from single-centre studies suggesting that MLN has a different serological profile than PLN, possibly reflecting different pathogenesis.

Background The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. Results A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet’s disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment ( p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported. Conclusions Reuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, allowing an efficient data-repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking.