Bachir Mansour Diallo, Stephane Bacourine, Haby Dione, Aboubakry Sow, Adama Berthe, Papa Souleymane Toure, Madoky Magatte Diop, Mamadou Mourtalla Ka
Systemic lupus erythematosus(SLE) is an autoimmune disorder with protean manifestations. It is considered the prototype of non–organ-specific autoimmune diseases. Numerous cases have been reported worldwide. The study aims to assess the prevalence of lupus and describe its manifestations at Abdou Aziz Sy Dabakh Hospital in Senegal. We conducted a retrospective study from 2017 to 2024 at the Abdou Aziz Sy Dabakh Hospital in Tivaouane. We included all patients hospitalised or monitored for systemic lupus whose diagnosis was established according to the recommended criteria. Systemic lupus was found in 84 patients, representing a prevalence of 0.15%. The average age was 36.67 years [11 and 70 years]. The 10–30 age group was the most representative (41.6%). Females (77 patients) predominated, with a sex ratio of 0.09. The clinical manifestations were dominated by general signs such as physical asthenia (47.6%), skin signs such as non-specific erythematous lesions (15.5%) and erythematosquamous lesions (16.7%). Inflammatory polyarthritis was found in 63.1% of patients. The activity of the disease was determined using the SLEDAI 2 K score. The average was estimated at 5.74 [0–19]. Activity was severe in 22 patients (26.19%), moderate in 19 patients (22.62%), and mild in 43 patients (51.19%). Severe systemic manifestations were dominated by cardiac (9.5%), renal (7.1%), and neurological (5.9%) complications. Most patients were treated with hydroxychloroquine (88%) and azathioprine (7.14%). SLE is a multifaceted condition that requires adequate awareness among healthcare providers and the general public.
Shrinking Lung Syndrome (SLS) is a rare pulmonary complication of Systemic Lupus Erythematosus (SLE) and other connective tissue diseases. SLS is characterised by dyspnoea, restrictive lung pattern and elevated hemidiaphragms. The pathophysiology of SLS is unclear and there are currently no defined diagnostic criteria or standardised treatment guidelines. Increasing clinical awareness is essential to reduce diagnostic delay and improve patient outcomes. A literature search was conducted on the MEDLINE and Embase databases from January 1946 to 29th December 2025 to identify studies evaluating shrinking lung syndrome in patients with systemic lupus erythematosus. Data extracted included age, sex, presenting symptoms, imaging, PFTs, phrenic nerve or diaphragm studies, immunologic profile, treatment and follow-up outcomes. A total of 34 studies were included, all of which consisted of case reports, case series and one cross-sectional study. We pooled 120 patients, 86% of which were females and ages ranged from 12 to 67 years, including paediatric cases. Dyspnoea was present in 98% of patients. Elevated hemidiaphragms were seen on imaging in 78% of patients. Pulmonary Function Tests (PFTs) showed a restrictive ventilatory pattern in 91% of patients. Corticosteroids were the first-line treatment in 93% of patients. Symptomatic improvement was seen in 77% of patients. Reported follow-up periods ranged from 3 weeks to 20 years. Early recognition of SLS and tailored immunosuppressive therapy can achieve good outcomes for patients.
Matthias Dreher, Jessica Amon, Simone Cosima Boedecker-Lips, Julia Weinmann-Menke, Arndt Weinmann, Andreas Schwarting
Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by multi-organ involvement, circulating autoantibodies, and relapsing-remitting progression. Effective management is particularly challenging for less-experienced clinicians. The Mainz Lupus Score (MLS) was developed as a pragmatic clinical support tool to facilitate treatment decisions and therapy management, emphasizing both objective measures and subjective patient-reported outcomes (PROs). The aim of the study was to evaluate the Mainz Lupus Score (MLS) for assessing disease activity and guiding therapy in systemic lupus erythematosus (SLE) by comparing its performance with established indices, including the SLE Disease Activity Index (SLEDAI), the European Consensus Lupus Activity Measurement Index (ECLAM), and the Systemic Lupus Erythematosus International Collaborating Clinics index (SLICC), and by examining its association with patient-reported outcomes such as the Fatigue Severity Scale (FSMC), the Beck Depression Inventory (BDI), and the Work Ability Index (WAI). Data from 458 SLE patients at the University Medical Center Mainz were analyzed, including 93 patients with at least three visits and a total of 422 visits. The MLS incorporates four items: new lupus symptoms, quality of life (QoL), laboratory abnormalities, and organ damage. Correlations between the MLS and established indices were assessed using Spearman correlation and Kruskal–Wallis tests. Significant correlations were observed between the MLS and SLEDAI (r = 0.403), SLICC (r = 0.427), and ECLAM (r = 0.537; p < 0.0001). The MLS demonstrated strong associations with therapy escalation and remission rates, aligning with treat-to-target goals. Although correlations with subjective scores, such as FSMC, BDI, and WAI were weak to moderate, the integration of PROs offered valuable insights for therapy adjustments. When testing the MLS during everyday clinical routine, no significant difference in remission change (p = 0.9398) was found between MLS-based and medical doctor (MD)-based therapy decisions. The MLS provides an easy-to-use, evaluate approach to managing SLE, balancing objective metrics with patient-centered evaluations. While effective in distinguishing disease states and guiding therapy, broader multi-center validation is recommended to enhance its applicability.
João Francisco Abrantes, Marina Dueñas-Ochoa, Roni Meidan, David Isenberg
Systemic lupus erythematosus (SLE) is a complex autoimmune rheumatic disease characterised by autoantibody production and dysregulated B- and T- cell activation, resulting in multisystem involvement. Despite therapeutic advances, persistently active and corticosteroid-dependent disease remain a significant clinical challenge. This systematic review will analyse targeted therapies in phase II and III clinical development. We conducted a systematic review of multicentre, randomised, placebo or standard of care-controlled phase II and phase III clinical trials investigating novel targeted and biological therapies in patients with SLE. Both peer-reviewed publications and registered international clinical trials were included (search date: from 2000–January of 2026). A total of 90 targeted agents were identified, including 70 biological Disease-Modifying Anti-Rheumatic Drugs (bDMARDs) and 20 targeted synthetic DMARDs (tsDMARDs), across 770 assessed clinical trials. Of these, 67 agents had reached phase II and 23 phase III in their clinical development. At the time of writing trials were classified as completed (n = 45), active and recruiting (n = 9), active but not recruiting (n = 7), active not yet recruiting (n = 5), terminated (n = 23) and withdrawn (n = 1). The treatment of SLE is rapidly evolving towards targeted, mechanism-based interventions. Emerging therapies directed at specific immune pathways offer the potential to reduce disease activity, minimise corticosteroid use and prevent organ damage. On-going investigation of promising agents in Phase II and III trials remains essential to confirm short and long-term efficacy, safety, and optimal patient selection, facilitating the time when meaningful personalised treatment strategies in SLE can become a reality.
