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SAPHO syndrome: a review.

Rukavina I - J Child Orthop (2015)

Bottom Line: 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.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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatrics, University Hospital Centre Zagreb, Kišpatićeva 12, 10000, Zagreb, Croatia, iva_rukavina@hotmail.com.

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.

No MeSH data available.


Related in: MedlinePlus

Bilateral sternoclavicular joint edema in the SAPHO patient
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Related In: Results  -  Collection


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Fig1: Bilateral sternoclavicular joint edema in the SAPHO patient

Mentions: Osteoarticular manifestations involve osteitis, hyperostosis, synovitis, arthropathy, and enthesopathy that present with pain, tenderness, and sometimes swelling over the affected areas and fever. Osteitis is the inflammation of bone, which may involve the cortex and the medullary cavity. Hyperostosis reflects excessive bone growth and may result in enthesopathic new bone formation and joint fusion (Fig. 1). Synovitis mostly manifests as nonerosive oligoarthritis of larger joints. Joint involvement can be primary arthritis or an extension of the osteitis adjacent to the articular structures. Arthritis has been reported in up to 92.5 % of SAPHO cases. The axial skeleton is involved in 91 % and the peripheral joints in 36 % of cases. Besides sternocostal and sternoclavicular joints, which are the most commonly affected, it mainly affects the sacroiliac or hip joints, knees, and ankles. For anterior chest wall disease, three stages have been described (Table 1). The costoclavicular ligament is involved in 48 % of cases, and it is considered a decisive early finding in SAPHO [7, 32, 33].Fig. 1


SAPHO syndrome: a review.

Rukavina I - J Child Orthop (2015)

Bilateral sternoclavicular joint edema in the SAPHO patient
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4340847&req=5

Fig1: Bilateral sternoclavicular joint edema in the SAPHO patient
Mentions: Osteoarticular manifestations involve osteitis, hyperostosis, synovitis, arthropathy, and enthesopathy that present with pain, tenderness, and sometimes swelling over the affected areas and fever. Osteitis is the inflammation of bone, which may involve the cortex and the medullary cavity. Hyperostosis reflects excessive bone growth and may result in enthesopathic new bone formation and joint fusion (Fig. 1). Synovitis mostly manifests as nonerosive oligoarthritis of larger joints. Joint involvement can be primary arthritis or an extension of the osteitis adjacent to the articular structures. Arthritis has been reported in up to 92.5 % of SAPHO cases. The axial skeleton is involved in 91 % and the peripheral joints in 36 % of cases. Besides sternocostal and sternoclavicular joints, which are the most commonly affected, it mainly affects the sacroiliac or hip joints, knees, and ankles. For anterior chest wall disease, three stages have been described (Table 1). The costoclavicular ligament is involved in 48 % of cases, and it is considered a decisive early finding in SAPHO [7, 32, 33].Fig. 1

Bottom Line: 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.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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Paediatrics, University Hospital Centre Zagreb, Kišpatićeva 12, 10000, Zagreb, Croatia, iva_rukavina@hotmail.com.

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.

No MeSH data available.


Related in: MedlinePlus