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The effectiveness of treatments for patients with SAPHO syndrome: a follow-up study of 24 cases from a single center and review of literature

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Abstract

Objectives

To evaluate the effectiveness of current treatments for SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) by delineating the therapeutic choices and the outcome of patients in a medical center of China and review of the literature.

Method

An observational study was performed on patients diagnosed as SAPHO syndrome in the Peking University First Hospital from 2009 to 2015. Clinical data including osteoarticular and cutaneous manifestations, laboratory and medical imaging findings, treatments, and outcomes were analyzed retrospectively. A literature detailing the usage of medicines and SAPHO syndrome patient responses to treatment were selected.

Results

Clinical data of 24 patients were analyzed in this study. The mean age was 42.4 ± 15.5 years old at the time of diagnosis. Of a total of 17 patients that received the combination treatments of NSAIDs and DMARDs, 15 of them experienced an improvement in their symptoms. Bisphosphonates were given to 18 patients. Four patients were treated with TNF blockers, and one of them had adalimumab ineffective and then improved by add-on of DMARDs. The mean follow-up period was 2.5 years. Twenty-one patients (87.5%) had a favorable outcome and exhibited improved disease condition in the last follow-up.

Conclusions

The majority of patients with SAPHO syndrome respond well to combination therapy of NSAIDs, DMARDs, and bisphosphonates. TNF blockers are effective in a patient refractory to NSAID and DMARDs. However, in case TNF blockers are ineffective, add-on of DMARDs may be effective. Thus, the proper application of conventional DMARDs is still worth a position in the treatment of SAPHO syndrome.

Key Points

•NSAIDs and DMARDs and/or bisphosphonates can alleviate symptoms of the majority of patients with SAPHO syndrome.

•TNF blockers are effective in patients who do not respond well to NSIADs and DMARDs.

•In case TNF blockers are ineffective, add-on of conventional DMARDs may be effective.

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References

  1. Witt M, Meier J, Hammitzsch A, Proft F, Schulze-Koops H, Grunke M (2014) Disease burden, disease manifestations and current treatment regimen of the SAPHO syndrome in Germany: results from a nationwide patient survey. Semin Arthritis Rheum 43(6):745–750. https://doi.org/10.1016/j.semarthrit.2013.10.010

    Article  PubMed  Google Scholar 

  2. Rohekar G, Inman RD (2006) Conundrums in nosology: synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome and spondylarthritis. Arthritis Rheum 55(4):665–669. https://doi.org/10.1002/art.22087

    Article  CAS  PubMed  Google Scholar 

  3. Dumolard A, Gaudin P, Juvin R, Bost M, Peoc’h M, Phelip X (1999) SAPHO syndrome or psoriatic arthritis? A familial case study. Rheumatology (Oxford, England) 38(5):463–467. https://doi.org/10.1093/rheumatology/38.5.463

    Article  CAS  Google Scholar 

  4. Aljuhani F, Tournadre A, Tatar Z, Couderc M, Mathieu S, Malochet-Guinamand S, Soubrier M, Dubost JJ (2015) The SAPHO syndrome: a single-center study of 41 adult patients. J Rheumatol 42(2):329–334. https://doi.org/10.3899/jrheum.140342

    Article  PubMed  Google Scholar 

  5. Kahn MF, Khan MA (1994) The SAPHO syndrome. Baillieres Clin Rheumatol 8(2):333–362. https://doi.org/10.1016/s0950-3579(94)80022-7

    Article  CAS  PubMed  Google Scholar 

  6. Mateo L, Sanint J, Rodríguez Muguruza S, Martínez Morillo M, Pérez Andrés R, Domenech Puigcerver S (2017) SAPHO syndrome presenting as an osteolytic lesion of the neck. Reumatol Clin 13(1):44–47. https://doi.org/10.1016/j.reuma.2015.11.016

    Article  PubMed  Google Scholar 

  7. Takigawa T, Tanaka M, Nakahara S, Sugimoto Y, Ozaki T (2008) SAPHO syndrome with rapidly progressing destructive spondylitis: two cases treated surgically. Eur Spine J 2(Suppl 2):S331–S337. https://doi.org/10.1007/s00586-008-0663-4

    Article  Google Scholar 

  8. Rukavina I (2015) SAPHO syndrome: a review. J Child Orthop 9(1):19–27. https://doi.org/10.1007/s11832-014-0627-7

    Article  PubMed  PubMed Central  Google Scholar 

  9. Hayem G, Bouchaud-Chabot A, Benali K, Roux S, Palazzo E, Silbermann-Hoffman O, Kahn MF, Meyer O (1999) SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum 29(3):159–171. https://doi.org/10.1016/s0049-0172(99)80027-4

    Article  CAS  PubMed  Google Scholar 

  10. Earwaker JW, Cotten A (2003) SAPHO: syndrome or concept? Imaging findings. Skelet Radiol 32(6):311–327. https://doi.org/10.1007/s00256-003-0629-x

    Article  CAS  Google Scholar 

  11. Ichikawa J, Sato E, Haro H, Ando T, Maekawa S, Hamada Y (2009) Successful treatment of SAPHO syndrome with an oral bisphosphonate. Rheumatol Int 29(6):713–715. https://doi.org/10.1007/s00296-008-0760-z

    Article  PubMed  Google Scholar 

  12. Sabugo F, Liberman C, Niedmann JP, Soto L, Cuchacovich M (2008) Infliximab can induce a prolonged clinical remission and a decrease in thyroid hormonal requirements in a patient with SAPHO syndrome and hypothyroidism. Clin Rheumatol 27(4):533–535. https://doi.org/10.1007/s10067-007-0767-y

    Article  PubMed  Google Scholar 

  13. Court C, Charlez C, Molina V, Clerc D, Miquel A, Nordin JY (2005) Isolated thoracic spine lesion: is this the presentation of a SAPHO syndrome? A case report. Eur Spine J 14(7):711–715. https://doi.org/10.1007/s00586-004-0791-4

    Article  PubMed  Google Scholar 

  14. Skoura E, Giannopoulou C, Zaromytidou E, Pianou N, Houssianakou I (2007) A case of non-malignant multifocal lesions on the methyl diphosphonate technetium-99m bone scan with enteropathy. SAPHO syndrome. Hellenic J Nucl Med 10(1):26–28

    Google Scholar 

  15. Galadari H, Bishop AG, Venna SS, Sultan E, Do D, Zeltser R (2009) Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome treated with a combination of isotretinoin and pamidronate. J Am Acad Dermatol 61(1):123–125. https://doi.org/10.1016/j.jaad.2008.10.046

    Article  PubMed  Google Scholar 

  16. Soyfoo MS, Gangji V, Margaux J (2010) Successful treatment of SAPHO syndrome with ibandronate. J Clin Rheumatol 16(5):253. https://doi.org/10.1097/RHU.0b013e3181e9be89

    Article  PubMed  Google Scholar 

  17. Just A, Adams S, Brinkmeier T, Barsegian V, Lorenzen J, Schilling F, Frosch P (2008) Successful treatment of primary chronic osteomyelitis in SAPHO syndrome with bisphosphonates. Journal der Deutschen Dermatologischen Gesellschaft 6(8):657–660. https://doi.org/10.1111/j.1610-0387.2008.06588.x

    Article  PubMed  Google Scholar 

  18. Spyridonidis T, Giannakenas C, Papandrianos N, Barla P, Apostolopoulos DJ (2007) Two cases of synovitis, acne, pustulosis, osteitis--SAPHO syndrome. Hellenic J Nucl Med 10(2):109–112

    Google Scholar 

  19. Amital H, Applbaum YH, Aamar S, Daniel N, Rubinow A (2004) SAPHO syndrome treated with pamidronate: an open-label study of 10 patients. Rheumatology (Oxford, England) 43(5):658–661. https://doi.org/10.1093/rheumatology/keh149

    Article  CAS  Google Scholar 

  20. Courtney PA, Hosking DJ, Fairbairn KJ, Deighton CM (2002) Treatment of SAPHO with pamidronate. Rheumatology (Oxford, England) 41(10):1196–1198. https://doi.org/10.1093/rheumatology/41.10.1196

    Article  CAS  Google Scholar 

  21. Zwaenepoel T, Vlam K (2016) SAPHO: treatment options including bisphosphonates. Semin Arthritis Rheum 46(2):168–173. https://doi.org/10.1016/j.semarthrit.2016.04.004

    Article  CAS  PubMed  Google Scholar 

  22. Susanto CR, Nanayakkara PW (2003) The skin is the clue. Eur J Intern Med 14(8):493–494. https://doi.org/10.1016/j.ejim.2003.08.005

    Article  PubMed  Google Scholar 

  23. Wagner AD, Andresen J, Jendro MC, Hülsemann JL, Zeidler H (2002) Sustained response to tumor necrosis factor alpha-blocking agents in two patients with SAPHO syndrome. Arthritis Rheum 46(7):1965–1968. https://doi.org/10.1002/art.10539

    Article  PubMed  Google Scholar 

  24. Kim CH, Kadhim S, Julien C (2014) Treatment of pain in SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome. PM & R 6(1):92–95. https://doi.org/10.1016/j.pmrj.2013.08.610

    Article  Google Scholar 

  25. Ben Abdelghani K, Dran DG, Gottenberg JE, Morel J, Sibilia J, Combe B (2010) Tumor necrosis factor-alpha blockers in SAPHO syndrome. J Rheumatol 37(8):1699–1704. https://doi.org/10.3899/jrheum.091086

    Article  CAS  PubMed  Google Scholar 

  26. Colina M, Govoni M, Orzincolo C, Trotta F (2009) Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a single center study of a cohort of 71 subjects. Arthritis Rheum 61(6):813–821. https://doi.org/10.1002/art.24540

    Article  PubMed  Google Scholar 

  27. Kamata Y, Minota S (2015) Successful treatment of a patient with SAPHO syndrome with certolizumab pegol. Rheumatol Int 35(9):1607–1608. https://doi.org/10.1007/s00296-015-3263-8

    Article  PubMed  Google Scholar 

  28. Pottecher P, Loffroy R, Estivalet L, Thouant P, Ornetti P, Ricolfi F, Krausé D (2014) SAPHO syndrome revealed by sclerosing mandibular osteomyelitis. Diagn Intervent Imaging 95(9):885–887. https://doi.org/10.1016/j.diii.2014.02.004

    Article  CAS  Google Scholar 

  29. Ornetti P, Pottecher P (2014) Clinical images: refractory SAPHO syndrome involving the mandible. Arthritis Rheumatol (Hoboken, NJ) 66(7):1966. https://doi.org/10.1002/art.38632

    Article  Google Scholar 

  30. Marí A, Morla A, Melero M, Schiavone R, Rodríguez J (2014) Diffuse sclerosing osteomyelitis (DSO) of the mandible in SAPHO syndrome: a novel approach with anti-TNF therapy. Systematic review. J Craniomaxillofac Surg 42(8):1990–1996. https://doi.org/10.1016/j.jcms.2014.09.004

    Article  PubMed  Google Scholar 

  31. Arias-Santiago S, Sanchez-Cano D, Callejas-Rubio JL, Fernández-Pugnaire MA, Ortego-Centeno N (2010) Adalimumab treatment for SAPHO syndrome. Acta Derm Venereol 90(3):301–302. https://doi.org/10.2340/00015555-0822

    Article  PubMed  Google Scholar 

  32. Eleftheriou D, Gerschman T, Sebire N, Woo P, Pilkington CA, Brogan PA (2010) Biologic therapy in refractory chronic non-bacterial osteomyelitis of childhood. Rheumatology (Oxford, England) 49(8):1505–1512. https://doi.org/10.1093/rheumatology/keq122

    Article  CAS  Google Scholar 

  33. Rosero A, Ruano R, Martin M, Hidalgo C, Garcia-Talavera J (2013) Acute venous thrombosis as complication and clue to diagnose a SAPHO syndrome case. A case report. Acta Reumatol Port 38(3):203–206

    CAS  PubMed  Google Scholar 

  34. Hayem G (2004) SAPHO syndrome. La Revue du Praticien 54(15):1635–1636

    PubMed  Google Scholar 

  35. Qiu D, Kao PN (2003) Immunosuppressive and anti-inflammatory mechanisms of triptolide, the principal active diterpenoid from the Chinese medicinal herb Tripterygium wilfordii Hook. f. Drugs R&D 4(1):1–18. https://doi.org/10.2165/00126839-200304010-00001

    Article  CAS  Google Scholar 

  36. Li C, Sun X, Cao Y, Xu W, Zhang W, Dong Z (2017) Case report: remarkable remission of SAPHO syndrome in response to Tripterygium wilfordii hook f treatment. Medicine 96(47):e8903. https://doi.org/10.1097/md.0000000000008903

    Article  PubMed  PubMed Central  Google Scholar 

  37. Assmann G, Kueck O, Kirchhoff T, Rosenthal H, Voswinkel J, Pfreundschuh M, Zeidler H, Wagner AD (2009) Efficacy of antibiotic therapy for SAPHO syndrome is lost after its discontinuation: an interventional study. Arthritis Res Ther 11(5):R140. https://doi.org/10.1186/ar2812

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Eyrich GK, Langenegger T, Bruder E, Sailer HF, Michel BA (2000) Diffuse chronic sclerosing osteomyelitis and the synovitis, acne, pustolosis, hyperostosis, osteitis (SAPHO) syndrome in two sisters. Int J Oral Maxillofac Surg 29(1):49–53

    Article  CAS  Google Scholar 

  39. Li C, Zhao Y, Zuo Y, Zhou Y, Zhang F, Liu S, Zhu Q, Chen J, Zhang W, Xu W, Gu Z, Li L, Li F, Tao W, Cao Y, Sun X, Jing H, Chen H, Zhang S, Dong Z, Liu J, Shi X, Hao W, Qiu G, Zhang W, Wu N, Wu Z (2019) Efficacy of bisphosphonates in patients with synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a prospective open study. Clin Exp Rheumatol 37(4):663–669

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Rheumatology and Clinical Immunology Department, Peking University First Hospital, Beijing, 10034, China

    Hong Huang, Zhuoli Zhang, Juan Zhao, Yanjie Hao & Wei Zhou

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  1. Hong Huang
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  2. Zhuoli Zhang
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  3. Juan Zhao
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  5. Wei Zhou
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Correspondence to Wei Zhou.

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Huang, H., Zhang, Z., Zhao, J. et al. The effectiveness of treatments for patients with SAPHO syndrome: a follow-up study of 24 cases from a single center and review of literature. Clin Rheumatol 40, 1131–1139 (2021). https://doi.org/10.1007/s10067-020-05322-x

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  • DOI: https://doi.org/10.1007/s10067-020-05322-x

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