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Clinical characteristics of pediatric synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome: the first Chinese case series from a single center

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Abstract

Introduction

Pediatric SAPHO syndrome is regarded as the equivalent of chronic recurrent multifocal osteomyelitis or chronic non-bacterial osteomyelitis. This study aimed to evaluate the clinical features and treatment options for Chinese pediatric patients with SAPHO syndrome.

Method

We conducted a single-center, retrospective study on a sample of 24 pediatric patients with SAPHO syndrome who were diagnosed at Peking Union Medical College Hospital from April 2014 to August 2018. The demographic, clinical, laboratory, imaging, histological, and therapeutic data were collected and analyzed.

Results

A total of 15 boys and 9 girls were included. The mean age of onset of bone and skin symptoms was 11.7 ± 3.8 and 14.4 ± 2.7 years, respectively. The mean follow-up period was 39.2 months. Seventeen patients had skin manifestations (46% had severe acne, 100% were boys; 21% had palmoplantar pustulosis, 100% were girls). Bone lesions were localized in four of the following major regions: anterior chest wall (42%), mandible (29%), peripheral bones (50%), and spine and sacroiliac joints (21%). Six patients had been treated with non-steroidal anti-inflammatory drugs, 10 with bisphosphonate, 10 with a tumor necrosis factor-α antagonist, and 1 with glucocorticoids, with variable responses. A total of 70% of the patients had complete remission after bisphosphonate or TNF-α antagonist therapy.

Conclusion

Pediatric patients with SAPHO syndrome have different characteristics from other cohorts in the sex ratio, frequency of mandibular involvement, and sex distribution of skin lesions. Bisphosphonate and TNF-α antagonists show a favorable response in pediatric SAPHO syndrome treatment.

Key points

•Being the first study that describes an Asian pediatric SAPHO case series.

•Chinese pediatric patients with SAPHO syndrome have different characteristics from Chinese adult patients and Caucasian pediatric patients.

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Data availability

All data and material generated or used during the study appear in the submitted article. Detailed data of patients included in this study are available upon request by contact with the corresponding author.

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Acknowledgments

We thank Ellen Knapp, PhD, from Liwen Bianji, Edanz Group China (www.liwenbianji.cn/ac), for editing the English text of a draft of this manuscript.

Funding

This work was supported in part by grants from National Natural Science Foundation of China (81822030 to W.N.), Beijing Natural Science Foundation (7172175 to W.N.), the Central Level Public Interest Program for Scientific Research Institute (2018RC31003 to W.N.), CAMS Innovation Fund for Medical Sciences (2017-I2M-3-001 to L.C.), and Capital Medical Research and Development Fund (2016-4-40112 to L.C.). The role of the funding body was in the design and collection of data of this study.

Author information

Author notes

  1. Nan Wu, Yuming Shao and Jianwei Huo contributed equally to this work.

Authors and Affiliations

  1. Department of Orthopedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Nan Wu

  2. Department of genetic research, Beijing Key Laboratory for Genetic Research of Skeletal Deformity, Beijing, China

    Nan Wu

  3. Medical Research Center of Orthopedics, Chinese Academy of Medical Sciences, Beijing, China

    Nan Wu

  4. Institute of Clinical Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Yuming Shao, Fa Zhang, Chenyang Yu & Yanying Yu

  5. Department of Radiology, Beijing Hospital of Traditional Chinese Medicine Affiliated to Capital Medical University, Beijing, China

    Jianwei Huo & Yanan Zhang

  6. Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Yihan Cao

  7. Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Hongli Jing

  8. Department of Traditional Chinese Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China, No. 1 Shuaifuyuan, Beijing, 100730, China

    Chen Li

  9. Department of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Hongmei Song

  10. Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Wen Zhang

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  1. Nan Wu
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Contributions

Nan Wu, Yuming Shao, and Jianwei Huo contributed equally this study and should be regarded as co-first authors. Chen Li, Nan Wu, and Jianwei Huo designed the manuscript. Yuming Shao, Yanying Yu, and Chenyang Yu carried out the literature search. Yuming Shao, Nan Wu, Yanan Zhang, Yihan Cao, Hongli Jing, Fa Zhang, Hongmei Song, and Wen Zhang contributed to data extraction, and data analysis. Nan Wu, Jianwei Huo, and Yihan Cao supports for data interpretation. Yuming Shao drafted the manuscript. All authors approved the final version to be published after critically revising the manuscript for important intellectual content.

Corresponding author

Correspondence to Chen Li.

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Disclosures None.

Ethics approval

This study was approved by the Institutional Review Board of Peking Union Medical College Hospital (approval number: ZS-944).

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Written informed consent was obtained from each patient.

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Not applicable.

Code availability

We used SPSS 22.0 for data analysis and no code were generated or used during the study.

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Wu, N., Shao, Y., Huo, J. et al. Clinical characteristics of pediatric synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome: the first Chinese case series from a single center. Clin Rheumatol 40, 1487–1495 (2021). https://doi.org/10.1007/s10067-020-05393-w

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