The syndrome of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) is a rare condition characterized by a combination of osteo-articular and cutaneous manifestations. Hallmark of the disease is the presence of osteitic/hyperostotic lesions involving osteo-articular structures of the anterior chest wall (ACW). Emerging evidence suggests that SAPHO might be a primitive osteitis, probably related to polygenic autoinflammatory disorders. The rarity of the disease and its unknown aetio-pathogenesis make targeted therapy unavailable. In this report we describe for the first time effective treatment of SAPHO syndrome with the interleukin-1 receptor antagonist (IL-1Ra) anakinra.
Dysregulation of P2X7 receptor-inflammasome axis in SAPHO syndrome: successful treatment with anakinra
COLINA, Matteo;PIZZIRANI, Cinzia;FALZONI, Simonetta;BRUSCHI, MARCO;TROTTA, Francesco;DI VIRGILIO, Francesco
2010
Abstract
The syndrome of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) is a rare condition characterized by a combination of osteo-articular and cutaneous manifestations. Hallmark of the disease is the presence of osteitic/hyperostotic lesions involving osteo-articular structures of the anterior chest wall (ACW). Emerging evidence suggests that SAPHO might be a primitive osteitis, probably related to polygenic autoinflammatory disorders. The rarity of the disease and its unknown aetio-pathogenesis make targeted therapy unavailable. In this report we describe for the first time effective treatment of SAPHO syndrome with the interleukin-1 receptor antagonist (IL-1Ra) anakinra.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
