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

Czech version

Authors: Zdeněk Adam 1;  Anna Šedivá 2;  David Zeman 3;  Zdeněk Fojtík 1;  Hana Petrášová 4;  Jana Diatková 1;  Miroslav Tomíška 1;  Zdeněk Král 1;  Jana Treglerová 5;  Vojtěch Peřina 5;  Kateřina Kamaradová 6;  Zdenka Adamová 7;  Luděk Pour 1
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno 1;  Oddělení imunologie FN Motol a 2. LF UK, Praha 2;  Ústav laboratorní medicíny – Oddělení klinické biochemie, LF MU a FN Brno 3;  Klinika radiologie a nukleární medicíny LF MU a FN Brno 4;  Klinika ústní, čelistní a obličejové chirurgie LF MU a FN Brno 5;  Fingerlandův ústav patologie, Fakultní nemocnice Hradec Králové 6;  Ordinace praktického lékaře pro děti a dorost, Obilní trh, Brno 7
Published in: Vnitř Lék 2023; 69(E-5): 4-14
Category:
doi: https://doi.org/10.36290/vnl.2023.065

Overview

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:

Osteomyelitis – anakinra – denosumab – osteitis – SAPHO syndrome – multiple bone osteolysis

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Internal Medicine

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2023 Issue E-5
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