SAPHO syndrome: A review

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 literat...

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description 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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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.</description><identifier>ISSN: 1863-2521</identifier><identifier>EISSN: 1863-2548</identifier><identifier>DOI: 10.1007/s11832-014-0627-7</identifier><identifier>PMID: 25585872</identifier><language>eng</language><publisher>London, England: SAGE Publications</publisher><subject>Arthritis ; Biopsy ; Cytokines ; Disease ; Etiology ; Hypotheses ; Immune system ; Medical research ; Medicine ; Medicine &amp; Public Health ; Mutation ; Original Clinical ; Original Clinical Article ; Orthopedics ; Pathogenesis ; Patients ; Pediatrics ; Traumatic Surgery</subject><ispartof>Journal of children's orthopaedics, 2015-02, Vol.9 (1), p.19-27</ispartof><rights>2015 European Pediatric Orthopaedic Society (EPOS), unless otherwise noted. Manuscript content on this site is licensed under Creative Commons Licenses.</rights><rights>The Author(s) 2015</rights><rights>2015. This work is published under https://creativecommons.org/licenses/by-nc/4.0/ (the “License”). 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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. 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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.</abstract><cop>London, England</cop><pub>SAGE Publications</pub><pmid>25585872</pmid><doi>10.1007/s11832-014-0627-7</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record>
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subjects Arthritis
Biopsy
Cytokines
Disease
Etiology
Hypotheses
Immune system
Medical research
Medicine
Medicine & Public Health
Mutation
Original Clinical
Original Clinical Article
Orthopedics
Pathogenesis
Patients
Pediatrics
Traumatic Surgery
title SAPHO syndrome: A review
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