The SAPHO syndrome in diffuse sclerosing osteomyelitis of the mandible: an oral and maxillofacial surgery perspective.

OBJECTIVES

To compare the characteristics of patients with isolated diffuse sclerosing osteomyelitis of the mandible (DSOM) and those with DSOM along with systemic bone, joint, and cutaneous lesions, and discuss the relationship between DSOM and the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome from the perspective of oral and maxillofacial surgery.

STUDY

DESIGN

A total of 446 DSOM patients were included. They were divided into simple DSOM and syndrome-type DSOM groups based on their systemic manifestations. Their demographic data, clinical features, imaging findings (maxillofacial CT and bone scintigraphy), and laboratory results (bone turnover markers (BTMs) and inflammatory markers) were compared.

RESULTS

Compared to patients with syndrome-type DSOM, those with simple DSOM had a younger median age of onset (13 vs 25 years), a higher proportion of women (69.8% vs 45.5%), lower bilateral mandibular involvement (18.3% vs 29.9%), a higher prevalence of periosteal reaction (70.6% vs 59.7%, p = .017), elevated BTMs (osteocalcin: 49.73 vs 21.69 ng/mL; β-CTX: 0.94 vs 0.53 ng/mL), and lower C-reactive protein levels (3.00 vs 7.48 mg/L).

CONCLUSION

Nearly half of all patients with DSOM exhibited systemic bone, joint, or cutaneous manifestations. The characteristics of simple DSOM and syndrome-type DSOM are highly overlapped, but the differences in age, gender distribution, radiographic findings and bone metabolic activity suggest that they may be different stages or subtypes of the same disease.