Ayduran S, Kılbaş G, Tığrak SN, Kaleli İ, Demir M , et al.
The Journal of rheumatology •
This study aimed to evaluate serum Phoenixin-14 (PNX-14) levels in Familial Mediterranean Fever (FMF) and Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) patients during both attack and attack-free periods to investigate its potential biomarker value. A total of 140 children were included in this cross-sectional study: 46 FMF, 48 PFAPA, and 46 healthy children. Blood samples were collected during both febrile and attack-free periods in the FMF and PFAPA patients, whereas samples from healthy controls were collected at a single time point. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum amyloid A (SAA), fibrinogen, and serum PNX-14 levels were evaluated. Serum PNX-14 levels were measured using ELISA. SPSS 25 was used for statistical analyses. PNX-14 levels were significantly higher during attack periods in FMF and PFAPA patients compared to attack-free periods and healthy controls (p < 0.001). PNX-14 levels during attack-free periods were also higher in both patient groups than in healthy controls (p < 0.001). While a positive correlation was observed between PNX-14 levels and CRP (rho = 0.222, p = 0.04), no significant correlation was observed with ESR, SAA, or fibrinogen. No significant associations were observed between PNX-14 levels and disease severity (PRAS), colchicine treatment duration, or daily colchicine dose in the FMF group. PNX-14 may be part of a neuroimmune response aimed at resolving inflammation in autoinflammatory processes and could be a potential immunomodulatory biomarker. PNX-14 may be a complementary indicator reflecting inflammatory burden or the healing process.
Türkuçar S, Bülbül G, Ünsal E, Özer E, Erdağ TK , et al.
Clinical rheumatology •
Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever syndrome during early childhood period with regular febrile attacks of sterile upper airway inflammation. The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily. The aim of this study is to explore the immunological basis of PFAPA by evaluating the cellular properties of tonsils, and microbial exposition such as Helicobacter pylori on tonsillectomy materials. The paraffinized tonsil samples of 26 PFAPA and 29 control patients with obstructive upper airway disorder were compared in terms of immunohistochemical staining features including CD4, CD8, CD123, CD1a, CD20, and H. pylori. The median number of CD8+ cells was 1485 (1218-1287) in PFAPA while it was 1003 (852-1261.5) in control group and the difference was statistically significant (p=0.001). Similarly, CD4+ cell counts were statistically higher in PFAPA group than control (833.5 vs 622). The ratio of CD4/CD8 did not differ between two groups; also, there was no statistically difference in terms of the other immunohistochemical staining results, such as CD20, CD1a, CD123 and H. pylori. This is the largest number of pediatric tonsillar tissue study of PFAPA patients in current literature and we emphasized the triggering effects of CD8+ and CD4+ T-cells on PFAPA tonsils. • The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily. • In current study, 92.3% of our patients did not experience any attacks following operation similarly with literature. • We observed the increased number of CD4+ and CD8+ T cell counts on PFAPA tonsils compared to control group and emphasized the active role of both CD4+ and CD8+ cells localized on PFAPA tonsils in immune dysregulation. • Some other cell types evaluated in this study such as CD19+ (B cells), CD1a (dendritic cells), and CD123 (IL-3 receptors, for pluripotent stem cells) and H. pylori did not differ in PFAPA patients compared to the control group.