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. 2015 Feb;9(1):19-27.
doi: 10.1007/s11832-014-0627-7. Epub 2015 Jan 14.

SAPHO syndrome: a review

Iva Rukavina  1
Affiliations

SAPHO syndrome: a review

Iva Rukavina. J Child Orthop. 2015 Feb.

Abstract

Introduction: Synovitis-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.

Aims: The 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.

Results: The 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.

Conclusion: It 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.

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Figures

Fig. 1
Fig. 1
Bilateral sternoclavicular joint edema in the SAPHO patient
Fig. 2
Fig. 2
Percentage distribution of arthritis in the body (SAPHO/CRMO)
Fig. 3
Fig. 3
Scintigraphy findings show intensive uptake of the radiopharmaceutical technetium-99m at the sternoclavicular joints and sternum, which represent a “bull’s head“ sign
See this image and copyright information in PMC

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