Summary
The acronym SAPHO stands for morbid alterations of the osteoarticular system: synovitis; acne; pustulosis), often psoriatic, mainly palmo-plantar, hyperostosis); as well as osteitis. As described by Kahn et al. in 1994, three diagnostic criteria characterize SAPHO syndrome: 1) multifocal osteomyelitis with or without skin manifestations; 2) sterile acute or chronic joint inflammation associated with either pustular psoriasis or palmo-plantar pustulosis, or acne, or hidradenitis; 3) sterile osteitis in the presence of one of the skin manifestations. According to Kahn, one of criteria is sufficient for the diagnosis of SAPHO. The syndrome is often chronic and eventually self-healing, though never septic or malignant, it is clinically heterogeneous, covering several diseases. Definite diagnosis can be hard to establish. For each case, clinical, radiological and histopathological signs need to be taken into account. The causes of SAPHO syndrome are hardly known, common genetic factors are still unclear, apart from a few exceptions. Incidence and prevalence for SAPHO syndrome are still unknown, no data are available, prevalence of CRMO (chronic recurrent multifocal osteomyelitis, one of the frequent manifestation of SAPHO) is estimated at 0.04% in Germany. Diagnosis confirmation relies on RMI, scintigraphy or histopathology. Multidisciplinary follow-up is required. Surgical operation is exceptional. Treatment by non-steroid anti-inflammatory drugs is symptomatic. Treatment of CRMO (Chronic recurrent multifocal osteomyelitis, one of the frequent feature of SAPHO) with antibiotics is not effective. Calcitonin or diphosphonates may have been proposed owing to their osteotropic effects. Very recently by analogy with spondylarthropathies, a treatment with TNF-inhibitors was successfully proposed in some cases. We recommend applying the following treatment: combination of anti-inflammatory and immunomodulating drugs (Azithromycin) and hormonal osteotropic drugs (calcitonin)..*Author: Prof F. Schilling (October 2004)*.
