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.

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.

[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]. / 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.