Assessment of serum and cutaneous JAK3 in juvenile scleroderma | ||||
| The Egyptian Journal of Pediatric Allergy and Immunology | ||||
| Volume 22, Issue 1, April 2024, Page 10-18 PDF (302.61 K) | ||||
| Document Type: Original Article | ||||
| DOI: 10.21608/ejpa.2024.279700.1065 | ||||
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| Authors | ||||
Dalia El-Ghoneimy   1; Fatema ElSaeed2; Marwa ElNajjar3; Naglaa Ahmad4; Eman Fahmy5; Naglaa Osman 6; Ghada Shousha 7
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| 1Pediatric Allergy, Immunology and Rheumatology Unit, Children's Hospital, Ain Shams University | ||||
| 2Department of Pediatrics, Specialized ElSalam Hospital-ElShorouk | ||||
| 3Department of clinical pathology, Ain Shams University | ||||
| 4Department of Pathology, Ain Shams University | ||||
| 5Pediatric Allergy, Immunology and Rheumatology Unit, Sohag University | ||||
| 6Pediatric Allergy, Immunology and Rheumatology Unit, Assuit University | ||||
| 7Pediatric Allergy, Immunology and Rheumatology Unit, Ain Shams University | ||||
| Abstract | ||||
| Background: Janus kinases (JAKs) family include JAK 1, 2, 3 and tyrosine kinase 2 play central role in cytokine and growth factor signalling and few studies suggest their possible contribution in the pathogenesis of scleroderma. Objective: To evaluate serum and cutaneous expression of JAK3 in pediatric patients with juvenile scleroderma (JSD) and their association with the disease severity. Patients and methods: This was a pilot study that included 16 pediatric patients with JSD; they were 11 patients with juvenile systemic sclerosis (JSSc) and 5 patients with juvenile localized scleroderma (JLS). The patients were compared to 17 healthy controls. Disease severity was assessed using modified Rodnan skin score (mRSSc) and JSSc severity score (J4S) in JSSc patients while localized scleroderma damage index (LoSDI) was used in JLS patients. Serum and cutaneous expression of JAK3 were measured by enzyme linked immunosorbent assay. Results: The median (IQR) of serum JAK3 was significantly higher among JSSc patients as compared to that of controls [430 (320-520) versus 270 (180-385), p = 0.005], while comparable values were found among JLS and controls as well as JLS and JSSc patients (p>0.05). Cutaneous expression of JAK3 was comparable between all patients and controls (p>0.05). Serum and cutaneous expression of JAK 3 did not correlate significantly with mRSSc and J4S in JSSc patients and LoSDI in JLS patients (p>0.05). Conclusion: JAK3 seems to contribute in the pathogenesis of JSSc and further studies are needed to establish its role and the usefulness of using JAK 3 inhibitors in these patients. | ||||
| Keywords | ||||
| JAK3; modified Rodnan skin score; Juvenile systemic sclerosis | ||||
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