Background/Purpose: Juvenile scleroderma (JS) is a rarely seen chronic connective tissue disorder. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Localized form is more frequent in childhood, characterized with benign clinical course and favorable prognosis. Although extremely rare, juvenile systemic scleroderma (JSS) has far worse prognosis with multi-organ involvement and possible life-threatening complications. Its main pathophysiological characteristics include microvascular abnormalities and excessive fibrosis of the skin, subcutaneous tissues and internal organs. Pentraxin 3 (PTX 3) is a multifunctional protein produced at the inflammation site by macrophages, dendritic cells, endothelial cells, smooth muscle cells and fibroblasts. Previously studies reported an increased level of PTX 3 among adult patients with scleroderma. Study among patients with JS has not been provided yet. We aimed to measure the level of PTX 3 in patients with juvenile scleroderma comparing to healthy children. Thereby, we have tried to give an answer on question whether the PTX 3 could be a marker of fibrosis in patients with juvenile scleroderma.

Methods:  We assessed patients with JSS and those with juvenile localized scleroderma (JLS) and age- and sex- matched health controls. A complete medical history, physical examination, and laboratory evaluation were performed for each patient at the time of enrollment. The same physical examination and laboratory investigation was performed in healthy controls, in order to exclude the coincidental disease. Circulating PTX3 levels were measured by enzyme immunoassay. The lower limits of detection for PTX3 was 0.1 ng/ml.

Results: We assessed 24 patients with JSS, 20 patients with JLS and 41 health controls. The mean age of patients was 15.38 ±3.156, 12.44 ±3.63 and 14.33±3.48 for JSS, JLS and healthy controls, respectively. Mean disease duration was 2 years (range: 0.6 -15 years) for JSS and 1.5 years (range: 0.6 – 18 years) for JLS patients. Mean serum level of PTX 3 was 10.63 ± 8.61 ng/ml, 11.75 ± 9.11 ng/ml and 2.76 ± 1.338 ng/ml for JSS, JLS and healthy controls, respectively. In both of patients group PTX 3 level was significantly higher comparing to healthy children (p<0.001). We didn`t find statistically significant difference between JSS and JLS patients according to level of PTX3. A mean modified Rodnan skin score of JSS patients was 19.95 ± 11.088. PTX3 level was found to be in positive correlation with modified Rodnan skin score, in patients with JSS (Rho=0.497, p=0.030).

Conclusion: The circulating PTX3 level is significantly higher in both JSS and JLS patients than in healthy control subjects. The possible explanation is that fibrosis and increased fibroblast activation represent main pathophysiological mechanism in both form of the disease. This result support relevance of PTX 3 measurement in order to determinate fibrosis activity. PTX 3 should be considered a relevant marker of fibrosis in patients with juvenile scleroderma.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/role-of-pentraxin-3-in-patients-with-juvenile-scleroderma/