Renier J C, Audran M
Rheumatology Department, Angers Regional Teaching Hospital, France.
Rev Rhum Engl Ed. 1997 Jan;64(1):35-43.
The mean annual rate of increase in the length of pagetic lesions was 8.5 mm for the skull and tibia and 9.4 mm for the femur, after a follow-up of nine to 16 years according to the bone. The fastest rate of progression was seen at the femur and was 24 mm per year. Thirty years were required for lesions to spread to the entire pelvis and 13 years to all the bones surrounding the obturator foramen. Saber shin deformity of the tibia without involvement of the distal fourth of the bone indicated a disease duration of 25 years, as did involvement of the entire skull. The annual rate of increase in the width of lesions varied widely across patients and was not influenced by gender. Thickening of the skull occurred at a rate of about 4 to 5 mm per decade after pagetization of the bone, although faster rates were seen in some patients; a sandwich-like appearance with a thickness exceeding 32 mm was seen in six of the 29 skulls studied. At the femur and tibia, the increase in width was 10% to 30% per decade after pagetization of the bone; faster thickening was seen in some tibias with saber shin deformity. The thickness of the ischial tuberosity increased by 3 to 4 mm per decade after pagetization. Determination of the degree of hypertrophy is useful for estimating the duration of pagetic lesions when the entire bone is involved at first presentation. Involvement of the entire pelvis indicates a disease duration of 30 to 40 years according to whether the bone is hypertrophied or not. An estimation of age at disease onset in 70 patients suggested that the first bone lesions probably appeared before the age of 30 years in 45 patients (64%), whereas the diagnosis was established before 30 years in only three patients. These data suggest that Paget's disease may be a disease of teenagers and young adults.
根据骨骼情况进行9至16年的随访后，颅骨和胫骨骨炎病变长度的年平均增长率为8.5毫米，股骨为9.4毫米。进展最快的是股骨，每年达24毫米。病变扩散至整个骨盆需要30年，扩散至闭孔周围所有骨骼需要13年。胫骨出现马刀胫畸形但未累及远端四分之一骨骼，表明病程为25年，累及整个颅骨时也是如此。病变宽度的年增长率在患者之间差异很大，且不受性别影响。骨骼发生畸形性骨炎后，颅骨增厚速度约为每十年4至5毫米，不过部分患者增厚速度更快；在研究的29个颅骨中，有6个出现了厚度超过32毫米的三明治样外观。在股骨和胫骨，骨骼发生畸形性骨炎后，宽度每十年增加10%至30%；一些有马刀胫畸形的胫骨增厚更快。坐骨结节厚度在骨骼发生畸形性骨炎后每十年增加3至4毫米。当首次出现病变时整个骨骼都受累时，确定肥大程度有助于估计畸形性骨炎病变的持续时间。根据骨骼是否肥大，整个骨盆受累表明病程为30至40年。对70例患者发病年龄的估计表明，45例患者（64%）的首次骨病变可能出现在30岁之前，而只有3例患者在30岁之前确诊。这些数据表明，畸形性骨炎可能是青少年和年轻人的疾病。
