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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

Scintigraphy findings show intensive uptake of the radiopharmaceutical technetium-99m at the sternoclavicular joints and sternum, which represent a “bull’s head“ sign
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Fig3: Scintigraphy findings show intensive uptake of the radiopharmaceutical technetium-99m at the sternoclavicular joints and sternum, which represent a “bull’s head“ sign

Mentions: Radiographs may show expanded bone, sclerosis and osteolysis, periosteal reaction, or enthesopathic new bone formation. Bone scintigraphy delineates increased uptake in affected bone and may reveal asymptomatic disease or abnormalities not apparent on radiographs. The advantage of scintigraphy is the demonstration of multiple sites of involvement, so it is helpful for the elimination of malignancy or infection. Symmetric uptake in the sternoclavicular region with a typical “bull‘s head” appearance shown in bone scintigraphy is characteristic of the SAPHO syndrome (Fig. 3) [46]. It was first described by Freyschmidt and Sternberg [47] but, even though it is considered to be pathognomonic, it is not a very sensitive indicator of SAPHO.Fig. 3


SAPHO syndrome: a review.

Rukavina I - J Child Orthop (2015)

Scintigraphy findings show intensive uptake of the radiopharmaceutical technetium-99m at the sternoclavicular joints and sternum, which represent a “bull’s head“ sign
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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

Fig3: Scintigraphy findings show intensive uptake of the radiopharmaceutical technetium-99m at the sternoclavicular joints and sternum, which represent a “bull’s head“ sign
Mentions: Radiographs may show expanded bone, sclerosis and osteolysis, periosteal reaction, or enthesopathic new bone formation. Bone scintigraphy delineates increased uptake in affected bone and may reveal asymptomatic disease or abnormalities not apparent on radiographs. The advantage of scintigraphy is the demonstration of multiple sites of involvement, so it is helpful for the elimination of malignancy or infection. Symmetric uptake in the sternoclavicular region with a typical “bull‘s head” appearance shown in bone scintigraphy is characteristic of the SAPHO syndrome (Fig. 3) [46]. It was first described by Freyschmidt and Sternberg [47] but, even though it is considered to be pathognomonic, it is not a very sensitive indicator of SAPHO.Fig. 3

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