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2021 Dec 30

Mod Rheumatol

SAPHO Syndrome and Pustulotic Arthro-Osteitis (PAO).


Kishimoto M, Taniguchi Y, Tsuji S, Ishihara Y, Deshpande GA, Maeda K, Okada M, Komagata Y, Kobayashi S, Okubo Y, Tomita T, Kaname S
Mod Rheumatol. 2021 Dec 30.
PMID: 34967407.


Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) syndrome is a rare inflammatory osteo-articular disorder, which encompassed many diseases, including pustulotic arthro-osteitis (PAO). Bone and joint manifestations, including osteitis, synovitis and hyperostosis, are the hallmark of the SAPHO syndrome and affect a variety of regions of the body. Recent GRAPPA survey indicated that more than 80 percent of cases of SAPHO syndrome in Japan were thought to be PAO, originally proposed by Sonozaki et al. in 1981, whereas severe acne was the most commonly reported skin ailment amongst participants with SAPHO syndrome in Israel. SAPHO syndrome is a rare disease and adequate data regarding its prevalence remains unavailable, whereas prevalence of PPP was reported to be 0.12 % in Japan and 10-30% of patients with PPP had PAO. SAPHO syndrome and PAO are predominantly found in patients in the third through fifth decades of life, and a female predominance are seen in both groups. The diagnosis of SAPHO syndrome/PAO is typically made by a rheumatologist or dermatologist. Identification of a variety of the clinical, radiological, and laboratory features outlined, as well as diagnostic criteria, are used to make the diagnosis. Goals for treating patients with SAPHO syndrome/PAO seek to maximize health-related quality of life by improving skin and articular symptoms, preventing structural changes and destruction, and normalizing physical function and social participation. Finally, we review the non-pharmacological (ie: smoking cessation and controlling focal infections) and pharmacological managements including NSAIDs, bisphosphonates, cs DMARDs, bDMARDs, and other treatments for SAPHO syndrome/PAO.