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SAPHO 综合征患者的临床特征和预后——一项真实世界的队列研究。

The clinical characteristics and prognosis of patients with SAPHO syndrome--a real-world cohort study.

机构信息

Department of Orthopedic Medicine, Beijing Jishuitan Hospital, Capital Medical University, 31 Xinjiekou East St, Xicheng District, Beijing, 100035, China.

Department of Hematology, Lymphoma Center, Peking University Third Hospital, Beijing, China.

出版信息

Clin Rheumatol. 2024 Jan;43(1):561-568. doi: 10.1007/s10067-023-06782-7. Epub 2023 Sep 27.

DOI:10.1007/s10067-023-06782-7
PMID:37755548
Abstract

OBJECTIVES

We aimed to analyze the clinical characteristics and outcomes of patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.

METHODS

The clinical records of 64 patients with SAPHO syndrome were collected, and the treatment and outcomes of 27 patients were followed up. The patients were divided into three groups according to the site of bone lesions: only anterior chest wall (ACW) involvement, only spinal involvement, and bone lesion involvement at both sites. The clinical characteristics and outcomes were compared. The clinical characteristics of the patients with and without peripheral joint involvement were compared.

RESULTS

Among all patients, 31.25% (20/64) had only ACW involvement, 15.63% (10/64) had only spinal involvement, and 53.12% (34/64) had both ACW and spinal involvement. Peripheral joint involvement was observed in 25.00% (16/64) of the patients. Patients with only spinal involvement were older than those with only ACW involvement (p = 0.006). Patients with both ACW and spinal involvement were older than those with only ACW involvement (p = 0.002) and had a longer diagnosis delay (p = 0.015). Patients with peripheral joint involvement were younger than those without peripheral joint involvement (p = 0.028). During follow-up, 88.89% (24/27) of patients had good outcomes. Twenty-two patients were treated with non-steroidal anti-inflammatory drugs + Iguratimod (IGU), and the outcomes of 90.91% (20/22) improved.

CONCLUSIONS

A relationship may exist between the sites of bone lesions and clinical characteristics of patients with SAPHO syndrome. The clinical outcomes of these patients may be good, and IGU may be effective in treating SAPHO syndrome. Key Points • This study is the first long-term follow-up on the effectiveness of iguratimod in treating patients with SAPHO. • This study revealed that patients with SAPHO and different bone lesion sites may present with different clinical characteristics.

摘要

目的

分析滑膜炎、痤疮、脓疱病、骨质增生和骨炎(SAPHO)综合征患者的临床特征和结局。

方法

收集了 64 例 SAPHO 综合征患者的临床资料,对其中 27 例患者进行了治疗和随访。根据骨病变部位将患者分为三组:仅前胸壁(ACW)受累、仅脊柱受累和 ACW 和脊柱均受累。比较三组的临床特征和结局。比较有和无外周关节受累患者的临床特征。

结果

所有患者中,31.25%(20/64)仅有 ACW 受累,15.63%(10/64)仅有脊柱受累,53.12%(34/64)ACW 和脊柱均受累。25.00%(16/64)的患者有外周关节受累。仅脊柱受累患者的年龄大于仅 ACW 受累患者(p=0.006)。ACW 和脊柱均受累患者的年龄大于仅 ACW 受累患者(p=0.002),且诊断延迟时间更长(p=0.015)。有外周关节受累的患者比无外周关节受累的患者年龄小(p=0.028)。随访期间,88.89%(24/27)的患者有良好的结局。22 例患者接受非甾体抗炎药+依那西普(IGU)治疗,90.91%(20/22)的患者病情改善。

结论

SAPHO 综合征患者骨病变部位与临床特征可能存在一定关系。这些患者的临床结局可能较好,IGU 可能对 SAPHO 综合征有效。

关键点

  1. 本研究是首次对依那西普治疗 SAPHO 患者有效性的长期随访。

  2. 本研究揭示了 SAPHO 患者不同的骨病变部位可能具有不同的临床特征。

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本文引用的文献

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Arthritis Res Ther. 2020 Sep 14;22(1):216. doi: 10.1186/s13075-020-02309-6.
2
Iguratimod as a New Drug for Rheumatoid Arthritis: Current Landscape.艾拉莫德作为类风湿关节炎的新药:现状
Front Pharmacol. 2020 Feb 26;11:73. doi: 10.3389/fphar.2020.00073. eCollection 2020.
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Chronic non-bacterial osteomyelitis: a comparative study between children and adults.慢性非细菌性骨髓炎:儿童与成人的对比研究。
Pediatr Rheumatol Online J. 2019 Jul 23;17(1):49. doi: 10.1186/s12969-019-0353-2.
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Reduction of peripheral natural killer cells in patients with SAPHO syndrome.SAPHO综合征患者外周自然杀伤细胞减少。
Clin Exp Rheumatol. 2019 Jan-Feb;37(1):12-18. Epub 2018 Jun 25.
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Clinical symptoms, imaging, and treatment of SAPHO syndrome: a single‑center study of 52 cases.SAPHO综合征的临床症状、影像学表现及治疗:一项52例单中心研究
Pol Arch Intern Med. 2018 Jun 30;128(6):396-399. doi: 10.20452/pamw.4261. Epub 2018 May 4.
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Serum levels of proinflammatory, anti-inflammatory cytokines, and RANKL/OPG in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.滑膜炎、痤疮、脓疱病、骨肥厚和骨炎(SAPHO)综合征患者血清中促炎、抗炎细胞因子及RANKL/OPG水平
Mod Rheumatol. 2019 May;29(3):523-530. doi: 10.1080/14397595.2018.1469580. Epub 2018 Sep 6.
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Pustulotic Arthro-Osteitis (Sonozaki Syndrome): A Case Report and Review of Literature.脓疱性关节骨炎(园崎综合征):一例报告及文献复习
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