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Phoenixin-14 as a Potential Limiting Neuropeptide for Exaggerated Inflammation in Familial Mediterranean Fever and
Semra Ayduran1, Gülşah Kılbaş2, Saadet Nilay Tığrak3
1S. Ayduran, MD, Department of Pediatric Rheumatology, Faculty of Medicine, Pamukkale University, Denizli.
Objective:
This study aimed to evaluate serum phoenixin-14 (PNX-14) levels in patients with familial Mediterranean fever (FMF) and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) during both attack and attack-free periods to investigate its potential biomarker value.
Methods:
In total, 140 children were included in this cross-sectional study: 46 with FMF, 48 with PFAPA, and 46 healthy children. Blood samples were collected during both febrile and attack-free periods in the patients with FMF and PFAPA, whereas samples from healthy controls were collected at a single timepoint. 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 (IBM) was used for statistical analyses.
Results:
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). Although a positive correlation was observed between PNX-14 levels and CRP (ρ 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 disease severity score), colchicine treatment duration, or daily colchicine dose in the FMF group.
Conclusion:
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.
Insights
Serum Phoenixin-14 (PNX-14) levels are elevated in children with Familial Mediterranean Fever (FMF) and Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA), particularly during active disease flares. This suggests PNX-14 may serve as a biomarker for inflammation in these autoinflammatory conditions.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Biomarker Discovery
Background:
- Familial Mediterranean Fever (FMF) and Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) are autoinflammatory disorders characterized by recurrent inflammatory episodes.
- Identifying reliable biomarkers is crucial for diagnosis, monitoring disease activity, and understanding the underlying pathophysiology of these conditions.
Purpose of the Study:
- To evaluate serum Phoenixin-14 (PNX-14) levels in pediatric patients with FMF and PFAPA.
- To compare PNX-14 levels during active disease flares versus attack-free periods and healthy controls.
- To investigate the potential of PNX-14 as a biomarker for autoinflammatory diseases.
Main Methods:
- A cross-sectional study involving 140 children: 46 FMF, 48 PFAPA, and 46 healthy controls.
- Serum samples collected during both febrile/attack and attack-free periods for FMF/PFAPA patients; single time point for controls.
- Assayed serum PNX-14, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum amyloid A (SAA), and fibrinogen using ELISA and standard laboratory methods.
Main Results:
- Serum PNX-14 levels were significantly higher in FMF and PFAPA patients during attack periods compared to attack-free periods and healthy controls (p < 0.001).
- Elevated PNX-14 levels were also observed in attack-free periods for both patient groups versus healthy controls (p < 0.001).
- A positive correlation was found between PNX-14 and CRP (rho = 0.222, p = 0.04), but not with ESR, SAA, or fibrinogen. No association with disease severity or colchicine treatment was noted in FMF patients.
Conclusions:
- Phoenixin-14 may play a role in the neuroimmune response to resolve inflammation in autoinflammatory conditions.
- PNX-14 shows potential as an immunomodulatory biomarker, reflecting inflammatory burden or healing processes.
- Further research is warranted to elucidate the precise role of PNX-14 in FMF, PFAPA, and other autoinflammatory diseases.
