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Vnitr Lek ; 69(E-5): 4-14, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37827817

RESUMO

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.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Osteomielite , Masculino , Humanos , Adulto , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Síndrome de Hiperostose Adquirida/diagnóstico , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Denosumab/uso terapêutico , Fluordesoxiglucose F18/uso terapêutico , Osteomielite/tratamento farmacológico , Osteomielite/complicações , Osteomielite/microbiologia , Acne Vulgar/complicações , Acne Vulgar/diagnóstico
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