Spectrum of systemic lupus erythematosus in Oman: from childhood to adulthood.

SLE is a disease that mainly affects women of childbearing age, however, a total of 15-20% of cases present in children. Although adult onset SLE (aSLE) and childhood onset SLE (cSLE) share the same diagnostic criteria, differences have been identified. The aim of this study is to compare the similarities and differences in between cSLE and aSLE in an Arab population from Oman. We evaluated 225 SLE patients, 139 adults and 86 children, who fulfilled the criteria for diagnosis. At disease onset, 99% of SLE cohort fulfilled the SLICC criteria; however the ACR 1997 criteria were fulfilled in 66% aSLE and 80% cSLE. The clinical features of SLE in cSLE showed higher frequency of renal (50 vs 19%; p < 0.001), musculoskeletal (67 vs 53%; p = 0.036) and pulmonary involvement (13 vs 2.9%, p = 0.005); while aSLE showed higher frequency of hematological (64 vs 49%; p = 0.25) and mucocutaneous (24 vs 10%; p = 0.13) involvement. The mean disease activity score at disease onset and during disease course was also higher in cSLE (13 vs 8.5; p < 0.0005) (16 vs 11.8; p < 0.0005), respectively. Differences in autoantibody profile were also noted in cSLE with higher positivity of anti-dsDNA and antiphospholipid antibody (94 vs 84%; p = 0.027) (53 vs 37%; p = 0.25), respectively. cSLE patients were more likely than aSLE to be treated with immunosuppressant such as cyclophosphamide (51 vs 22%; p < 0.001) and MMF (70 vs 54%; p = 0.019). Similarities and differences between aSLE and cSLE in a cohort from Oman of Arab ethnicity were identified. It appears that individual races and ethnicities may exhibit differences in disease susceptibility and manifestations.

Single-cell analysis with childhood and adult systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE), a heterogeneous and chronic autoimmune disease, exhibit unique changes in the complex composition and transcriptional signatures of peripheral blood mononuclear cells (PBMCs). While the mechanism of pathogenesis for both childhood-onset SLE (cSLE) and adult-onset SLE (aSLE) remains unclear, cSLE patients are considered more unpredictable and dangerous than aSLE patients. In this study, we analysed single-cell RNA sequencing data (scRNA-seq) to profile the PBMC clusters of cSLE/aSLE patients and matched healthy donors and compared the PBMC composition and transcriptional variations between the two groups. Our analysis revealed that the PBMC composition and transcriptional variations in cSLE patients were similar to those in aSLE patients. Comparative single-cell transcriptome analysis between healthy donors and SLE patients revealed IFITM3, ISG15, IFI16 and LY6E as potential therapeutic targets for both aSLE and cSLE patients. Additionally, we observed that the percentage of pre-B cells (CD34-) was increased in cSLE patients, while the percentage of neutrophil cells was upregulated in aSLE patients. Notably, we found decreased expression of TPM2 in cSLE patients, and similarly, TMEM150B, IQSEC2, CHN2, LRP8 and USP46 were significantly downregulated in neutrophil cells from aSLE patients. Overall, our study highlights the differences in complex PBMC composition and transcriptional profiles between cSLE and aSLE patients, providing potential biomarkers that could aid in diagnosing SLE.

Oral lesions in adult- and juvenile-onset systemic lupus erythematosus patients: A case series report.

Systemic lupus erythematosus (SLE) is an autoimmune disease with various oral manifestations, including ulceration, white keratotic plaques, oral discoid lupus erythematosus, oral lichen planus (OLP)-like lesions, non-specific erythema, purpura, petechiae, and cheilitis, which resemble lesions of other systemic diseases. Recognizing the oral manifestation of SLE is essential for comprehensive patient management. This study reports 4 cases of SLE with various oral lesions, underlying conditions and diagnostic methods.In September 2019, 2 adult SLE patients and 2 juvenile SLE patients were consulted at the Oral Medicine Clinic. The assessment of systemic diseases was conducted by the Internal Medicine and Pediatrics resident, whereas the Oral Medicine resident performed the intraoral examinations. The medical history, clinical findings and laboratory results were analyzed to establish the diagnosis.The first patient was a 38-year-old female presenting with multiple white keratotic plaques throughout the mucosa, an OLP-like lesion on the right buccal mucosa, petechiae on the hard palate, and petechiae and purpura on the upper and lower extremities. The second case was a 24-year-old female with a malar rash and multiple ulcerations on the vermilion zone, an OLP-like lesion on the left buccal mucosa, and a palatal ulcer. The third and fourth cases were 16-year-old females with a prominent butterfly rash. The patients presented with acute pseudomembranous candidiasis, an aphthous-like ulcer and keratotic plaques. They received antimicrobial therapy for the intraoral lesions and showed promising results.The oral lesions in adultand juvenile-onset SLE patients varied depending on the disease severity and treatment received.

Belimumab for the treatment of pediatric patients with lupus nephritis.

INTRODUCTION: The FDA approved the anti-BAFF monoclonal antibody, belimumab, in 2011 for adult systemic lupus erythematosus (SLE), in 2019 for pediatric SLE, in 2020 for adult lupus nephritis (LN), and in 2022 for pediatric LN. AREAS COVERED: We performed a PUBMED database search through November 2022, using 'belimumab and lupus nephritis,' 'belimumab and childhood systemic lupus erythematosus,' 'belimumab and pediatric systemic lupus erythematosus,' and 'belimumab and juvenile systemic lupus erythematosus' as the search phrases. We also vetted pertinent references cited in the papers gleaned from the above search, and we drew from our personal literature collections. EXPERT OPINION: Based on clinical-trials and real-world experience, belimumab is useful and safe in adult SLE and LN. In contrast and despite FDA approval, evidence of effectiveness in pediatric SLE and pediatric LN is very limited. Whereas there was a trend favoring belimumab in the only randomized, controlled trial to date in pediatric SLE, the difference between the belimumab and placebo groups failed to achieve statistical significance. Moreover, there have been no randomized, controlled trials for belimumab in pediatric LN. Based largely on information gleaned from experience in adults, the clinician can cautiously prescribe belimumab to his/her pediatric LN patient and hope for benefit.