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PurposeThe aim of this study is to evaluate the retinal microvascular density in SLE patients using optical coherence tomography angiography (OCTA) and to correlate vascular density with the disease activity and damage risk.MethodsTwenty eyes of 20 SLE patients were compared with 20 eyes of normal subjects. The retinal capillary plexuses were examined by OCTA. The disease activity and damage risk were evaluated by the SLEDAI-2 K and SLICC/ACR SDI scoring systems.ResultsNo difference was found between SLE patients’ central foveal thickness (CFT) and foveal avascular zone (FAZ) area and the normal (P > 0.05). SLE patients had slightly lower superficial vessel densities than normal in the upper and lower macular regions (P < 0.05), sparing the middle sectors (P > 0.05). In the deep plexus, vessel density loss was detected in all sectors (P < 0.001). The vessel density in 300-μm-wide region around the FAZ (FD-300) and the acircularity index (AI) were affected in the SLE in comparison to the normal group (P < 0.05). No significant correlation was found between the SLEDAI-2 k and the retinal vessel density in either layer, while the SLICC/SDI had moderate inverse correlation with vessel density in some sectors (P < 0.05). Receiver operating characteristic (ROC) curve analysis showed that the deep capillary plexus had high sensitivity and specificity for detecting vascular damage in SLE patients.ConclusionsOCTA permits noninvasive quantitative assessment of retinal vessel density in SLE, allowing early detection of altered retinal circulation. Vessel density could be included in future assessment of SLE activity and damage scores.

Objective Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease in which early and atypical presentations frequently challenge existing classification frameworks. The Systemic Lupus Erythematosus Risk Probability Index (SLERPI) was developed as a probabilistic diagnostic aid, but its real-world performance relative to established classification criteria across disease phenotypes remains incompletely characterized. Methods In this multicenter, cross-sectional study, we evaluated 1,281 participants, including 655 expert-confirmed SLE patients and 626 controls with other rheumatic diseases. Diagnostic performance of SLERPI, ACR-1997, SLICC-2012, and EULAR/ACR-2019 criteria was assessed against expert clinical diagnosis as the reference standard. Subgroup analyses were performed for early disease (≤ 1 year), sex, disease duration, and major organ involvement. Concordance and discordance between criteria were examined using UpSet plots, detailed phenotypic comparisons, and hierarchical cluster analysis of discordant cases. Net reclassification improvement (NRI) was used to quantify incremental diagnostic information. Results All four systems demonstrated high diagnostic accuracy, with sensitivities ranging from 95.1–99.2% and specificities from 87.7–90.4%. SLERPI achieved the highest sensitivity (99.2%) and AUC (0.989), particularly excelling in early disease (≤ 1 year, sensitivity 98.0%, AUC 0.987). Net reclassification improvement favored SLERPI over ACR-1997 (+ 2.7%), SLICC-2012 (+ 1.6%), and EULAR/ACR-2019 (+ 4.3%). Concordance across systems was substantial, with 91.6% of patients classified by all four sets. Discordant cases (8.4%) revealed phenotype-specific patterns: ACR-1997 frequently missed immunologically active or hematologic-dominant cases, while EULAR/ACR-2019 underperformed in mucocutaneous-predominant disease. Cluster analysis identified four coherent subgroups, underscoring heterogeneity in missed classifications. SLERPI showed the lowest discordance, with residual misclassifications confined to hematologic-dominant phenotypes. Conclusion SLE classification frameworks show substantial overlap in real-world practice, with discordance driven by phenotype-specific prioritization of disease domains rather than random failure. SLERPI complements established classification criteria by supporting identification of early and atypical SLE presentations, while traditional criteria remain essential for research standardization. Integrating probabilistic diagnostic tools with classification frameworks may enhance SLE recognition across diverse clinical contexts.

ObjectiveTo evaluate the impact of the coronavirus disease 2019 (COVID-19) pandemic on rheumatology practice.MethodA cross-sectional web survey was designed by the members of the Arab League of Associations for Rheumatology (ArLAR), validated by its scientific committee and disseminated through e-mail and social media. It included close-ended questions about the impact of the pandemic on the rheumatology activities, including outpatient visits and hospitalizations (in percentage, 100% corresponds to complete suspension) and open-ended questions about unmet needs. Univariate and multivariable logistic regression analyses were used to evaluate the predictors of impact. Suggestions were developed to improve the practice.ResultsA total of 858 rheumatologists were included in the analysis (27.3% of registered in ArLAR), 37% were 35–44 years old, 60% were females, and 48% worked in the private sector. The impact of COVID-19 was a decrease of 69% in hospitalizations, 65% in outpatient clinic, 56% in infusion centers, and 43% in income. It was associated with the region (highest in the Gulf), use of telemedicine, impact on income and practice sector (lowest in private). There was a hydroxychloroquine shortage in 47%. Telemedicine was mostly based on traditional telephone contacts and e-mails and reimbursed in 12%. Fifteen rheumatologists (1.8%) were infected and 156 cases of COVID-19 were reported among patients. The top-cited unmet needs in rheumatology practice were access to drugs and a telemedicine platform.ConclusionsThe negative impact of the COVID-19 pandemic on rheumatology practice may compromise rheumatic diseases control. Better access to drugs and providing telemedicine platforms are recommended to improve the practice.Key Points• The COVID-19 pandemic had a significant negative impact on the rheumatology practice, including access to outpatient clinic, hospitalization, and to anchor drugs.• The compromised access to rheumatology care may jeopardize the control of chronic rheumatic diseases and the long-term prognosis.• Better access to drugs and providing telemedicine platforms are strongly recommended.

Patients with Sjögren's syndrome are at a higher risk to develop oral candidiasis than the general population. As antifungals have many side-effects, new approaches are needed to address this problem. This randomized controlled study aimed to evaluate the short-term efficacy of probiotics in the reduction of oral candidal growth in patients with SS. Thirty-two Sjogren’s syndrome patients were randomly allocated in two groups receiving either Probiotics or placebo capsules twice a day for 5 weeks. The strains included in the probiotic capsule were Lactobacillus acidophilus, Lactobacillus bulgaricus, Streptococcus thermophilus and Bifidobacteriumbifidum. Oral rinse solution samples were collected and candidal levels were determined (CFU/mL) at baseline and after the 5-week experimental period. Pain, erythema and angular cheilitis were also assessed at baseline and after 2, 4 and 5-week. In the probiotic group, there was a statistically significant reduction of the candidal load from baseline to the 5th week respectively. However, the change in candidal load at the same time in the placebo group was not statistically significant. The tested probiotic product may represent an unconventional method to reduce candidal colonization, to prevent oral candidosis in patients with Sjogren’s syndrome.Clinical trials registration ID NCT03840538 (https://clinicaltrials.gov/show/NCT03840538).

Background Gut microbial dysbiosis and leaky gut play a role in systemic lupus erythematosus (SLE). Geographical location and dietary habits affect the microbiome composition in diverse populations. This study explored the gut microbiome dysbiosis, leaky gut, and systemic immune response to gut bacterial consortium in patients with SLE exhibiting mild/moderate and severe disease activity. Methods Fecal and blood samples were collected from patients with SLE and healthy volunteers. Genomic DNA was extracted from the stool samples and subjected to 16S rRNA amplicon sequencing and microbiome profiling. Additionally, enzyme-linked immunosorbent assays were employed to determine the serum lipopolysaccharide level, as an assessment of gut permeability, and the systemic immune response against gut bacteria. Results Patients with SLE showed significantly lower gut bacterial richness and diversity, indicated by observed OTUs (56.6 vs. 74.44; p  = 0.0289), Shannon (3.05 vs. 3.45; p  = 0.017) and Simpson indices (0.91 vs. 0.94; p  = 0.033). A lower Firmicutes-to-Bacteroidetes ratio (1.07 vs. 1.69; p  = 0.01) was observed, with reduced genera such as Ruminococcus 2 (0.003 vs. 0.026; p  = 0.0009) and Agathobacter (0.003 vs. 0.012; p  < 0.0001) and elevated Escherichia-Shigella (0.04 vs. 0.006; p  < 0.0001) and Bacteroides (0.206 vs. 0.094; p  = 0.033). Disease severity was associated with a higher relative abundance of Prevotella (0.001 vs. 0.0001; p  = 0.04). Medication effects included lower Romboutsia (0.0009 vs. 0.011; p  = 0.005) with azathioprine and higher Prevotella (0.003 vs. 0.0002; p  = 0.038) with cyclophosphamide. Furthermore, categorization by prednisolone dosage revealed significantly higher relative abundances of Slackia (0.0007 vs. 0.00002; p  = 0.0088), Romboutsia (0.009 vs. 0.002; p  = 0.0366), and Comamonas (0.002 vs. 0.00007; p  = 0.0249) in patients receiving high-dose prednisolone (> 10 mg/day). No differences in serum lipopolysaccharide levels were found, but SLE patients exhibited elevated serum gut bacterial antibody levels, suggesting a systemic immune response. Conclusion This study confirms the gut microbiome dysbiosis in patients with SLE, influenced by disease severity and specific medication usage.

ObjectivesSystemic lupus erythematosus (SLE) is a systemic autoimmune disease. Cyclophosphamide (CYC) is a cytotoxic drug of a narrow therapeutic window that is commonly used in lupus nephritis (LN) treatment. However, 30–40% of patients experience CYC resistance. CYC inactivation is mediated by the glutathione S transferases (GSTs) superfamily: GST class A (GSTA) has the greatest activity and contains 5 isoenzymes. Polymorphisms of genes involved in the drug metabolism could alter the drug pharmacokinetics and effectiveness. CYC pharmacokinetics and pharmacogenomics are extensively studied in malignancies; however, scarce data are available about this issue in the autoimmune rheumatic diseases. Prediction of the drug response helps the achievement of the highest benefit-to-risk ratio. The aim of this case-control study was to address the association between GSTA1 polymorphism (-69C > T, rs3957356), and the rate of response to and side effects of intravenous CYC in LN patients.MethodsNinety-four patients were included and divided into matched groups: resistant and responsive. Genotyping was performed using restriction fragment length polymorphism method after amplification.ResultsA significant association between the TT genotype, and CYC resistance and partial response was observed. Concerning the recessive model, none of the patients within the TT group achieved complete remission. CYC side effects were more common with the polymorphism under the genotype, recessive model, and allele distributions. When patients’ pre- and post-treatment characteristics were compared, patients with the TT genotype did not show any significant improvement.ConclusionLN patients with GSTA1 (-69C > T, rs3957356) TT genotype have the highest risk of CYC unresponsiveness and toxicity.Key-Points• LN patients with the wild genotype of GSTA1 have the greatest probability of achieving a complete renal response to IV CYC.• The homozygous GSTA1 (-69C > T, rs3957356) TT genotype is associated with the highest risk of LN unresponsiveness to IV CYC.• The homozygous GSTA1 (-69C > T, rs3957356) TT genotype is associated with the highest risk of CYC-related side effects.

Background The aim of this study was to retrospectively investigate the prevalence and characteristics of neuropsychiatric (NP) involvement in a cohort of systemic lupus erythematosus (SLE) patients from a single tertiary center. Results Of 301 included patients’ medical records, the prevalence of NPSLE, that was defined according to the American College of Rheumatology Nomenclature of 1999, was 33.5% (101/301), of whom 10 (9.9%) were males. The mean age at the last visit of patients with NP involvement was 29.1 ± 8.2 years, whereas the mean age at onset was 21.9 ± 7.3 years, and the mean disease duration was 89.8 ± 59.4 months. The most common NP manifestations were psychosis [34/101 (33.7%)], followed by seizures [22/101 (21.8%)]. Compared to those without NPSLE, patients with NP involvement were characterized by having a younger age of onset ( p < 0.001) had a longer disease duration ( p = 0.02). Of the cumulative characteristics recorded, NPSLE patients showed a higher prevalence of cutaneous vasculitis ( p = 0.002), discoid rash ( p = 0.03), pleurisy and pleural effusion ( p = 0.004, p = 0.03, respectively), pericarditis ( p = 0.007), thrombocytopenia ( p = 0.04), and secondary antiphospholipid (APS) ( p = 0.04); however, there was no difference in any of the included serologic features between the two groups. Patients with NPSLE had a higher median disease activity score [Systemic Lupus Erythematosus Disease Activity Index-2 K (SLEDAI-2 K)] at the disease onset ( p = 0.008), yet it was comparable to those without NP involvement at the last visit ( p = 0.3). NPSLE patients demonstrated a higher median damage score ( p < 0.001) that was assessed according to the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score. NPSLE patients with secondary APS showed a higher prevalence of cerebrovascular accidents (CVA) ( p < 0.001), while those without APS developed psychosis more frequently ( p = 0.03). Conclusion Neuropsychiatric SLE patients (33.5%) demonstrated a younger age of onset, higher prevalence of secondary APS and distinct clinical characteristics, and had higher disease damage. APS-positive NPSLE patients had a higher prevalence of CVA, while APS-negative patients showed a higher prevalence of psychosis.

IgA vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is the most common cause of systemic vasculitis in childhood. Given its potential life-threatening systemic complications, early and accurate diagnosis as well as management of IgAV represent a major challenge for health care professionals. This study was carried out to attain an evidence-based expert consensus on a treat-to-target management approach for IgAV using Delphi technique. The preliminary scientific committee identified a total of 16 key clinical questions according to the patient, intervention, comparison, and outcomes (PICO) approach. An evidence-based, systematic, literature review was conducted to compile evidence for the IgAV management. The core leadership team identified researchers and clinicians with expertise in IgAV management in Egypt upon which experts were gathered from different governorates and health centers across Egypt. Delphi process was implemented (two rounds) to reach a consensus. An online questionnaire was sent to expert panel (n = 26) who participated in the two rounds. After completing round 2, a total of 20 recommendation items, categorized into two sections were obtained. Agreement with the recommendations (rank 7–9) ranged from 91.7–100%. Consensus was reached (i.e. ⩾75% of respondents strongly agreed or agreed) on the wording of all the 20 clinical standards identified by the scientific committee. Algorithms for the diagnosis and management have been suggested. This was an expert, consensus recommendations for the diagnosis and treatment of IgAV and IgA vasculitic nephritis, based on best available evidence and expert opinion. The guideline presented a strategy of care with a pathway to achieve a state of remission as early as possible. Plain Language Summary Given its potential life-threatening systemic complications, early and accurate diagnosis of immunoglobulin A vasculitis represents a major challenge for health care professionals. This work provided cornerstone principles for the management of the condition. Adopting PICO approach and implementing Delphi process a consensus was reached on evidence-based treat-to-target treatment recommendations. This will endorse enhancement and consistency of care of this cohort of patients in standard practice.

Background The spectrum of Juvenile Idiopathic Arthritis (JIA) in Africa is still largely unknown. We thus set out to illustrate how we set up the PAFLAR JIA registry and describe the clinical profile of Juvenile Idiopathic Arthritis across various regions in Africa. Methods We carried out a retrospective observational cohort study where collaborators were trained on use of the existing PAFLAR REDCAP database to enter data for the JIA patients currently under their care capturing their epidemiological data, clinical features, laboratory investigations, diagnosis and therapy at initial diagnosis. Descriptive statistics including means, standard deviations, medians, interquartile ranges (IQR) for continuous variables and proportions for categorical variables were calculated as appropriate. Tests for difference between groups were performed between categorical variables using Pearson’s chi-square or Fisher’s exact tests. All analyses were performed using SPSS version 22 software. Results We enrolled 302 patients, 58.6% (177 of 302) of whom were female. The median age of disease onset was 7 years (range 3–11 years) and the median age at diagnosis was 8.5 years (range 5–12 years). The median duration delay in diagnosis was 6 months (range 1-20.8 months). The JIA categories included Systemic JIA 18.9% (57), Oligoarticular JIA 19.2% (83), Polyarticular RF + ve 5% (15), Polyarticular RF-ve 17.9% (54), Enthesitis Related Arthritis (ERA) 18.2% (55), Psoriatic Arthritis 7% (21) and undifferentiated JIA 5.6% (17). As regards treatment the commonest therapies were NSAID therapy at 31.1%, synthetic DMARDs at 18.1%, synthetic DMARDs combined with NSAIDs at 17.5% and steroid therapy at 9.6%. Biological DMARDs accounted for 2.3% of therapies offered to our patients at diagnosis. The average JADAS score was 10.3 (range 4.8–18.2) and the average CHAQ score was 1.3 (range 0.7-2.0). Conclusion Our study highlights strategies involved in setting up a Pan-African paediatric rheumatology registry that embraces our broad diversity and the vast spectrum of JIA in Africa while comparing the various therapies available to our patients. The PAFLAR JIA registry strives to ensure a comprehensive representation of the diverse healthcare landscapes within the continent. Further longitudinal observation studies are required to ascertain the long-term outcomes of our patients and ultimately help inform policy to create a more favorable health ecosystem to support the healthcare needs of JIA patients in Africa.

Background The coronavirus disease 2019 (COVID-19) pandemic has become a global health, social, and economic crisis. Healthcare professionals, patients, healthy individuals, and the whole community are under inevitable psychological pressure which may cause different psychological problems as fear, anxiety, depression, and insomnia. The aim was to assess the impact of the COVID19 pandemic on the attitude, behavior, and mental health of rheumatic patients and to compare them with healthy individuals. This is a case-control study, 360 participants were included and divided into a patient group composed of 180 patients with rheumatic diseases, and a control group composed of 180 healthy people. Data were collected via a self-administered structured questionnaire designed on Google forms. It was sent to participants via social networks and emails to different rheumatic patients and healthy individuals. Mental health was measured by the 5-item Brief Symptom Rating Scale (BSRS-5). Results The mean age of cases and control were (35.05 ± 8.79 vs 34.56 ± 9.06) years. In comparing attitudes and behavior toward COVID 19, there was a statistically significant difference ( p ≤ 0.05) between both groups regarding washing hands, going outdoors, wearing masks and gloves outdoors, and staying in their rooms. Patients depended mainly on telehealth more than usual where about 50% used either phone calls, internet or sent their relatives to their physicians; moreover, 20% did not contact their physicians at all the past few months. There was a statistically significant difference ( p ≤ 0.05) between both groups regarding feeling angry/irritated, inferior and insomniac. The BSRS-5 total score and being defined as a psychiatric case (according to the BSRS-5 scale) also differed significantly between patients and controls. Systemic lupus erythematosus (SLE) patients showed more adherence to their medications and stayed mostly at home and they have higher BSRS scores. Conclusion Patients with rheumatic diseases show comparable degrees of anxiety and depression to healthy individuals, but higher distress symptoms and panic in the form of anger, irritability, and insomnia. They have a significantly higher sense of inferiority and a higher total BSRS compared to controls. SLE patients show more adherence to their medications and stay mostly at home as a reflection of feeling more vulnerable. Moreover, they have higher degrees of psychological affection in the form of higher BSRS scores. Abandoning drug purchasing without medical prescription is necessary in Egypt to protect our patients from unnecessary drug shortages adding to their fear and anxiety. Mental health should be addressed in the same manner we deal with the infectious disease itself, being of no less importance. Mental health professionals, social workers, and support groups need to provide psychological support to vulnerable populations, including patients with rheumatic diseases. Rheumatologists should be aware of the need for psychiatric consultation for their patients whenever necessary.