SAPHO syndrome with TMJ involvement: review of the literature and case presentation

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare group of sterile, inflammatory osteoarticular disorders classically associated with skin manifestations. The etiology is unknown but probably involves genetic, infectious, and immunological components. The characteristic feature of the disease is found in the bone lesions, which typically involve the anterior chest wall and axial skeleton. In the literature review, six case reports discussed involvement of the TMJ. Treatment of SAPHO is geared toward symptom management as there is no cure. Surgery for mandibular lesions is usually a last resort as results are reported to be temporary with symptoms recurring within a year. Surgery appears to be performed early after diagnosis of TMJ related pathology; probably because lesions affecting the TMJ involve some limitation in mouth opening with varying degrees of ankylosis. The authors provide a literature review and describe a case of SAPHO syndrome with ankylosis of the left TMJ. The patient was treated with joint reconstruction using a patient-fitted total joint prosthesis (TMJ Concepts Inc., Ventura CA) in single stage surgery. This paper is the first to report maxillary involvement in SAPHO syndrome.