The sternoclavicular syndrome: experience from a district general hospital and results of a national postal survey.
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OBJECTIVE: To report our local experience of the sternoclavicular syndrome and sample the experience of other rheumatologists in the UK. METHODS: We studied case records of 23 patients referred to the Southend rheumatology clinic and data obtained from a postal questionnaire survey of British rheumatologists. RESULTS: We describe 58 cases (20 males and 38 females, mean age 47.2 yr). The disease was unilateral in 40 patients. Shoulder and/or arm pain (38 cases) with limitation of shoulder movements was an important presenting feature; other presenting features were anterior chest wall pain (14 cases) and neck pain (15 cases). Peripheral joint involvement was seen in 12 cases. Skin rash was reported in 12 cases (psoriasis, 6; acne, 2; none had pustulosis). No patients had symptoms or signs of sacroiliitis, and HLA-B27 was negative in 22 out of 23 patients. 99Technetium scintiscanning showed increased uptake in the sternoclavicular region in 31/34 patients (91.1%), but not in the sacroiliac areas. Plain radiographs were abnormal in 18 cases (sclerosis, 9; erosions, 2; soft tissue swelling, 2; bony expansion, 5). CT and/or MRI scans (available in 27 cases) showed erosions in 12 and osteitis in 18. Available histology showed a variable picture, including inflammation, bone erosion, sterile osteomyelitis and fibrosis. The majority of patients (45) were treated with non-steroidal anti-inflammatory drugs: 12 received steroids and 10 received disease-modifying anti-rheumatic drugs (methotrexate, 4; sulphasalazine, 6). Follow-up information was available for 38 patients, of whom 14 became asymptomatic and 24 had chronic disease with intermittent flares. CONCLUSION: Sternoclavicular disease is not uncommon in the UK. It can present with pain in the shoulder, neck or anterior chest wall, and may be underdiagnosed. Our results do not show a link with acne or pustulosis. Features of spondyloarthropathies, such as sacroiliitis and HLA-B27 positivity, were rare in this survey. (+info)
Sternocostoclavicular hyperostosis: its progression and radiological features. A study of 12 cases.
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Twelve cases of sternocostoclavicular hyperostosis were followed up over four to 16 years. The patients underwent repeated radiological examinations of the sternocostoclavicular joints and the sternum, and the extrasternal osseous manifestations of the disease were studied to show changes in the radiological features during long term follow up. Five of 12 (41%) patients had extrasternal manifestations. With the exception of one patient extrasternal manifestations were first detected by scintigraphs because they were asymptomatic. With respect to the sternal manifestations the initial radiological diagnosis was made during an acute phase while painful swelling over the sternum and decreased mobility of the shoulders occurred. The radiological examinations showed the signs of a proliferative destructive arthritis in most patients. In contrast with the frequent occurrences of clinical symptoms, the radiological signs of progression take several years to become detectable. There are no specific bacteriological, serological or histological findings. Usually a permanent increase in the erythrocyte sedimentation rates is found. Sternocostoclavicular hyperostosis is a slowly progressing disease, characterised by a chronic aseptic destructive sternoclavicular arthritis with a reactive low turnover sclerosis that begins in a similar way to an enthesopathy and ends after several decades with total ankylosis. The radiological identification of retrosternal proliferation of soft tissue by computed tomography was found to be a valuable criterion for the differential diagnosis from other benign hyperostotic processes of the sternoclavicular region. (+info)
In SAPHO syndrome anti-TNF-alpha therapy may induce persistent amelioration of osteoarticular complaints, but may exacerbate cutaneous manifestations.
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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. (+info)
Calcitonin treatment for intersternocostoclavicular ossification: clinical experience in two cases.
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Intersternocostoclavicular ossification is a benign arthro-osteitis of the upper anterior chest of unknown cause. Two patients with acute exacerbation of this disorder were successfully treated with intramuscular injections of an eel calcitonin analogue (40 units three times a week). Besides symptomatic relief of local pain and swelling, serial scintigrams showed quantitative improvement in radiophosphonate uptake. The rapid alleviation of pain implies that the hormone has a central analgesic effect, in addition to its direct influence on bone cells and antiinflammatory action. In one patient the disease was associated with palmoplantar pustulosis, which was cured with oral colchicine, whereas the other patient did not have such skin lesions. Despite a hypothetical link between palmoplantar pustulosis and intersternocostoclavicular ossification, colchicine had no beneficial impact on the bone pain. Salmon calcitonin delivered by nasal spray was tried for the second patient but failed, probably because of insufficient drug delivery. The initial favourable results described here warrant future use of calcitonin injection on a larger number of patients with intersternocostoclavicular ossification. (+info)
SAPHO syndrome: misdiagnosed and operated.
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SAPHO is a rare disorder that results in synovitis, acne, pustulosis, hyperostosis and osteitis. Patients with this syndrome typically present with musculoskeletal complaints, frequently localized to the anterior chest wall. However, diagnosis can be difficult in case of involvement of only one symptomatic bone without skin lesions. Awareness of SAPHO syndrome is necessary for accurate diagnosis and to prevent inappropriate and unnecessary treatment. (+info)
Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a single center study of a cohort of 71 subjects.
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The psychological burden of an initially unexplained illness: patients with sternocostoclavicular hyperostosis before and after delayed diagnosis.
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Sternocostoclavicular hyperostosis presenting with thoracic sinus formation.
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Sternocostoclavicular hyperostosis (SCCH) is a condition which is well described in the Japanese literature but is rare in Western Europe. It is characterised by pain and swelling in the upper anterior part of the chest, which tends to be progressive. A patient is described with bilateral chronic discharging sinuses over the anterior ends of the clavicles in whom the diagnosis appeared to be one of SCCH. (+info)