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1/13. The sternoclavicular syndrome: experience from a district general hospital and results of a national postal survey.

    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.
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2/13. Rapid pain relief and remission of sternocostoclavicular hyperostosis after intravenous ibandronate therapy.

    Sternocostoclavicular hyperostosis (SCCH) is an infrequent but painful, localized disturbance of bone metabolism of unknown etiology. The diagnosis of SCCH is generally one of exclusion, and it is therefore frequently missed or delayed, leaving patients with pain that frequently fails to respond to standard analgesic therapy. Consequently, SCCH leads to significantly impaired quality of life. Characteristic increased localized bone turnover and inflammatory osteitis provide a strong rationale for using intravenous bisphosphonates to treat the condition. We report on three patients with long-standing, treatment-refractory SCCH in whom intravenous ibandronate injections (a single administration of 4 mg followed by 2 mg every 3 months for up to a year) produced prompt, dramatic, persistent pain relief and resolution of the other symptoms of the disease. We also review recent evidence suggesting that SCCH is more common than generally believed and that technetium-99 bone scanning can aid in making an accurate diagnosis.
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3/13. 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.
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4/13. calcitonin treatment for intersternocostoclavicular ossification: clinical experience in two cases.

    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.
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5/13. MR appearance of sternal hyperostosis.

    Sternal hyperostosis is characterized by prominent new bone formation and fibrosis with less pronounced areas of granulation tissue and round cell infiltration. In previously reported cases the process involved not only the sternum but adjacent bony areas as well. Depending upon the extent of disease, acquiring biopsy material for histologic analysis can be difficult. We report a case of sternal hyperostosis involving the sternum exclusively and extensively. magnetic resonance imaging was useful in directing biopsy for optimal histologic yield. This unusual case of sternal hyperostosis is believed to be the result of an inflammatory process.
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6/13. Sternocostoclavicular hyperostosis or pustulotic arthroosteitis.

    We describe a 68-year-old woman who had suffered pain, swelling, heat, and redness in the region of both clavicles for the last 2 years. Her erythrocyte sedimentation rate was markedly elevated; tests for rheumatoid factor were negative. At surgical exploration, ankylosis of the sternoclavicular joints, especially on the left side, was found. biopsy revealed chronic nonspecific inflammation with new bone formation, consistent with the diagnosis of sternocostoclavicular hyperostosis or pustulotic arthroosteitis.
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7/13. Sternocostoclavicular hyperostosis (SCCH) presenting as a mass in the neck.

    We report three cases of sternocostoclavicular hyperostosis (SCCH), all of which presented as a solitary neck mass. This hypertrophic bone disorder has been well described in the Japanese literature, but is rarely encountered in north america. We discuss the clinical and pathological features of this disease, and review the world literature in order to bring this entity to the attention of the otolaryngologist, to whom it may present as a neck mass.
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8/13. Chronic recurrent multifocal osteomyelitis and psoriasis--a report of a new association and review of related disorders.

    In summary, we have described two patients with CRMO and psoriasis, and have reviewed the musculoskeletal manifestations associated with pustular eruptions of the palms and soles. In view of the frequent occurrence of PPP in patients with CRMO, we suggest that the occurrence of psoriasis in our two patients is more than coincidence, and that noninfectious, inflammatory lesions of bone may be another musculoskeletal manifestation of psoriasis. This rare association, as well as the association of PPP with disorders associated with new bone formation, may shed new insights on the relatively common finding of periosteal elevation associated with psoriatic arthritis and the occasional severe juxta-articular osteolytic destructive bone lesions seen in psoriatic arthritis.
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9/13. Computed tomography in sternocostoclavicular hyperostosis.

    Sternocostoclavicular hyperostosis is a rare but recognized entity characterized by new bone formation and ligamentous ossification which limits mobility of the sternoclavicular joints. Aetiology is obscure and debated. There may be considerable delay in establishing the diagnosis, even by specialists. In confirming the diagnosis plain radiography is of limited value but radionuclide bone scan and computed tomography (CT) may be helpful. Four cases are described to emphasize these points and illustrate the clinical and radiological features of the condition. High resolution CT with thin sections (5 mm or less) is the examination of choice for demonstrating the characteristic features and is essential if the condition is to be confirmed in its early stages when treatment might be more successful in delaying progression.
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10/13. Sternocostoclavicular hyperostosis.

    Sternocostoclavicular hyperostosis is a chronic arthro-osteitis affecting mostly juxtasternal structures. It is characterized clinically by painful swelling of the upper anterior chest wall and occasionally is associated with pustulosis palmaris and plantaris. Pathologically there is osteitis, periostitis, and new bone formation of periarticular structures. Two patients are described, and the clinical, laboratory, and radiological findings of 251 reported cases in the English literature are reviewed.
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