Center for Bone Quality, Division of Endocrinology, Department of Internal Medicine, Leiden University Medical Center, Leiden, Netherlands.
Center for Bone Quality, Department of Radiology, Leiden University Medical Center, Leiden, Netherlands.
Front Endocrinol (Lausanne). 2021 Feb 18;12:621604. doi: 10.3389/fendo.2021.621604. eCollection 2021.
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.
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.
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.
胸骨锁骨骨肥厚症（SCCH）是一种罕见疾病，构成了一种慢性无菌性骨髓炎，伴轴向骨骼中骨转换升高，导致疼痛和肩部功能障碍。SCCH 严重干扰了日常活动、工作和生活质量。SCCH 具有复发性缓解疾病过程，但受累区域炎症诱导的硬化性转化是缓慢进展的。在此，我们报告了两名经临床和影像学诊断为 SCCH 的患者，他们接受了帕米膦酸盐静脉治疗，导致两人的临床缓解，但其中一人的硬化完全消退，这是我们在临床经验中的一个新发现。
两名成年女性 SCCH 患者表现为长期胸痛、前胸壁肿胀和肩部功能受损。随后的单光子发射计算机断层扫描-计算机断层扫描（SPECT/CT）显示 SC 区域的骨骼活性和硬化升高，伴骨肥厚，确认了 SCCH 的诊断。由于两名患者的症状最终对非甾体抗炎药（NSAIDs）等标准止痛药均无反应，开始每 3 个月进行一次静脉帕米膦酸盐治疗。帕米膦酸盐可有效减轻疼痛，改善肩部功能，也可降低骨骼闪烁显像中的骨转换。第一个患者的硬化持续存在。然而，在第二个患者中，观察到了硬化的完全消退。
SCCH 仍然是一种罕见的骨骼疾病，目前尚无循证治疗方法。尽管疾病负担很高，但 SCCH 缺乏认识，通常在出现症状很久后才被诊断出来。就本报告中的病例而言，帕米膦酸盐成功地减轻了症状，在第二个病例中甚至导致 CT 成像上的硬化性改变消退。
