BackgroundSystemic lupus erythematosus (SLE) is a complex autoimmune disease with 0.4 million new cases diagnosed annually. With its wide variety of visible and invisible manifestations, people living with SLE report being exposed to stigmatization, which impacts their personal and professional lives. However, the current literature is unclear on whether healthcare management teams assess this concern during follow-up. This study aims to synthesize existing evidence on the prevalence and determinants of stigma among people living with SLE.MethodsThis systematic review and meta-analysis gathered evidence from observational studies identified from three databases on 16 July 2025. Dual independent screening, data extraction, and risk-of-bias assessment (using the Newcastle-Ottawa Scale) were performed. Results were synthesized using descriptive statistics, narrative synthesis, and indicator-level meta-analyses.ResultsWithin the past two decades, 11 studies comprising 2254 people living with SLE reported and measured stigma- and discrimination-related events using various scales. Stigma was found to be prevalent across its three constructs: interpersonal, perceived, and intrapersonal stigma. This review demonstrated that people living with SLE reported a moderate overall burden of stigma (34.71 [95% CI 26.15, 43.27]), with average stigma scores indicating psychological impact. Additionally, nearly one in two persons (46% [95% CI 28-66%]) experienced at least one form of stigma or discrimination, most commonly social isolation and unfair treatment. Mental health associations were correlated with higher stigma burden.ConclusionThis review demonstrates that stigma and discrimination are not just social challenges but also critical determinants of health. With cautious interpretation, pooled evidence reveals a consistent high prevalence of stigma and discrimination, which act as "toxic" stressors, creating a vicious cycle with psychological stress and psychiatric manifestations and disease activity. There is an urgent clinical need to move beyond a mere biological approach to disease assessment and management and to begin screening for the "invisible" burden of invalidation and discrimination.

Objective Patients with SLE have increased risk of both clinical cardiovascular disease (CVD) and subclinical atherosclerosis. Reports have shown that controlling CVD risk factors reduces subclinical plaque progression in patients with SLE. We investigated whether this finding was confirmed in our ethnically diverse cohort of patients, measuring total plaque area (TPA) as well as the number of plaques.Methods 69 patients with SLE underwent ultrasound scans of the carotid and common femoral arterial bifurcations on two occasions (mean 63 months apart). Clinical, demographic, CVD risk and treatment factors were recorded for each patient. Change in plaque number and increase in TPA between scans were the outcome measures.Results 31 patients had plaque at the second scan. 13 had unchanged number of plaques while 18 had increased plaque numbers including six who were initially free of plaque. All 31 patients had increased TPA with median increase 4.59 mm2/year (IQR 2.4–7.33) and these patients were subdivided into two groups with change in TPA above or below the median. Factors associated with both increased plaque number and above-median TPA increase at the second scan compared with the first were age at baseline, positive lupus anticoagulant, negative anti-La and failure to attain at least three CVD risk targets within the follow-up period between scans. We found no associations with disease activity or medication.Conclusion In this ethnically diverse (40% non-Caucasian) population, we confirmed earlier findings that better control of CVD risk targets reduces progression of atherosclerotic plaque. Anti-La positivity was associated with less plaque progression, which was unexpected.

Abstract Background/Aims Studies in many units have looked at associations of sex and ethnicity with clinical manifestations and outcomes in patients with systemic lupus erythematosus (SLE). Some reports have suggested that male patients have a more severe form of disease than female patients but small numbers hamper analysis. Non-white patients have worse outcomes in some studies but this may be partly related to socio-economic factors such as access to healthcare. In previous studies of our large lupus cohort, we found no difference related to ethnicity or sex in terms of survival but non-white patients were more likely to develop nephritis. In this study, we looked at manifestations on presentation to our unit, use of immunosuppressant medications and development of damage. Methods The Lupus Clinic at University College London Hospital has been running since 1978. We carried out detailed analysis of the medical and research records of 655 patients with SLE who had been recruited between 1978 and 2023. Forty patients were excluded because they were only followed up for a very short time and/or because key information was not available. For the remaining 615 patients, ethnicity was classified as Caucasian, Afro-Caribbean, South Asian, East Asian or Mixed Race/Other. Sex was classified as male or female. The five outcome measures analysed were ever-use of cyclophosphamide, mycophenolate or biologics, lifetime damage and manifestations at presentation. Damage was assessed using the Systemic Lupus International Collaborative Clinics Damage Index. The symptoms at presentation were classified as mild (mucocutaneous, musculoskeletal and/or changes in blood counts only) or severe (any other presentation). Differences between groups were analysed statistically using the Chi squared test. Results There were 54 male and 561 female patients. The breakdown of ethnicity was Caucasian 329, Afro-Caribbean 132, South Asian 89, East Asian 42, Mixed Race/Other 23. The outcome measures are shown in Table 1. Conclusion Male patients had more severe manifestations and developed more damage but did not differ in terms of use of medications. Caucasian patients had less severe manifestations at presentation and were less likely to be treated with cyclophosphamide, mycophenolate or biologics but developed more damage. Disclosure M. Pisliakova: None. N. Rege: None. G.S. Uzun: None. R. Abida: None. D.A. Isenberg: None. A. Rahman: None.

Abstract Background/Aims Patients with systemic lupus erythematosus (SLE) have increased risk of both clinical cardiovascular disease (CVD) and subclinical atherosclerosis. Reports from a mono-ethnic population in Greece have shown that controlling CVD risk factors reduces subclinical plaque progression in patients with SLE. We investigated whether this finding also holds in our ethnically diverse cohort of patients, measuring total plaque area (TPA) as well as number of plaques. Methods Sixty-nine patients with SLE but with no previous history of clinical CVD underwent ultrasound scans of both carotid and both common femoral artery bifurcations (four sites in all) on two occasions, a mean of 63 months apart. Clinical, demographic, CVD risk and treatment factors were recorded for each patient. We assessed use of medications and time in low disease activity state or remission between scans. We enumerated the number of CVD risk targets (BMI, blood pressure, smoking cessation and low density lipoprotein) achieved for each patient. Targets were as defined in the 2021 European Society for Cardiology guidelines for prevention of CVD. The outcome measures were change in number of sites with plaque (range 0-4) and total plaque area (TPA) between scans. We carried out statistical analysis to identify factors that were significantly associated with an increase in plaque number and/or with increased TPA. Results Thirty-one of 69 patients had plaque at the second scan, of whom 18 had an increased number of plaque sites since the first scan, while 13 had an unchanged plaque number. TPA had increased in all 31 patients with a median increase of 4.59mm2/year. Increased plaque number was associated with increased age at baseline (p < 0.001), positive lupus anticoagulant (p = 0.006), negative anti-La (p = 0.027) and failure to attain at least three CVD risk targets by the time of the second scan (p < 0.005). Increased TPA was also associated with increased age at baseline (p < 0.001), positive lupus anticoagulant (p = 0.018), negative anti-La (p = 0.023) and failure to attain at least three CVD risk targets by the time of the second scan (p < 0.001). We found no associations of either outcome with cumulative steroid dose between scans, use of immunosuppressants or time in remission/low activity state. Conclusion In this ethnically diverse (40% non-Caucasian) population, we confirmed earlier findings that better control of CVD risk targets reduces progression of atherosclerotic plaque. Anti-La positivity was associated with less plaque progression, which was unexpected. Lupus anticoagulant positivity was associated with increased plaque progression, consistent with previous reports. Disclosure J. Bakshi: None. R. Abida: None. S. Croca: None. M. Griffin: None. K. Chandwar: None. F. Farinha: None. T. McDonnell: None. D.A. Isenberg: None. A. Nicolaides: None. A. Rahman: None.

Objective The question of sustained remission in SLE has been highlighted recently following data published on the use of chimeric antigen receptor-T cell therapy in SLE. With the review, we wanted to investigate the prevalence of sustained remission of 2 years or more in SLE with the current available treatments and to assess the predictive factors associated with this state.Methods A systematic review of Embase via Ovid and Medline via Ovid was performed. We considered articles of adult patients with SLE that report the prevalence and/or the predictive factors of sustained remission in SLE. We considered that the latter state is sustained if it was for 2 years or more.Results 20 studies were included for final review. The definition of remission was different between studies, and most authors considered different types of remission based on the serological activity and the current therapy. The prevalence of long-term remission was different across studies depending on the definition used. It ranged from 0.3% to 63%. Older age at diagnosis, lower disease activity at baseline and the absence of renal disease involvement were the most commonly found factors likely associated with sustained remission.Conclusion Long-term remission is an achievable state in SLE with current treatment. Studies report a striking heterogeneity in the current prevalences. This is likely to be caused by the lack of objective measures to confirm the state of sustained remission in SLE.

Sarcoidosis often manifests with pulmonary involvement, making isolated extrapulmonary presentations rare and diagnostically challenging. We present the case of a 43-year-old woman who presented with prolonged fever, deep lymphadenopathy, massive splenomegaly, hepatomegaly, and severe hypercalcemia, mimicking malignancy. The diagnosis of sarcoidosis was established after the failure of anti-tubercular therapy, exclusion of differential diagnosis, and the subsequent development of cutaneous sarcoids. This case highlights the importance of recognizing isolated extrapulmonary pseudo-tumoral presentations of sarcoidosis, which can lead to delayed diagnosis.

Antinuclear antibodies are present in high titers in over 95% of patients with systemic lupus erythematosus (SLE). The precise targets of these antibodies are, however, highly variable and include double-stranded (ds)DNA, histones, ribonucleoprotein (RNP), and Sm antigens. The presence of antinuclear antibodies (ANAs) is a criterion for SLE but these antibodies are not specific and may be found in healthy individuals (some, but not all, of whom will go on to develop lupus) and those with infections in whom the ANA is a transient phenomenon. Titers of ANA are of little use in reflecting disease activity in SLE.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a heterogeneous course and systemic involvement. It is the result of a complex pathogenic pathway that culminates in autoantibody formation. The interaction between environmental triggers and genetic susceptibility is key in this process. Genome-wide association study technology has allowed the recognition of >80 loci associated with SLE that lead to the formation of key proteins, each contributing a small increase to the risk. Advances in the management of the disease include new validated standardized tools to capture disease activity, damage and quality of life, for clinical and research purposes. The prognosis of SLE has much improved in the last 50 years because of better general management and specific treatment, including better use of immunosuppressive agents and development of a new group of drugs – biologic therapies.

Systemic lupus erythematosus (SLE) is characterized by increased expression of type I interferon (IFN)-regu-lated genes in 50%-75% of patients. We report that out of 501 patients with SLE analyzed, 73 (14%) present autoantibodies against IFNa (anti-IFN-Abs). The presence of neutralizing-anti-IFN-Abs in 4.2% of patients inversely correlates with low circulating IFNa protein levels, inhibition of IFN-I downstream gene signatures, and inactive global disease score. Hallmarks of SLE pathogenesis, including increased immature, double -negative plasmablast B cell populations and reduction in regulatory B cell (Breg) frequencies, were normal-ized in patients with neutralizing anti-IFN-Abs compared with other patient groups. Immunoglobulin G (IgG) purified from sera of patients with SLE with neutralizing anti-IFN-Abs impedes CpGC-driven IFNa-dependent differentiation of B cells into immature B cells and plasmablasts, thus recapitulating the neutralizing effect of anti-IFN-Abs on B cell differentiation in vitro. Our findings highlight a role for neutralizing anti-IFN-Abs in con-trolling SLE pathogenesis and support the use of IFN-targeting therapies in patients with SLE lacking neutral-izing-anti-IFN-Abs.

Systemic lupus erythematosus (SLE) is one of the most studied autoimmune diseases. The interest shown for this pathology is translated into international scientific journals, congresses, meetings and, recently, in large data available online. Social networking sites (SNS) have gradually advanced from ways to facilitate interpersonal relations to important sources of information, including medical data regarding SLE, with sites largely accessed by both doctors and patients. Albeit the use of SNS can be valuable in providing education and promoting development of public health, it can be misleading if unprofessional sources of information are used; therefore, both "friends and foes" of the data accessed on large scale should always be considered. This viewpoint is a discussion of the potential benefits and harms related to the SNS use for SLE patients as well as for their physicians.

Oral cavity involvement in tuberculosis (TB), particularly palatine, is extremely rare and mostly described in case reports. Management of these cases usually responds to classic antitubercular therapy. Some serious complications such as paradoxical reactions (PRs) may however occur, making it more challenging for physicians to treat and to manage. We present a case of a 30-year-old female patient with a history of juvenile idiopathic arthritis and systemic lupus erythematosus who presented a bifocal form of TB involving the palate and the cervical lymph nodes. Follow-up after 2 months of proper antitubercular treatment revealed a PR of the lymph nodes contrasting with a favorable outcome of the oral lesions. It seems useful to raise all clinicians' awareness to suspect TB when they deal with chronic drug-resistant oral erosions and to keep in mind the diagnosis of PR when there is a worsening of one lesion and a favorable outcome of another.

Diagnosis of pheochromocytoma can be simple when classic manifestations are present. It can also be challenging and complicated in some cases because of its wide array of faces and presentations. We present a case of a 30-year-old female patient who came with acute respiratory distress, chest pain, hemoptysis, asthenia, anorexia, weight loss of 20kg, and paresthesia in her lower limbs. Clinical examination found high blood pressure, accelerated heart and respiratory rates, signs of acute right heart failure with jugular venous distention and ankle edema, reticularis livedo in the four limbs, ulcers in both knees and in the 3rd metacarpo-phalangeal articulations and necrotic lesions in both calcaneal tendons and in the right toes. Further investigations concluded on myocarditis associated with alveolar hemorrhage, pericardic and pleuritic effusions and a segmental pulmonary embolism of the right inferior lobe. Neuro-muscular biopsy was suggestive of myositis. Cutaneous biopsy found nonspecific chronic dermatitis. ANCA antibodies were tested twice and were negative. Cryoglobulinemia was also negative. Thoraco-abdomino-pelvic scan was performed showing a large right adrenal mass suggestive of pheochromocytoma. Diagnosis of right adrenal pheochromocytoma was confirmed by MIBG-I123 hyperfixation findings and urinary normetanephrin levels. The patient was treated surgically. Postoperative outcomes were remarkably favorable with a complete regression of the cutaneous lesions and normalization of the blood pressure. Paresthesia significantly decreased. Control echocardiography at 3 months showed an improved heart function with a persistent apical and septal akinesis.

Anti-glomerular basement membrane (anti-GBM) disease was usually described as a small vessel vasculitis presenting with acute kidney injury, haematuria and non-nephrotic proteinuria. We report a case of anti-GBM disease revealed by an intense nephrotic syndrome. The urinary protein level was 12g/day. Renal biopsy only showed crescent glomerulonephritis with linear staining of IgG in direct immunofluorescence without other glomerulonephritis. Immunoglobulin G (IgG) anti-GBM antibody titer was elevated.

Objective To identify clinical and serological features that distinguish patients with SLE who require single as opposed to repeated rituximab (RTX) cycles. Methods All 175 SLE patients followed up at University College Hospital from 2000 onwards were retrospectively reviewed. They were divided into a one-RTX-cycle group and a multiple-cycle group (2 or more cycles). Patients included had a follow-up of at least 3 years after their first RTX cycle, unless they needed a second infusion sooner. Results A total of 131 patients were included; 44 (33.6%) received one cycle of RTX and 87 (66.4%) received two or more. The former were older at diagnosis (31.4 vs 21 years, P < 0.001) and at first RTX infusion (39.9 vs 29 years, P < 0.001). This group of patients had more organs/systems involved (P = 0.044), more leukopenia, lymphopenia and thrombocytopenia (P = 0.001, P < 0.0001 and P = 0.003, respectively) and lower C3 levels (P = 0.035). They also had fewer immunosuppressive drugs before RTX therapy compared with those who required multiple RTX cycles (P = 0.003). There was no statistical difference in either the clinical or serological response after the first RTX cycle between both groups. Furthermore, patients who had received more immunosuppressive treatments were more likely to require more than one cycle of RTX infusions (P = 0.007). Conclusions RTX is an effective option for SLE patients with severe flares. Patients who received more immunosuppressive drugs were more likely to receive more than one set of RTX infusions. This suggests that RTX is best used for SLE patients with no history of refractory disease.

La maladie de Degos-Köhlmeier ou papulose atrophiante maligne est une entité rare. Presque 200 cas dans le monde ont été décrits. Nous rapportons le cas d’une maladie de Degos survenue chez un patient de 51 ans révélée par des accidents vasculaires cérébraux (AVC) ischémiques récidivants. Il s’agit d’un patient âgé de 51 ans sans antécédents familiaux ou personnels notables qui nous a été adressé pour exploration d’AVC ischémiques à répétition. Il se plaignait de céphalées hémicrâniennes gauches associées à une irritabilité importante et à des troubles mnésiques. L’examen a trouvé un réflexe styloradial vif diffusé des 2 côtés avec extension de la zone réflexogène, un réflexe achilléen plus vif à droite et une parésie du nerf oculomoteur droit. L’examen cutané a objectivé des lésions papuleuses hypochromiques millimétriques dont certaines sont légèrement atrophiques au niveau du tronc, du dos et des bras. Le reste de l’examen était sans anomalies. L’exploration biologique était sans particularités hormis une légère hypercholestérolémie. L’imagerie cérébrale par résonance magnétique a objectivé des lésions ischémiques d’allure récente intéressant le territoire des artères cérébrales postérieures gauche et droite, une sténose serrée des artères cérébrales moyennes droite et gauche et de l’artère cérébrale postérieure gauche sans autres anomalies. L’échographie cardiaque transthoracique et trans-œsophagienne ainsi que l’échographie doppler des troncs supraaortique étaient sans anomalies. L’examen ophtalmologique était également normal. La recherche d’une thrombophilie constitutionnelle (Protéines C et S, résistance à la Protéine C activée) ou d’un syndrome des antiphospholipides était négative. L’étude histologique des lésions cutanées était caractéristique d’une papulose atrophiante avec des images d’ischémie dermique triangulaire dont la pointe se situe dans le derme profond bordée par un infiltrat lymphocytaire modéré. Le diagnostic de maladie de Degos a donc été retenu. Nous avons prescrit un traitement antiagrégant au long cours. Après un an de suivi, le patient n’a pas présenté de récidive thrombotique. La papulose atrophiante est une vasculopathie thrombo-oblitérante à expression essentiellement cutanée. Elle se manifeste typiquement par des lésions à centre blanc-porcelaine atrophique et à bordure télangiectasique. Si la forme cutanée bénigne évolue favorablement, les formes systémiques, de cette maladie, en l’occurrence neurologiques, peuvent être graves et engagent le pronostic fonctionnel voire vital du malade. Les mécanismes de survenue de ces complications restent inconnus du fait de sa rareté et de sa complexité. La papulose atrophiante est entité qui mérite une attention particulière de par sa gravité potentielle. L’étude des mécanismes étiopathogéniques peut être amorcée par une investigation approfondie des 200 cas déjà décrits dans la littérature.

Les thromboses veineuses superficielles ont reçu un regain d’intérêt remarquable ces dernières années. Longtemps réputées bénignes, elles sont désormais considérées comme redoutables par la présence potentielle d’une étiologie sous-jacente. Nous proposons d’étudier le profil étiologique des thromboses veineuses superficielles dans un service de médecine interne. Étude rétrospective incluant les patients hospitalisés entre 2000 et 2016 dans un service de médecine interne ayant présenté une thrombose veineuse superficielle. Le diagnostic positif de la thrombose a été posé grâce à l’échographie Doppler du réseau veineux. Quarante-quatre (44) dossiers ont été retenus. L’âge moyen était de 48,2 ans (25–78 ans). Le sex-ratio était H/F = 0,69. Les thromboses veineuses superficielles siégeaient dans 75 % des cas au niveau des membres inférieurs et dans 25 % des cas au niveau des membres supérieurs. Aucun cas de grossesse, de post-partum ou de contraception œstro-progestative n’avait été retrouvé. On avait noté un antécédent de maladie veineuse thromboembolique chez cinq patients (11,3 %) et un antécédents de néoplasie chez 2 sujets (4,5 %). La recherche d’une thrombophilie ou d’une néoplasie sous-jacente n’était pas systématique. L’existence d’un contexte favorable évident ou d’une affection pouvant expliquer le tableau avaient permis de sursoir à une recherche étiologique plus poussée, ainsi, un contexte postopératoire avait été retenu dans 2 cas, une obésité était retrouvée dans 6 cas et 16 patients avaient une insuffisance veineuse. Par ailleurs, 3 patients étaient suivis pour un lupus érythémateux systémique, 2 patients pour une bêta-thalassémie majeure, un patient pour une maladie de Behçet, un patient était suivi pour une néoplasie du poumon et un patient pour une néoplasie thyroïdienne. Le bilan étiologique réalisé avait noté une résistance à la protéine C activée chez 2 malades, un déficit en protéine C chez un seul malade, une hyperhomocystéinémie dans un cas et une hyperhomocystinurie dans un autre cas. La thrombose veineuse superficielle s’est compliquée d’une embolie pulmonaire chez 2 patients. Une récidive thrombotique avait été notée chez cinq malades. La thrombose veineuse superficielle fait partie intégrante de la maladie veineuse thromboembolique. Son diagnostic impose une prise en charge adéquate et une étude étiologique raisonnée.