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

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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.

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Data availability

The dataset supporting the conclusions of this article is included within the article.

Abbreviations

SAPHO :

Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis

ACW :

Anterior chest wall

NSAIDS :

Non-steroidal anti-inflammatory drugs

DMARDS :

Disease-modifying anti-rheumatic drugs

TNF-α :

Tumor necrosis factorα

IGU :

Iguratimod

MTX :

Methotrexate

SSZ :

Sulfasalazine

RANKL :

Receptor activator of NF-κB ligand

OPG :

Osteoprotegerin

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Authors and Affiliations

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

    Hongji Duan & Xiaoli Deng

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

    Shuang Gao

  3. Department of Radiology, Peking University Third Hospital, Beijing, China

    Lihua Zhang

  4. Department of Nuclear Medicine, Peking University Third Hospital, Beijing, China

    Le Song

  5. Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China

    Jiayu Zhai

Authors
  1. Hongji Duan
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  2. Shuang Gao
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  3. Lihua Zhang
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  4. Le Song
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  5. Jiayu Zhai
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  6. Xiaoli Deng
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Contributions

Hongji Duan and Xiaoli Deng wrote the manuscript. Shuang Gao, Lihua Zhang, Le Song, and Jiayu Zhai prepared the tables. All authors reviewed the paper.

Corresponding author

Correspondence to Xiaoli Deng.

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Ethical approval

The Ethics Committee of Peking University Third Hospital approved this study.

RECORD guidelines were followed.

Written informed consent was received from each patient.

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Duan, H., Gao, S., Zhang, L. et al. The clinical characteristics and prognosis of patients with SAPHO syndrome——a real-world cohort study. Clin Rheumatol 43, 561–568 (2024). https://doi.org/10.1007/s10067-023-06782-7

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  • DOI: https://doi.org/10.1007/s10067-023-06782-7

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