Al-Mayouf Sulaiman Mohammed, Almutairi Nora, Alismail Khalid
King Faisal Specialist Hospital and Research Center, Clinic of Pediatric Rheumatology, Riyadh, Saudi Arabia Phone: +996114427761 E-mail:
Mol Imaging Radionucl Ther. 2017 Feb 5;26(1):33-37. doi: 10.4274/mirt.29484.
Camptodactyly-arthropathy-coxa-vara-pericarditis (CACP) syndrome is an autosomal recessive disorder caused by mutations in gene that encodes for proteoglycan 4, the main lubricant for joints and tendon surfaces. It is a non-inflammatory arthropathy, characterized by joint effusions and synovial hypertrophy. So far, there is no effective treatment for this disorder. To evaluate the effectiveness of yttrium-90 radiosynovectomy in arthropathy of patients with CACP syndrome.
Consecutive patients with CACP syndrome were prospectively evaluated at the enrollment and 3 months after the right knee injection with yttrium-90. The outcome variables were patient/parent and physician's global assessment measured by a 3-point scale, right knee swelling and range of motion on a 3-point scale, in addition to magnetic resonance imaging (MRI) assessment of the right knee for bone, cartilage, fluid, synovial hypertrophy and soft tissue changes.
Six (three boys, three girls) patients with a mean age of 12 years and mean follow-up duration of 8.5 years completed a single right knee intra-articular yttrium-90 injection with 5 mCi. The procedure was well tolerated without adverse events apart from mild and transient joint pain in two patients. There was a minimal radioisotope leakage to soft tissue in two patients. During the 3-month follow-up interval, there was no improvement in the outcome variables. Patients and parents did not notice favorable therapeutic effects and global physician assessment was unsatisfactory. There was no difference in knee joint swelling or range of motion. Furthermore, MRI findings were unchanged. However, there was a minimal increase in synovial fluid post injection.
Yttrium-90 radiosynovectomy seems to be a safe and well tolerated procedure, however, it did not show a beneficial therapeutic effect in arthropathy of CACP syndrome with the given dosage and interval. Studies including a larger number of patients and probably repeated injections are needed to derive satisfactory results about the effectiveness of yttrium-90 in CACP syndrome patients.
屈曲指-关节病-髋内翻-心包炎（CACP）综合征是一种常染色体隐性疾病，由编码蛋白聚糖4的基因突变引起，蛋白聚糖4是关节和肌腱表面的主要润滑剂。它是一种非炎症性关节病，其特征为关节积液和滑膜增生。到目前为止，这种疾病尚无有效治疗方法。本研究旨在评估钇-90放射性滑膜切除术对CACP综合征患者关节病的疗效。
对连续性入选的CACP综合征患者在入组时及右膝注射钇-90后3个月进行前瞻性评估。观察指标包括患者/家长及医生的整体评估（采用3分制）、右膝肿胀情况（采用3分制）及活动范围，此外还包括对右膝进行磁共振成像（MRI）评估，观察骨骼、软骨、积液、滑膜增生及软组织变化。
6例（3男3女）患者，平均年龄12岁，平均随访时间8.5年，均接受了单次右膝关节内注射5毫居里的钇-90。该操作耐受性良好，除2例患者出现轻微短暂的关节疼痛外，未发生不良事件。2例患者出现微量放射性同位素渗漏至软组织。在3个月的随访期内，观察指标无改善。患者及家长未注意到明显的治疗效果，医生整体评估不满意。膝关节肿胀及活动范围无差异。此外，MRI检查结果无变化。不过，注射后滑膜液有微量增加。
钇-90放射性滑膜切除术似乎是一种安全且耐受性良好的操作，然而，在给定剂量和间隔下，它对CACP综合征关节病未显示出有益的治疗效果。需要开展纳入更多患者且可能需重复注射的研究，以得出关于钇-90对CACP综合征患者有效性的满意结果。
