The clinical characteristics and prognosis of patients with SAPHO syndrome——a real-world cohort study,Clinical Rheumatology

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The clinical characteristics and prognosis of patients with SAPHO syndrome——a real-world cohort study
Clinical Rheumatology ( IF 2.9 ) Pub Date : 2023-09-27 , DOI: 10.1007/s10067-023-06782-7
Hongji Duan ₁ , Shuang Gao ₂ , Lihua Zhang ₃ , Le Song ₄ , Jiayu Zhai ₅ , Xiaoli Deng ₁

Affiliation

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.

中文翻译：

SAPHO综合征患者的临床特征及预后——一项真实世界队列研究

目标

我们的目的是分析滑膜炎、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果。

方法

收集64例SAPHO综合征患者的临床资料，并对27例患者的治疗和转归进行随访。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，以及两个部位都有骨病变受累。比较临床特征和结果。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）有ACW和脊柱受累。 25.00% (16/64) 的患者观察到周围关节受累。仅脊柱受累的患者比仅 ACW 受累的患者年龄更大 ( p = 0.006)。同时患有 ACW 和脊柱受累的患者比仅受累 ACW 的患者年龄更大 ( p = 0.002)，并且诊断延迟时间更长 ( p = 0.015)。周围关节受累的患者比无周围关节受累的患者年轻（ p = 0.028）。随访期间，88.89%（24/27）的患者预后良好。 22例患者接受非甾体类抗炎药+艾拉莫德（IGU）治疗，90.91%（20/22）的结局得到改善。