1/7. In SAPHO syndrome anti-TNF-alpha therapy may induce persistent amelioration of osteoarticular complaints, but may exacerbate cutaneous manifestations.OBJECTIVES: SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a rare disease combining skin, bone and joint manifestations. In recent years new therapeutic strategies have been tried, among them TNF-alpha-blocking agents. We report our experience with infliximab in four cases of SAPHO syndrome refractory to conventional therapies. methods: Between 2002 and 2005, four cases of SAPHO syndrome (two females and two males; mean age 49.7 yr) responding poorly to conventional drugs were treated with infliximab. The dose was 5 mg/kg, according to the protocol used in spondyloarthropathies, with infusions at 0, 2 and 6 weeks followed by 6 weeks intervals. No active cutaneous manifestations were present at the time of starting therapy. RESULTS: Complete remission of osteoarticular involvement was achieved after the second or third infusion, and the positive response was maintained for up to 12 months. A patient relapsed after discontinuation of infliximab, because of infectious complication. Palmoplantaris pustulosis relapsed in two patients after three and six infusions, respectively; there was slight improvement after discontinuation of anti-TNF-alpha drugs. CONCLUSIONS: Infliximab seems to be a very effective therapy for osteoarticular complaints of SAPHO syndrome. Cutaneous involvement responded less favourably, palmoplantaris pustulosis relapse being a possible complication.- - - - - - - - - - ranking = 1keywords = synovitis (Clic here for more details about this article) |
2/7. The SAPHO syndrome.We report two cases of the SAPHO syndrome (synovitis, acne conglobata, pustulosis palmoplantaris, hyperostosis and osteitis). This syndrome has been published in the pediatric and rheumatological literature in recent years.- - - - - - - - - - ranking = 1keywords = synovitis (Clic here for more details about this article) |
3/7. SAPHO syndrome.Four women with pustulosis palmoplantaris (PPP) and associated sternocostoclavicular hyperostosis are described. The connection between the two diseases is not coincidental: it is encountered in 9.4% of PPP patients, and was reported in the French literature as part of the SAPHO (synovitis acne pustulosis hyperostosis osteomyelitis) syndrome. This syndrome is linked to the spondyloarthropathies, having an increased prevalence of HLA B27 and occurrence of sacroiliitis. None of our patients had HLA B27, but HLA A26 was found in the 3 patients examined. No conclusion can be drawn from this finding, however, since this antigen is found in 21.7% of Ashkenazi jews. Recognition of this syndrome by dermatologists will improve diagnosis and treatment.- - - - - - - - - - ranking = 1keywords = synovitis (Clic here for more details about this article) |
4/7. Arthro-osteitis--a clinical spectrum.Arthro-osteitis is an uncommon condition which can be associated with palmoplantar pustulosis. It forms part of a group of conditions which include the synovitis, acne, pustulosis, hyperostosis, osteitis syndrome (SAPHO) and sternocostoclavicular hyperostosis. We report four cases illustrating the clinical spectrum of this condition which occurred in the absence of concomitant skin lesions. One patient had extensive aortic calcification a feature not previously reported in this condition, which may represent a low grade inflammatory aortitis.- - - - - - - - - - ranking = 1keywords = synovitis (Clic here for more details about this article) |
5/7. propionibacterium acnes isolated from sternal osteitis in a patient with SAPHO syndrome.A woman presented with palmar pustulosis and deep chest pain in association with osteitic lesions in the lower part of the sternum. propionibacterium acnes was isolated and grew in pure culture from 6 surgically obtained bone specimens. The patient received clindamycin treatment for 6 months. synovitis in both her wrists persisted and, based on a clinical suspicion of seronegative rheumatoid arthritis, she was treated with intramuscular gold and methotrexate with no apparent benefit. Subsequently, she was diagnosed with SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteomyelitis). Our patient provides further data on the potential association between P. acnes and SAPHO syndrome.- - - - - - - - - - ranking = 1keywords = synovitis (Clic here for more details about this article) |
6/7. Osseous manifestations of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.The SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a complex group of disorders characterized by peculiar bone lesions, most commonly involving the anterior chest wall, and sometimes accompanied by dermatologic manifestations. The clinical and radiographic features of this syndrome are well described, but few studies have examined the histologic features of the bone lesions. We describe the clinical, radiographic, and histologic features of the osseous lesions encountered in eight patients with a clinical diagnosis of SAPHO syndrome. The patients included five female and three male patients ranging in age from 5 to 63 years (mean, 35.3 years). The most common clinical presentation was pain related to the sites of osseous involvement. Two patients also had some form of pustular dermatosis. The radiographic features of the osseous lesions varied but often suggested the possibility of a neoplasm. Nine pathologic specimens were available for review, five from the clavicle, two from the first rib, one from the calcaneus/cuboid, and one from the tibia. The histologic features varied but seemed related to the duration of the patients' musculoskeletal symptoms. Early lesions contained acute inflammation, edema, and prominent periosteal bone formation, histologically indistinguishable from ordinary bacterial osteomyelitis, whereas late lesions demonstrated markedly sclerotic bone trabeculae with prominent marrow fibrosis and only mild chronic inflammation; one of these biopsies appeared virtually identical to Paget's disease. One biopsy was performed after an intermediate duration of symptoms and contained prominent chronic inflammation only. The histologic findings in SAPHO are variable and nonspecific and may depend on the duration of disease, but it is important to recognize the spectrum of histologic changes possible in the syndrome and to realize that clinicopathologic correlation is necessary to avoid misdiagnosis and unnecessary long-term antibiotic therapy.- - - - - - - - - - ranking = 5keywords = synovitis (Clic here for more details about this article) |
7/7. Case report. synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome.A case of the SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome in a 35-year-old woman is presented. Ignorance of this entity on the part of the physicians treating the patient may have contributed to her having repeated diagnostic procedures and treatment, some of which may have been unnecessary. dentists are encouraged to suspect the SAPHO syndrome when they encounter a patient with mandibular osteomyelitis together with symptoms involving other bones and skin lesions such as pustulosis or psoriasis.- - - - - - - - - - ranking = 5keywords = synovitis (Clic here for more details about this article) |