Sternocostoclavicular Hyperostosis: Positive Clinical and Radiological Response on Pamidronate.

Background: Sternocostoclavicular hyperostosis (SCCH) is a rare disease, constituting a chronic sterile osteomyelitis with elevated bone turnover in the axial skeleton, causing pain and shoulder dysfunction. SCCH severely interferes with daily activities, work, and quality of life. SCCH has a relapse-remitting disease course, but inflammatory-induced sclerotic transformation in the affected area is slowly progressive. Here we present two patients with clinical and radiological diagnosis of SCCH treated with intravenous pamidronate, leading to clinical remission in both, but complete resolution of sclerosis in one of them, which is a novel finding in our experience. Case Presentation: Two adult female SCCH-patients presented with longstanding pain, swelling of the anterior chest wall, and compromised shoulder function. Subsequent single photon emission computed tomography-computed tomography (SPECT/CT) illustrated elevated bone activity and sclerosis in the SC region, with hyperostosis, confirming the diagnosis of SCCH. As symptoms in both patients were eventually refractory to standard painkillers such as non-steroidal anti-inflammatory drugs (NSAIDs), intravenous pamidronate treatment in 3-month cycles was started. Pamidronate was effective in reducing pain and improving shoulder function and also led to decreased bone turnover on skeletal scintigraphy. Sclerosis in the first patient persisted. In the second patient, however, a complete resolution of sclerosis was observed. Conclusions: SCCH remains a rare bone disorder for which no evidence-based therapies are yet available. While disease burden is high, SCCH lacks recognition and is often diagnosed long after symptomatic presentation. As for the cases in this report, pamidronate was successful in reducing symptoms, and in the second case even led to regression of sclerotic changes on CT-imaging.

Treatment-Refractory Sternocostoclavicular Hyperostosis.

Sternocostoclavicular hyperostosis (SCCH) is an infrequent chronic inflammatory disorder of the axial skeleton of unknown origin. SCCH goes often unrecognized due to a low level of awareness for the disorder. It typically presents with relapsing and remitting pain in the shoulder, neck, and anterior chest wall area with occasional swelling and tenderness of the sternoclavicular area. The diagnosis is confirmed radiologically by sclerosis and hyperostosis of the sternoclavicular joints. There have been several reports in which intravenous bisphosphonates and tumor necrosis factor-inhibitors have shown reasonable efficacy in the treatment of this disorder. We report a patient with a long history of SCCH in whom pamidronate 60 mg intravenously every 3 months for 3 years failed to reduce symptom severity and improve radiologic findings.

[Case of SAPHO syndrome with significant sternocostoclavicular hyperostosis].

A 59-year-old man with hypertension developed general fatigue, fever, and precordialgia in early March 2007. An antibacterial agent was intravenously administered; however, no improvement in his symptoms or laboratory findings was observed. He had acne and pustulosis, and radiographs and CT of the chest revealed sternocostoclavicular hyperostosis. As a result, the diagnosis was revised to SAPHO syndrome, and he improved with steroid administration. SAPHO syndrome is a condition that is rarely found at the initial examination in the Department of Internal Medicine. We report a case with a discussion of the literature.

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.

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.

[Sternocostoclavicular hyperostosis--case reports and differential diagnosis]. / Sternocostoclaviculäre Hyperostose--Kasuistik und Differentialdiagnose.

Sternocostoclavicular hyperostosis (SCCH) is a disorder related to collagenoses. SCCH is typically characterised by the triad of swelling of the sternoclavicular and/or sternocostal joints of the first or second rib, palmar and plantar pustulosis (PPP), only limited elevation of inflammation indicating laboratory parameters. Till today the etiology is unclear and no causal therapy is known. Symptomatic treatment consists of administration of NSAID and cortisone, irradiation and even limited resection of the clavicle and rib.

[Sternocostoclavicular hyperostosis]. / Sternokostoklavikulaer hyperostose.

The syndrome of synovitis, acne, pustulosis, hyperostosis and osteomyelitis (SAPHO syndrome) includes disorders with common clinical and radiological manifestations. One of these, sternocostoclavicular hyperostosis, is characterized by inflammation and ossification of ligaments, bones and joints in the upper anterior parts of the trunk. The author reports the case of a 20 year-old female who developed unilateral sternocostoclavicular hyperostosis and complete ankylosis of the second, third and fourth lumbar vertebrae. Prior to onset of musculoskeletal complaints, she experienced a brief episode of dermatitis of the scalp, accompanied by transient alopecia. The condition was controlled by administration of ketoprofen and naproxen.

Sternocostoclavicular hyperostosis. Presentation and long-term follow-up of three cases.

Sternocostoclavicular hyperostosis is a rare disease characterized by recurrent pain and skeletal swelling in the upper part of the chest. The clinical manifestations are closely linked to pustulosis palmo-plantaris but the etiology is still obscure. We present three cases of sternocostoclavicular hyperostosis with a follow-up period of 9-22 years at our department.

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.

[Sternocostoclavicular hyperostosis]. / Hyperostosis sternocostoclavicularis.

On the basis of 4 patients from our own records we describe the rare syndrome of "Hyperostosis sternocostoclavicularis". The etiology of this disorder is still unclarified. In addition to a swelling that is generally only slightly tender to pressure, the most important symptom is usually a long-standing, intermittent, dull pain in the upper sternum, the claviculae and the adjacent uppermost ribs. Laboratory diagnosis usually shows an accelerated blood sedimentation rate, but other laboratory findings are unremarkable and rheumatological serology gives a negative result. The changes detected by radiography are described, as are the histological findings. Primary or secondary malignant osteomas are also to be taken into account in differential diagnosis. For therapy most authors recommend medicinal treatment with non-steroidal antiphlogistic agents.