Vnitr Lek 2023, 69(5):E4-E14 | DOI: 10.36290/vnl.2023.065

Successful treatment of SAPHO syndrome (chronic nonbacterial osteomyelitis and acne) with anakinra and denosumab. Case report and review of therapy

Zdeněk Adam1, Anna Šedivá2, David Zeman3, Zdeněk Fojtík1, Hana Petrášová4, Jana Diatková1, Miroslav Tomíška1, Zdeněk Král1, Jana Treglerová5, Vojtěch Peřina5, Kateřina Kamarádová6, Zdenka Adamová7, Luděk Pour1
1 Interní hematologická a onkologická klinika LF MU a FN Brno
2 Oddělení imunologie FN Motol a 2. LF UK, Praha
3 Ústav laboratorní medicíny - Oddělení klinické biochemie, LF MU a FN Brno
4 Klinika radiologie a nukleární medicíny LF MU a FN Brno
5 Klinika ústní, čelistní a obličejové chirurgie LF MU a FN Brno
6 Fingerlandův ústav patologie, Fakultní nemocnice Hradec Králové
7 Ordinace praktického lékaře pro děti a dorost, Obilní trh, Brno

SAPHO is an acronym derived from capital letters of Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO). SAPHO syndrome is an umbrella term covering a constellation of bone lesions and skin manifestations. A 40-year-old male complained about his jaw and back pain, swelling of multiple joints and weight loss accompanied by physical deterioration and acne type skin lesions. Laboratory tests revealed abnormal elevation of inflammatory markers. Imaging studies illustrated multiple osteolytic bone lesions and paraosseal infiltrates. According to the set of criteria diagnosis of SAPHO syndrome was stated. The patient was treated with glucocorticoids and non-steroidal anti-inflammatory drugs (NSAIDs), but only high dose dexamethasone and prednisone were effective. Daily subcutaneous administration of anakinra at the dose of 100 mg was initiated due to limited response to more classical therapies. Because of planned mandibular osteosynthesis initiation of denosumab was preferred before bisphosphonates. Therapeutic response was confirmed by FDG-PET/MR after 5 months of anakinra and denosumab therapy, showing decreased accumulation of FDG in periosteal and paraosseal infiltrates. Inflammatory markers significantly decreased, bone pain deferred but skin manifestation receded only partially. Therefore the response was evaluated as partial remission.

Keywords: osteitis, osteomyelitis, SAPHO syndrome, anakinra, denosumab, multiple bone osteolysis.

Accepted: September 4, 2023; Published: September 12, 2023  Show citation

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Adam Z, Šedivá A, Zeman D, Fojtík Z, Petrášová H, Diatková J, et al.. Successful treatment of SAPHO syndrome (chronic nonbacterial osteomyelitis and acne) with anakinra and denosumab. Case report and review of therapy. Vnitr Lek. 2023;69(5):E4-14. doi: 10.36290/vnl.2023.065.
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    References

    1. Benhamou CL, Chamot AM, Kahn MF, et al. Synovitis­‑acne­‑pustulosis hyperostosis­‑osteomyelitis syndrome (SAPHO). A new syndrome among the spondyloarthropathies? Clin Exp Rheumatol. 1988;6(2):109-112. Go to PubMed...
    2. Němec P. Revmatologie pro praxi. 2., přepracované a doplněné vydání. Praha: Grada Publishing, 2021;819 s
    3. Rukavina I. SAPHO syndrome: a review. J Child Orthop. 2015;9(1):19-27. Go to original source... Go to PubMed...
    4. Kishimoto M, Taniguchi Y, Tsuji S, et al. SAPHO syndrome and pustulotic arthro­‑osteitis.
    5. Girschick H, Finetti M, Orlando F, et al. The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry. Rheumatology. 2018;57(8):1504-1506. Go to original source... Go to PubMed...
    6. Roy NBA, Zaal AI, Hall G, et al. Majeed syndrome: description of a novel mutation and therapeutic response to bisphosphonates and IL-1 blockade with anakinra. Rheumatology. (Oxford) 2020;59(2):448-451. Go to original source... Go to PubMed...
    7. Li C, Xiang Y, Wu X, Cao Y, et al. Serum IgG4 elevation in SAPHO syndrome: does it unmask a disease activity marker? Clin Exp Rheumatol. 2020;38(1):35-41.
    8. Zhao Y, Sato TS, Nielsen SM, et al. Development of a scoring tool for chronic nonbacterial osteomyelitis magnetic resonance imaging and evaluation of its interrater reliability. J Rheumatol. 2020;47(5):739-747. Go to original source... Go to PubMed...
    9. Korčáková E, Jeremiáš P, Ríčař J, et al. SAPHO - vzácná diagnóza. Česká radiologie. 2017;71(3):201-206.
    10. Skotáková J, Červinková I, Šenkyřík J, et al. Význam MR vyšetření u diagnózy chronická rekurentní multifokální osteomyelitida Česká radiologie. 2017;71(3):197-200.
    11. Watanabe S, Sawa N, Mizuno H, et al. Bone histomorphometric and immunohistological analysis for hyperostosis in a patient with SAPHO syndrome: A case report. Bone Rep. 2020;13:100296. doi:10.1016/j.bonr.2020.100296 Go to original source... Go to PubMed...
    12. Kahn MS: Proriatic arthritis and synovitis, acne, pustulosis, hyperostosis et osteitis. Current Opinion in Rheumatology. 1993;5:428-435. Go to original source... Go to PubMed...
    13. Hayem G. SAPHO syndrome. Rev Prat. 2004;54(15):1635-1636. Go to PubMed...
    14. Bouchalová K, Fráňová J, Schüller M, et al. Chronická rekurentní multifokální osteomyelitida (CRMO) v dětském věku - přehled a vlastní výsledky. Česká Revmatol. 2019;27(3):116-124.
    15. Růžičková­‑Jarešová L. Machovcová A. SAPHO syndrom. Dermatologie pro praxi. 2009;3(4):188-190.
    16. Cheng W, Li F, Tian J, et al. New Insights in the Treatment of SAPHO Syndrome and Medication Recommendations. J Inflamm Res. 2022;15:2365-2380. Go to original source... Go to PubMed...
    17. Otto S, Troeltzsch M, Burian E, et al. Ibandronate treatment of diffuse sclerosing osteomyelitis of the mandible: pain relief and insight into pathogenesis. J Craniomaxillofac Surg. 2015;43:1837-42. Go to original source... Go to PubMed...
    18. Vekic DA, Woods J, Lin P, et al. SAPHO syndrome associated with hidradenitis suppurativa and pyoderma gangrenosum successfully treated with adalimumab and methotrexate: a case report and review of the literature. Int J Dermatol. 2018;57(1):10-18. Go to original source... Go to PubMed...
    19. Vilar­‑Alejo J, Dehesa L, de la Rosa­‑del Rey P, et al. SAPHO syndrome with unusual cutaneous manifestations treated successfully with etanercept. Acta Derm Venereol. 2010;90(5):531-532. Go to original source... Go to PubMed...
    20. Colina M, Pizzirani C, Khodeir M, et al. Dysregulation of P2X7 receptor­‑inflammasome axis in SAPHO syndrome: successful treatment with anakinra. Rheumatology. 2010;49(7):1416-1418. Go to original source... Go to PubMed...
    21. Daoussis D, Konstantopoulou G, Kraniotis P, et al. Biologics in SAPHO syndrome: a systematic review. Semin. Arthritis Rheum. 2019;48(4):618-625. Go to original source... Go to PubMed...
    22. Pardeo M, Pires, S, Marafon D, et al. Anakinra in a cohort of children with chronic nonbacterial osteomyelitis. J. Rheumatol. 2017;44(8):1231-1238. Go to original source... Go to PubMed...
    23. Maniscalco V, Abu­‑Rumeileh S, Mastrolia MV, et al. The off­‑label use of anakinra in pediatric systemic autoinflammatory diseases. Ther Adv Musculoskelet, DiS. 2020;12:1759720X20959575. doi:10.1177/1759720X20959575. Go to original source... Go to PubMed...
    24. Firinu D, Garcia­‑Larsen V. SAPHO Syndrome: Current Developments and Approaches to Clinical Treatment. Curr Rheumatol Rep. 2016;18(6):35. doi: 10.1007/s11926-016-0583-y Go to original source... Go to PubMed...
    25. Wendling D, Prati C, Aubin F. Anakinra treatment of SAPHO syndrome: short­‑term results of an open study. Ann. Rheum, DiS. 2012;71(6):1098-1100. Go to original source... Go to PubMed...
    26. Sun XC, Liu S, Li C, et al. Failure of tocilizumab in treating two patients with refractory SAPHO syndrome: a case report. J Int Med Res. 2018;46(12):5309-5315. Go to original source... Go to PubMed...
    27. Sun B, Cao Y, Wang L, Wang M, Li C. Successful treatment of refractory mandibular lesions in SAPHO syndrome with secukinumab. Rheumatology (Oxford). 2021;60(1):473-474. Go to original source... Go to PubMed...
    28. Wang L, Sun B, Li C. Clinical and radiological remission of osteoarticular and cutaneous lesions in SAPHO patients treated with secukinumab: a case series. J Rheumatol. 2021;48(6):953-955. Go to original source... Go to PubMed...
    29. Cornillier H, Kervarrec T, Tabareau­‑Delalande F, et al. Interstitial granulomatous dermatitis occurring in a patient with SAPHO syndrome one month after starting leflunomide, and subsequently disappearing with ustekinumab. Eur J Dermatol. 2016;26(6):614-615. Go to original source... Go to PubMed...
    30. Yang Q, Zhao Y, Li C, et al. Case report: successful treatment of refractory SAPHO syndrome with the JAK inhibitor tofacitinib. Medicine (Baltimore). 2018;97(25):e11149. Go to original source... Go to PubMed...
    31. Li C, Li Z, Cao Y, et al. Tofacitinib for the treatment of nail lesions and palmoplantar pustulosis in synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome. JAMA Dermatol. 2021;157(1):74-78. Go to original source... Go to PubMed...
    32. Adamo S, Nilsson J, Krebs A, et al. Successful treatment of SAPHO syndrome with apremilast. Br J Dermatol. 2018; 179(4):959-962. Go to original source... Go to PubMed...
    33. Wang L, Gong L, Zhang X, et al. Tripterygium wilfordii Hook F. in the treatment of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a clinical trial. Clin Rheumatol. 2021;40(6):2427-2438. Go to original source... Go to PubMed...
    34. Li C, Sun X, Cao Y,et al. Case report: remarkable remission of SAPHO syndrome in response to Tripterygium wilfordii hook f treatment. Medicine (Baltimore). 2017;96(47):e8903. Go to original source... Go to PubMed...
    35. Ščudla V, Horák P, Karásek D. Základy diferenciální diagnostiky ve vnitřním lékařství 1. vydání. Olomouc: Univerzita Palackého v Olomouci, 2021. 679 stran.
    36. Němec P, Řehák Z, Fabián P. Využití pozitronové emisní tomografie (18 F- FDG PET) v diagnostice chronických periaortitid. Vnitř Lék. 2008;54(11):1093-1099. Go to PubMed...

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