Renier J C, Audran M
Rheumatology Department, Angers Regional Teaching Hospital, France.
Rev Rhum Engl Ed. 1997 Apr;64(4):233-42.
We conducted a medical record-based study of 169 patients with polyostotic involvement identified among 200 Paget's disease patients. Follow-up was 15 to 41 years in 31 cases. The pelvis was the only bone that was more likely than not to be involved bilaterally. All the other paired bones were more likely to be involved unilaterally and when both sides were involved the two lesions were very often frankly asymmetric. In a given patient, the duration of the various pagetic lesions, estimated from their size and from data provided by an earlier study on the rate of progression of pagetic lesions, was similar in some cases and showed marked differences in others. Aggregation of the lesions into two or three disease duration groups was seen in some patients, suggesting that Paget's disease may occur in two or three waves. When we reviewed the radiographs from 30 patients with a mean follow-up of 23 years, we found new lesions in ten patients. However, a review of bone scans from 18 patients with a mean follow-up of 11 years failed to uncover any firm evidence of new lesion development, perhaps because all these patients received bisphosphonate therapy (etidronate, tiludronate, pamidronate). We also found data suggesting that the disease process spread across a joint in some patients, even in the absence of degenerative joint disease. In particular, in several cases an extensive pagetic lesion was seen on one side of a joint and a considerably smaller lesion on the other side.
我们对200例佩吉特病患者中确诊的169例多骨受累患者进行了一项基于病历的研究。31例患者的随访时间为15至41年。骨盆是唯一比双侧受累更常见的骨骼。所有其他成对骨骼更易单侧受累，且当双侧受累时，两处病变常常明显不对称。在特定患者中，根据各种佩吉特病病变的大小以及早期关于佩吉特病病变进展速度的研究数据估算，不同病变的病程在某些情况下相似，而在其他情况下则有显著差异。部分患者的病变可归为两到三个病程组，这表明佩吉特病可能以两到三个阶段发生。当我们回顾30例平均随访23年的患者的X光片时，发现10例患者出现了新病变。然而，回顾18例平均随访11年的患者的骨扫描结果，未发现任何新病变发展的确切证据，这可能是因为所有这些患者均接受了双膦酸盐治疗（依替膦酸、替鲁膦酸、帕米膦酸）。我们还发现数据表明，即使在没有退行性关节病的情况下，疾病进程在某些患者中也会跨越关节扩散。特别是，在几例病例中，关节一侧出现广泛的佩吉特病病变，而另一侧病变则小得多。
