Di Carlo M, Silveri F, Tardella M, Carotti M, Salaffi F
Rheumatology Department, Polytechnic University of the Marche, c/o Ospedale "Carlo Urbani", Via Aldo Moro, 25, 60035, Jesi (AN), Italy.
Internal Medicine Department, "C. and G. Mazzoni" Hospital, Area Vasta 5, Ascoli Piceno, Italy.
Osteoporos Int. 2016 Oct;27(10):3127-31. doi: 10.1007/s00198-016-3604-9. Epub 2016 Apr 22.
Sclerosing bone disorders are uncommon diseases and represent a diagnostic challenge. Osteocondensation is a bone alteration, involving both acquired and hereditary conditions. Multiple diaphyseal sclerosis (Ribbing disease) is an inherited condition. It is characterized by excessive proliferation of endosteal and periosteal osseous tissue at the diaphyses of long bones, especially of tibias and femurs. The conventional radiology depicts cortical thickening of diaphyses of long bones while bone scintigraphy shows characteristically an abnormal tracer concentration in the involved diaphyses. The magnetic resonance imaging (MRI) examination confirms the presence of sclerosis and reveals bone marrow edema in the diaphyses of the afflicted bones. Due to the lack of knowledge of the pathophysiology, the treatment is empirical with glucocorticoids or bisphosphonates. Concerning bisphosphonates, the literature reports are conflicting. We report the case of a patient that showed lack of response to intravenous neridronate within 1 year of treatment, both in terms of pain and persistence of bone marrow edema at MRI.
硬化性骨病是罕见疾病,构成诊断挑战。骨致密化是一种骨质改变,涉及后天性和遗传性疾病。多发性骨干硬化症(里宾病)是一种遗传性疾病。其特征是长骨干骺端,尤其是胫骨和股骨的干骺端骨内膜和骨膜骨组织过度增生。传统放射学显示长骨干骺端皮质增厚,而骨闪烁显像特征性地显示受累干骺端有异常示踪剂浓聚。磁共振成像(MRI)检查证实硬化的存在,并显示受累骨骼干骺端有骨髓水肿。由于对病理生理学缺乏了解,治疗采用糖皮质激素或双膦酸盐进行经验性治疗。关于双膦酸盐,文献报道相互矛盾。我们报告了一例患者,在治疗1年内,静脉注射奈立膦酸后,无论是疼痛还是MRI上骨髓水肿的持续存在,均显示无反应。