Background/Purpose:

Camptodactyly-arthropathy-coxa-vara-pericarditis (CACP) syndrome is an autosomal recessive disorder caused by mutations in PRG4 gene that encodes for proteoglycan 4, the main lubricant for joints and tendon surfaces. It is non-inflammatory arthropathy, characterized by joint effusions and synovial hypertrophy. So far, there is no effective treatment for this disorder. We evaluated the effectiveness of yttrium-90 radiosynovectomy in arthropathy of patients with CACP syndrome

Methods:

Consecutive patients with CACP syndrome 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.

Results:

Six (3 boys, 3 girls) patients with 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 2 patients. There was a minimal radioisotope leakage to soft tissue in 2 patients. During the 3 months 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. No difference in knee joint swelling or range of motion. Furthermore, MRI findings were unchanged. However, there was minimal increase in synovial fluid post injection.

Conclusion:

Yttrium-90 radiosynovectomy seems to be safe and well tolerated procedure, but with the given dose and interval time it didn’t show a beneficial therapeutic effect in arthropathy of CACP syndrome.

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-of-yttrium-90-radiosynovectomy-in-camptodactyly-arthropathy-coxa-vara-pericarditis-syndrome/