Sternocostoclavicular Hyperostosis: Positive Clinical and Radiological Response on Pamidronate

Abstract

BackgroundSternocostoclavicular 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 PresentationTwo 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.ConclusionsSCCH 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.

Diagnostic and therapeutic practices in adult chronic nonbacterial osteomyelitis (CNO)

“…Why MRI also appears preferred in adult CNO cannot be fully deduced from our data, but it may be favoured owing to its ability to track inflammation more adequately than CT and bone scintigraphy. Typical findings on MRI – like bone marrow oedema - may resolve after treatment [ 19 ], while the consequences of inflammation detected by CT (sclerosis, hyperostosis) usually accumulate over time and are non-reversible (although one case was published showing full resolution of these changes after treatment [ 20 ]). Similarly, bone scintigraphy continues to demonstrate increased bone lesion isotope uptake despite clinical improvement, indicating an “imprinting pattern” [ 21 ].…”

Treatment and monitoring of SAPHO syndrome: a systematic review

“… 80–82 Similar arguments have been made around SCCH being a SAPHO subset and part of the CNO spectrum, and two cases have been reported with success in pain and functional improvement. 83 …”

Different consequences of the treatment of osteoarthritis in gastrointestinal comorbidity with exocrine pancreatic insufficiency

“…Many studies have been conducted to identify the effect of NSAIDs on the development of cardiovascular pathology in patients with OA [11,12]. A study was also carried out on the effect of different combinations of disease-modifying drugs and NSAIDs on the course of OA, reducing pain, and improving the quality of life of patients [13][14][15]. The influence of different NSAIDs on the level of pain syndrome in OA and on the condition of the cartilage was also researched and compared.…”