Sapho Syndrome

Imaging of chronic recurrent multifocal osteomyelitis of childhood first presenting with isolated primary spinal involvement

“…It is an unusual inflammatory process occurring in children and adolescents. It remains contentious as to whether this entity is separate from the SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) syndrome which occurs in adults or whether these two entities are part of a continuum [1,3,15]. In CRMO multiple osseous sites are usually involved and there is a fluctuating clinical course.…”

“…Chronic recurrent multifocal osteomyelitis (CRMO) is often a diagnosis of exclusion in patients who present with lytic or sclerotic bone findings and periosteal reaction with serological and histological findings negative for infection and tumor [1,2]. It is regarded by some authors as part of the spectrum of SAPHO syndrome [3]; however, others consider it to represent a distinct clinical and radiological entity [4]. More sinister pathologies such as histiocytosis X, leukemia, neuroblastoma and rhabdomyosarcoma [1,2] and osteomyelitis or bacterial discitis need to be excluded [1].…”

Loss and gain of bone in spondyloarthritis: what drives these opposing clinical features?

“…The earliest references of ‘osteitis’ in SpA were in relation to either radiographically described osteitis pubis, 27 erosion, 3 or as a bone scintigraphy abnormality 28 perhaps best referenced to SAPHO syndrome, 29,30 with abnormally increased localisation of technetium-99m-labelled diphosphonate. This radionuclide locates abnormally according to increased blood delivery, accessing bone tissue through changes in the vascular endothelium and binding to hydroxyapatite, which in turn correlates with bone turnover and reflects the rate of new bone formation.…”

Paediatric pustulotic arthro‐osteitis patient with an IL36RN variant, heterozygous c.115+6T>C, who was successfully treated with tonsillectomy: A case report and literature review

“…Despite the longstanding existence of the concept of pustulotic arthro‐osteitis (PAO), 2,3 the medical management within this field has remained ambiguous, partly due to the emergence of SAPHO syndrome and other contributing factors 4–6 . To address this issue, a guidance for PAO was published recently in Japan, to advance the diagnosis and management of this condition.…”

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Palmoplantar pustulosis (PPP) is a chronic inflammatory skin disease that typically manifests in individuals aged in their 40s and 50s.Based on the domestic receipt information, a previous Japanese epidemiological survey reported a prevalence of 0.12% for PPP, 1 and peripheral arthritis occurred in 4.2% of patients with PPP.Despite the longstanding existence of the concept of pustulotic arthro-osteitis (PAO), 2,3 the medical management within this field has remained ambiguous, partly due to the emergence of SAPHO syndrome and other contributing factors. [4][5][6] To address this issue, a guidance for PAO was published recently in Japan, to advance the diagnosis and management of this condition.As mentioned in the aforementioned guidance, approximately threequarters of the cases are associated with focal infection, 7,8 and smoking is recognized as a potential etiological or exacerbating factor. [9][10][11] The treatment approach encompasses pharmacotherapy, 12 and if focal infection is suspected as the underlying cause, localized treatment is also incorporated.

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