SAPHO syndrome of the temporomandibular joint associated with sudden deafness.
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We report a case of arthritis of the temporomandibular joint (TMJ) associated with sclerosing osteomyelitis of the mandible and temporal bone, causing deafness. The presence of a palmoplantar pustulosis established the diagnosis of SAPHO syndrome. SAPHO (an acronym referring to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis) syndrome is defined by the association of characteristic osteoarticular and dermatologic manifestations, with diffuse sclerosing osteomyelitis of the mandible being a part of this entity. We review the literature of SAPHO syndrome with mandibular manifestations and discuss the mechanisms of inflammatory spread from the TMJ to the cochlea. To our knowledge, this is the first description of skull base involvement in a patient with SAPHO syndrome leading to sudden deafness. (+info)
SAPHO syndrome or psoriatic arthritis? A familial case study.
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OBJECTIVE: To discuss the relationships between SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome and the group of spondylarthropathies. METHODS: Few reports of familial SAPHO have been published. We describe three children, two sisters and one brother, whose clinical and radiological presentation was in accordance with SAPHO syndrome. RESULTS: Two children developed psoriasis, and one child palmoplantar pustulosis. Both sacroiliac and sternoclavicular joints were involved in these three cases. Some features in our observations are also common to psoriatic arthritis. No association was found with HLA antigens, but a history of trauma preceding the onset of symptoms was present in all three children. CONCLUSIONS: We can consider that SAPHO is nosologically related to spondylarthropathies. Psoriatic arthritis could be the missing link between SAPHO and spondylarthropathies. It is likely that both genetic and environmental factors are involved. (+info)
Diagnostic points and possible origin of osteomyelitis in synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome: a radiographic study of 77 mandibular osteomyelitis cases.
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OBJECTIVES: To find diagnostic points and to identify the origin of osteomyelitis in synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome. METHODS: Fifty-two patients with mandibular suppurative osteomyelitis and 25 patients with mandibular osteomyelitis in SAPHO syndrome were included in the study. Radiographic patterns of the lesion, types of periosteal reaction and the presence of external bone resorption and bone enlargement were investigated in each case and compared between the two entities. RESULTS: Suppurative osteomyelitis demonstrated an osteolytic pattern and a lamellated type of periosteal reaction, whereas SAPHO syndrome revealed a mixed-pattern, solid-type periosteal reaction, external bone resorption and bone enlargement. CONCLUSIONS: Radiographic examination is suggested to be convenient and a useful diagnostic method of differentiating osteomyelitis in SAPHO syndrome from suppurative osteomyelitis. The periosteum is suspected to be the original site of osteomyelitic lesions in SAPHO syndrome. (+info)
SAPHO syndrome treated with pamidronate: an open-label study of 10 patients.
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BACKGROUND: In recent years the SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) has been encountered more frequently. However, clinical evidence indicating superiority of a specific therapeutic modality is still absent. Pamidronate, a second-generation bisphosphonate, has a pronounced effect on bone metabolism by suppressing bone resorption. We report our clinical experience with intravenous pamidronate in SAPHO syndrome. METHODS: Between the years 1999 and 2003 we treated 10 patients with the SAPHO syndrome who did not respond to NSAIDs, oral corticosteroids, colchicine, methotrexate, sulphasalazine or infliximab. All patients were treated with 60 mg pamidronate, given intravenously within an hour. In cases of no response a subsequent dose was given within a month and if there was a partial response an additional infusion was given after 4 months. The primary endpoint was the disappearance of recurrent bouts of bone pain, osteitis or hyperostosis, or recurrent synovitis. Reduction of the frequency of attacks by 50% was regarded as a partial response. RESULTS: Seven of the patients were females and three were males. The age at diagnosis ranged from 26 to 68 yr. All patients had axial or peripheral arthritis and cutaneous involvement; three had severe acne, eight had pustulosis and two had concomitant psoriasis vulgaris. Hyperostosis of the anterior chest wall involving either sternocostal or sternoclavicular joints, as seen on technetium 99 bone scintigraphy, was detected in all patients. Complete remission was observed following therapy in six patients, three others partially responded and only one patient had no response. Two patients needed four cycles of pamidronate infusion, one patient needed three, six needed two infusions and one patient remitted following a single pamidronate infusion. In all but one patient pamidronate was effective in preventing recurrent bouts of pustulosis. CONCLUSION: Pamidronate seems to be a very effective mode of therapy for patients with the SAPHO syndrome, by promoting remission in all components of the disorder, such as bone, joint and skin involvement, and ceases the bouts that characterize this disorder. (+info)
Pamidronate in the treatment of childhood SAPHO syndrome.
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BACKGROUND: SAPHO syndrome is increasingly recognized within the paediatric population. Conventional therapeutic approaches have often not been effective. Pamidronate is a second-generation bisphosphonate that affects bone turnover and demonstrates anti-inflammatory properties. In small case series it has given symptomatic relief to adults with this condition. OBJECTIVES: To report the clinical experience with pamidronate in childhood SAPHO syndrome. METHODS: A retrospective observational study of all children with SAPHO syndrome treated with pamidronate between 1996 and 2003 at a tertiary rheumatology centre. The standard dosing regime for pamidronate was 1 mg/kg to a maximum of 30 mg, administered daily for three consecutive days, repeated 3-monthly as required. Response to treatment was determined by clinical observation, patient subjective response and reduction in other treatments RESULTS: Seven girls were treated, with a median (range) age at diagnosis of 11 yr (9-15 yr). All patients demonstrated a beneficial clinical response, with relief of pain, increased activity and improved well-being. Subsequent courses of pamidronate were used in all patients. Other medications including corticosteroids and methotrexate could subsequently be stopped. Transient symptoms were associated with the initial course of pamidronate in some patients. No serious adverse events were reported. CONCLUSIONS: Pamidronate was associated with a marked improvement in function and well-being, and a reduction of pain and use of other medications in all patients, with no significant adverse effects. This study represents preliminary clinical data. A prospective multicentre study is necessary to assess the role and long-term safety of pamidronate in the management of childhood SAPHO syndrome (+info)
Successful treatment of SAPHO syndrome with zoledronic acid.
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The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a chronic, relapsing, inflammatory condition with skin and osteoarticular manifestations. Its etiology remains unclear, and various treatment regimens with steroids and nonsteroidal antiinflammatory drugs frequently fail to control the disease, while exposing patients to the side effects of these drugs. Because the SAPHO syndrome manifests as a destructive inflammatory bone disease, use of bisphosphonates that possess antiosteoclastic and probably antiinflammatory properties has been suggested to be helpful. To our knowledge, this is the first reported case of successful treatment with zoledronic acid of SAPHO syndrome that was resistant to conventional treatment. (+info)
Isolated thoracic spine lesion: is this the presentation of a SAPHO syndrome? A case report.
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A case of an isolated lesion of the thoracic spine attributed to SAPHO syndrome is presented. A 51-year-old man was referred for inflammatory pain in the thoracic spine. The general examination was normal (especially cutaneous and rheumatologic examinations). Laboratory analysis showed only a mild inflammatory reaction. Standard radiographs showed partial condensation of T8. Computed tomography showed osteolysis of the anterior corner of T8, and MRI revealed an abnormal signal of T8, with enlargement of the prevertebral soft tissue. Percutaneous and thoracoscopic biopsies showed a nonspecific inflammatory process, and cultures were sterile. Initially, several diagnoses were advanced: infectious spondylitis, malignant tumor, lymphomas, Paget disease, seronegative spondyloarthropathies and finally atypical SAPHO syndrome. Three months later, the patient experienced more pain. General examination was still normal. The radiological findings worsened, while the inflammatory blood tests were normal. A new thoracoscopic biopsy revealed a nonspecific inflammatory process. A diagnosis of SAPHO syndrome was made, despite the lack of typical lesions. Dramatically improving with anti-inflammatory therapy, the patient's condition was favorable at 3-year follow-up. This atypical presentation of an isolated lesion in the spine makes the diagnosis of a SAPHO syndrome difficult but possible. Spine surgeons must be aware of this rare entity, to avoid misdiagnosis and unnecessary repeated surgical biopsies. (+info)
The usefulness of bone remodelling markers in predicting the efficacy of pamidronate treatment in SAPHO syndrome.
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OBJECTIVES: Pamidronate has recently been used in SAPHO syndrome due to its anti-osteoclastic effect. The aim of this study is to determine the usefulness of bone remodelling markers for determining the efficacy of pamidronate treatment. METHODS: Thirteen patients with SAPHO syndrome were treated with pamidronate. The treatment evaluation was done using a visual analogue scale (VAS) and also erythrocyte sedimentation rate, C-reactive protein, serum crosslaps (sCTX) and osteocalcin initially and after 3 months. A relevant clinical response was defined as an improvement in VAS of at least 40%. RESULTS: At 3 months, 7 of 13 patients had a good clinical response, as previously defined. Five of the seven patients maintained the good response over 6 months. Before the first perfusion 6 of the 13 patients had increased sCTX (upper 3250 pmol/l). In this small cohort we tried to analyse whether the increase in bone remodelling markers was associated with a good clinical response. In the responders group the mean levels of sCTX and osteocalcin at baseline were 6783.17 and 24.66, respectively, and in the non-responders group the levels were 2152 and 11.8, respectively. There was a significant difference in sCTX between the responders and the non-responders (P = 0.0044). CONCLUSION: Infusion of pamidronate is effective in SAPHO in some patients. Increased sCTX might be a prognostic marker for a good clinical response but results have to be confirmed in a larger cohort. (+info)