The clinical significance of antinuclear antibodies and specific autoantibodies in juvenile and adult systemic lupus erythematosus patients.

BACKGROUND: Juvenile systemic lupus erythematosus (JSLE) and adult SLE (ASLE) patients present with different clinical manifestations, but it is unknown if there are differences in their antinuclear autoantibody (ANA) profiles or if staining patterns are associated with specific autoantibodies and clinical manifestations. OBJECTIVE: To determine whether distinct types and numbers of ANA-staining patterns are associated with specific autoantibodies and clinical manifestations in JSLE and ASLE patients. METHODS: A retrospective study was performed in Thai children (n = 146) and adults (n = 180) diagnosed with SLE using the Systemic Lupus International Collaborating Clinics classification criteria. RESULTS: JSLE patients with a homogeneous pattern of staining and anti-dsDNA or anti-nucleosome antibodies in serum, developed renal involvement, leukopenia and acute/subacute cutaneous LE. Coarse speckled pattern with anti-RNP or anti-Sm showed thrombocytopenia and renal involvement in JSLE patients, but leukopenia in both groups. JSLE patients with fine-coarse speckled pattern and anti-RNP, anti-Sm, anti-Ro-52 or anti-SSA developed leukopenia, thrombocytopenia and renal involvement, whilst hemolytic anemia and serositis were commonly found in those with anti-Ro-52. Median SLEDAI score was higher in JSLE than ASLE patients. CONCLUSIONS: Detailed ANA-staining patterns with specific autoantibodies show particular clinical manifestations and hence prompt further clinical investigations in both JSLE and ASLE patients. Therefore, this study demonstrates that distinct patterns of ANA staining and specific autoantibodies are clinically important in both children and adults with SLE.

Revisiting Fruit Allergy: Prevalence across the Globe, Diagnosis, and Current Management.

Fruit allergies manifest with a diverse array of clinical presentations, ranging from localized contact allergies and oral allergy syndrome to the potential for severe systemic reactions including anaphylaxis. The scope of population-level prevalence studies remains limited, largely derived from single-center or hospital-based investigations. In this comprehensive review, we conducted a systematic literature search spanning the years 2009 to 2023, with full acknowledgment of potential analytical biases, to provide a global overview of fruit allergy prevalence. The primary mechanistic underpinning of fruit allergies stems from cross-reactivity between aeroallergens and food allergens, a consequence of structurally similar epitopes-a phenomenon recognized as pollen food allergy syndrome (PFAS). In the era of molecular allergology, numerous studies have dissected allergen components with substantial clinical relevance. Within this review, we explore important allergenic molecules found in plant-based foods, scrutinize pertinent cross-reactivity patterns, and offer insights into management recommendations. Additionally, we compare guideline recommendations to enhance clinical understanding and inform decision making.

Meta-analysis of genome-wide association study identifies FBN2 as a novel locus associated with systemic lupus erythematosus in Thai population.

BACKGROUND: Differences in the expression of variants across ethnic groups in the systemic lupus erythematosus (SLE) patients have been well documented. However, the genetic architecture in the Thai population has not been thoroughly examined. In this study, we carried out genome-wide association study (GWAS) in the Thai population. METHODS: Two GWAS cohorts were independently collected and genotyped: discovery dataset (487 SLE cases and 1606 healthy controls) and replication dataset (405 SLE cases and 1590 unrelated disease controls). Data were imputed to the density of the 1000 Genomes Project Phase 3. Association studies were performed based on different genetic models, and pathway enrichment analysis was further examined. In addition, the performance of disease risk estimation for individuals in Thai GWAS was assessed based on the polygenic risk score (PRS) model trained by other Asian populations. RESULTS: Previous findings on SLE susceptible alleles were well replicated in the two GWAS. The SNPs on HLA class II (rs9270970, A>G, OR = 1.82, p value = 3.61E-26), STAT4 (rs7582694, C>G, OR = 1.57, p value = 8.21E-16), GTF2I (rs73366469, A>G, OR = 1.73, p value = 2.42E-11), and FAM167A-BLK allele (rs13277113, A>G, OR = 0.68, p value = 1.58E-09) were significantly associated with SLE in Thai population. Meta-analysis of the two GWAS identified a novel locus at the FBN2 that was specifically associated with SLE in the Thai population (rs74989671, A>G, OR = 1.54, p value = 1.61E-08). Functional analysis showed that rs74989671 resided in a peak of H3K36me3 derived from CD14+ monocytes and H3K4me1 from T lymphocytes. In addition, we showed that the PRS model trained from the Chinese population could be applied in individuals of Thai ancestry, with the area under the receiver-operator curve (AUC) achieving 0.76 for this predictor. CONCLUSIONS: We demonstrated the genetic architecture of SLE in the Thai population and identified a novel locus associated with SLE. Also, our study suggested a potential use of the PRS model from the Chinese population to estimate the disease risk for individuals of Thai ancestry.

Anti-neutrophil cytoplasmic antibodies and their clinical significance.

Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay. A positive staining of ANCA can be classified into three main categories based on the staining patterns: cytoplasmic, perinuclear, and atypical. Patients with granulomatosis with polyangiitis (GPA) mostly have a positive cytoplasmic staining pattern (c-ANCA) whilst a perinuclear pattern (p-ANCA) is more common in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) patients. Atypical pattern (a-ANCA) is rarely seen in patients with systemic small-vessel vasculitis but it can be found in other conditions. Here, techniques for ANCA detection, ANCA staining patterns and their clinical significances are reviewed.

Oral Ulcers in Juvenile-Onset Systemic Lupus Erythematosus: A Review of the Literature.

Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE) specific, where the lesional biopsy shows a unique pattern of mucosal change in LE, and LE nonspecific, where the ulcers and their histopathological findings can be found in other oral diseases. Here, the clinical manifestations, diagnosis and management of oral ulcers in JSLE patients are reviewed.