De Luca Giacomo, Campochiaro Corrado, Burastero Samuele E, Matucci-Cerinic Marco, Doglioni Claudio, Dagna Lorenzo
Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy.
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.
Front Med (Lausanne). 2024 Jan 24;10:1214523. doi: 10.3389/fmed.2023.1214523. eCollection 2023.
This study aimed to evaluate periostin serum levels and skin expression in patients with systemic sclerosis (SSc).
We enrolled 35 patients with diffuse (d-SSc) or limited (l-SSc) SSc, 15 patients with very early diagnosis of systemic sclerosis (VEDOSS), and 30 sex-matched healthy controls. Periostin serum levels were determined by an enzyme-linked immunosorbent assay (ELISA). Periostin skin expression was determined by immunohistochemistry (IHC) on paired involved and uninvolved 5-mm skin biopsy samples in a subgroup of 10 d-SSc and 10 L-SSc patients. A 12-month follow-up was considered.
We included 50 patients (mean age 53.1 ± 16.1 years; women 94%; mean disease duration 38.2 ± 45.1 months; anti-centromere 50%; anti-Scl70 40%), 35 of them with a definite SSc (68.8% l-SSc; 31.4% d-SSc; mean mRSS 9.0 ± 7.2) and 15 with VEDOSS; 30 controls were also included in this study. Periostin serum levels were higher in SSc patients compared to controls (32.7 ± 8.0 ng/mL vs. 27.7 ± 7.3 ng/mL; < 0.001), while these levels were comparable among different groups of patients (29.7 ± 6.9 ng/mL in VEDOSS, 33.4 ± 7.8 ng/mL in lc-SSc; and 34.0 ± 8.5 in dc-SSc; = ns). SSc patients with digital ulcers had higher periostin serum levels (36.2 ± 7.9 ng/mL vs. 30.6 ± 7.3 ng/mL, < 0.02). Samples from the involved skin of l-SSc and d-SSc patients showed a significant dermal expression of periostin; an identical periostin expression was evident in the uninvolved skin of patients with d-SSc. In 7 out of 10 L-SSc patients, periostin expression was absent on uninvolved skin. In the remaining three l-SSc patients, a mild periostin expression on IHC was detectable on uninvolved skin and all of these three l-SSc patients presented a dramatic skin progression.
Periostin skin expression may be a useful biomarker to indicate the presence of a disease at a higher risk of rapid cutaneous involvement.
本研究旨在评估系统性硬化症（SSc）患者血清骨膜蛋白水平及皮肤表达情况。
我们纳入了35例弥漫性（d-SSc）或局限性（l-SSc）SSc患者、15例极早期诊断的系统性硬化症（VEDOSS）患者以及30例性别匹配的健康对照者。采用酶联免疫吸附测定（ELISA）法测定血清骨膜蛋白水平。在10例d-SSc和10例l-SSc患者的亚组中，通过免疫组织化学（IHC）法对配对的5mm受累及未受累皮肤活检样本进行骨膜蛋白皮肤表达检测。进行为期12个月的随访。
我们纳入了50例患者（平均年龄53.1±16.1岁；女性占94%；平均病程38.2±45.1个月；抗着丝点抗体阳性率50%；抗Scl70抗体阳性率40%），其中35例为确诊的SSc（68.8%为l-SSc；31.4%为d-SSc；平均改良Rodnan皮肤评分9.0±7.2），15例为VEDOSS；本研究还纳入了30例对照者。与对照组相比，SSc患者血清骨膜蛋白水平更高（32.7±8.0ng/mL vs. 27.7±7.3ng/mL；P<0.001），而不同患者组之间这些水平相当（VEDOSS组为29.7±6.9ng/mL，lc-SSc组为33.4±7.8ng/mL，dc-SSc组为34.0±8.5ng/mL；P=无统计学意义）。有指端溃疡的SSc患者血清骨膜蛋白水平更高（36.2±7.9ng/mL vs. 30.6±7.3ng/mL，P<0.02）。l-SSc和d-SSc患者受累皮肤样本显示骨膜蛋白有显著的真皮表达；d-SSc患者未受累皮肤也有相同的骨膜蛋白表达。10例l-SSc患者中有7例未受累皮肤无骨膜蛋白表达。其余3例l-SSc患者未受累皮肤在免疫组织化学上可检测到轻度骨膜蛋白表达，且这3例l-SSc患者均出现了明显的皮肤进展。
骨膜蛋白皮肤表达可能是一种有用的生物标志物，可用于指示疾病的存在，提示有快速皮肤受累的较高风险。
