SAPHO syndrome: A review

Abstract

IntroductionSynovitis–acne–pustulosis–hyperostosis–osteitis (SAPHO) is an acronym for various osteoarticular and dermatological manifestations that can appear in the same patient. It is a rare syndrome, but since its awareness has increased, there have been more and more such reports in the literature.AimsThe objectives of this review are to summarize the current state of knowledge on pediatric and adult-onset SAPHO syndrome, and to discuss treatment strategies that should be considered.ResultsThe SAPHO syndrome can affect patients of any age, and its etiology is still not known. The syndrome has its cognizable radiological characteristics that are most important in making the diagnosis. There are several diagnostic criteria as well, but they need further validation. No standard treatment protocols are available and current treatment options are not evidenced-based due to the rarity of the syndrome. Therapy is empirical and aimed at easing pain and modifying the inflammatory process. It includes nonsteroidal anti-inflammatory drugs (NSAIDs) as the first-line agents. Antibiotics, corticosteroids, disease-modifying anti-rheumatic drugs, biologicals targeting tumor necrosis factor alpha or interleukin-1, and bisphosphonates have all been used with variable success. Surgery is reserved to treat complications. Even though it is a disease with good long-term prognosis, its treatment remains a challenge and the results are known to be disappointing, especially with the skin component of the disease.ConclusionIt is expected that these patients present at the time of diagnosis and the treatment should be as early, effective, and safe as possible in order to prevent osteoarticular progression and to limit the adverse events associated with pharmacological drugs.

An Okhotsk adult female human skeleton (11th/12th century AD) with possible SAPHO syndrome from Hamanaka 2 site, Rebun Island, northern Japan

“…In addition, HLA-B27 antigen may increase the risk for SAPHO syndrome in the European population. Therefore, it is likely that an autoimmune reaction combined with some genetic predisposition and infectious agents leads to the onset of SAPHO syndrome (Rukavina, 2015).…”

SAPHO syndrome in which certolizumab pegol was effective: A case report

“…In addition, IgA upregulation has been previously reported in a cohort of 29 SAPHO patients [4,5] . In our report, IgA was also upregulated; hence, it could be useful for the definitive diagnosis of SAPHO syndrome.…”

“…The first-line treatment of SAPHO syn drome involves the use of NSAIDs [6] . Other treat ment options are colchicine, glucocorticoid, bis phosphonates, and disease-modifying agents such as methotrexate, sulfasalazine, and anti-TNFalpha therapy [4] . The effectiveness of infliximab [7] , etanercept [8] , and adalimumab [9] have been previously reported for SAPHO syndrome.…”

SAPHO Syndrome: A Chameleon in Medical Diseases

“…Our patient experienced the classic PPP, however, only in the later stages of her disease. Her pustular rash was 3 years after the onset of her leg pain, as comparison to other SAPHO cases where approximately 70% patients will exhibit skin manifestations within 2 years from the time of onset [4]. Because of the rarity of SAPHO syndrome, no randomized controlled trials have been conducted on the effectiveness of medical therapy.…”

“…SAPHO is a rare condition with a prevalence of 1 in 10,000, affecting predominantly adults between ages of 30 -50 years old, though as young as 15 months has been observed [4]. In the original French SAPHO cases, the gender distribution was approximately equal; however, later studies deemed female preponderance to be the majority [5,6].…”