Mandibular involvement in SAPHO syndrome: a retrospective study

Abstract

Background Mandible osteomyelitis can occur in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, a rare chronic inflammatory disease; however, few studies have explored its characteristics and management. Methods We reviewed the medical records of consecutive SAPHO patients with mandible involvement diagnosed in Peking Union Medical College Hospital from September 2014 to July 2019. Demographic, clinical, laboratory, and imaging data were collected at baseline. Prescription data and follow-up magnetic resonance imaging (MRI) and cone beam computed tomography (CBCT) images were collected from the hospital information system. An electronic questionnaire was distributed to all patients to obtain their latest symptoms. Results A total of 26 SAPHO patients with mandibular involvement were involved, all of whom responded to the questionnaire (38.5% male; median age, 28 years; median follow-up duration, 2.1 years). Ten patients (38.5%) had undergone an oral procedure 1 month before the onset of mandibular symptoms. All 14 of the patients who underwent a surgical intervention relapsed within a median duration of 2 months (range 0.25–4.0 months), and 24 patients (92.3%) achieved improvement with conservative treatment. Following bisphosphonate treatment, remission of bone marrow oedema and osteolysis was observed on MRI and CBCT, and 5 patients receiving bisphosphonates with follow-up CBCT after remission did not relapse in 5.4 months (mean 6.0, range 3.2–9.9 months). Conclusion Mandibular involvement of SAPHO syndrome predominantly occurs in young women. Dental procedures are a possible risk factor. Conservative treatment, especially intravenous bisphosphonates, can lead to oral improvement.

SAPHO syndrome can cause masseter muscle involvement

“…SAPHO syndrome is a rare disease characterized by osteoarticular and dermatological manifestations 1 . The most commonly affected area of bone damage is the anterior chest wall, and mandibular involvement can also be observed in our cohort 2 . Lesions involving the masseter muscle have been rarely reported, and we are the first to report masseter muscle involvement in SAPHO syndrome.…”

“…1 The most commonly affected area of bone damage is the anterior chest wall, and mandibular involvement can also be observed in our cohort. 2 Lesions involving the masseter muscle have been rarely reported, and we are the first to report masseter muscle involvement in SAPHO syndrome. We suspect that enthesitis of the mandibular joint may result in the involvement of the masseter muscle.…”

New insights on multigenic autoinflammatory diseases

“…154,165,166 Clinical manifestations and diagnosis Patients with SAPHO syndrome present with sterile joint inflammation (synovitis), skin manifestations, such as severe acne and palmoplantar pustulosis, hyperostosis and non-infectious osteitis. 146,167,168 In CRMO/CNO, symptoms are heterogeneous, such as swelling of the affected area, severe bone pain, fever, malaise, and weight loss.…”

Pro and contra: is synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) a spondyloarthritis variant?

“…The synovial fluid analysis showed a mild inflammatory pattern [10]. Mandibular involvement in a form of diffuse sclerosing sterile osteomyelitis was reported predominantly among in young women [57]. Soft tissues adjacent to involved joints and bones can be affected, presenting as swelling, often considered a lymphatic or neoplastic mass.…”