Background/Purpose: SAPHO syndrome is a disorder characterized by pustular skin lesions and osteoarticular lesions, which was proposed by Chamot et al. in 1987. Clinical studies based on the diagnostic criteria of SAPHO syndrome are mainly reported from Europe, and still limited in East-Asia.

Methods: We investigated the clinical features and treatment results in 31 Japanese patients with SAPHO syndrome (male 10, female 21) diagnosed and treated between 2003 and 2011. HLA-A and -B typing was performed in 30 patients, and their allele frequencies were compared with those in the healthy Japanese controls, using Fisher’s exact test.

Results: The age at onset was ranged between 16 and 68 years old (y.o.) (average: 48.3), and the age at diagnosis from 16 to 74 y.o. (average: 53.8). The average follow-up period was 42 months. Sternocostoclavicular hyperostosis was the main manifestation and recognized in 29 cases (94%). Pustular dermatitis including palmoplantar pustulosis  was seen in 26 cases (84%). As other manifestations, recurrent oral ulceration was seen in 6 cases (19%), and inflammatory bowel disease in 2 cases. Most patients had intermittent attacks of pain, therefore oral NSAIDs were needed in all cases and oral prednisolone (PSL) in 14 cases (45%). The oral NSAIDs and/or PSLwere effective for temporary pain relief. DMARDs (SSZ and/or MTX) were used in 14 cases (45%) with recurrent chronic pain. Pain relief more than 50% was seen in only 4 cases (29%) out of DMARDs users. In two refractory cases with severe spondylitis, adalimumab (ADA) was tried. Both cases showed immediate pain-relief and ADA was effective during at least one year. HLA tests revealed that the allele frequencies of HLA-B27 and HLA-B51were respectively 0% and 12%, which were similar with those in healthy Japanese controls. On the other hand, the frequency of HLA-B61 was 27 % and significantly higher than that (12%) in healthy controls.

Conclusion: Mucosal lesions seem to be a rather frequent complication of SAPHO syndrome in our study. The efficacy of DMARDs (SSZ and/or MTX) was observed in a small number of patients. ADA was effective in two refractory cases with severe spondylitis. This study revealed that HLA-B61 was significantly increased in Japanese patients with SAPHO syndrome.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-and-treatment-results-of-japanese-patients-with-sapho-synovitis-acne-pustulosis-hyperostosis-osteitis-syndrome/