Spinal involvement in Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome in two Yemeni sisters

Yasser Emad*, Yasser Ragab, Osama Ibrahim, Maher Khalifa, Ahmed Dawood, Johannes J. Rasker

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

Aim of the work The objective of this clinical report is to describe the detailed magnetic resonance imaging (MRI) findings of the spine, knee and hip joints in two young sisters with Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome. Cases report In two young sisters, both had normal levels of inflammatory markers and typical features of the CACP syndrome with camptodactyly, symmetric joint swelling of both hand joints, knees, ankles and both feet. The affected joints were not tender on palpation with no overlying redness or hotness. Coxa vara on plain X-ray and all cases have evidence of pericarditis. The MRI studies showed the typical rim pattern enhancement of the synovial membrane in the knee and hip joints in both, with no evidence of pannus formation, yet a sizable amount of joint effusion. Both had protrusio acetabuli and one had a unilateral synovial cyst of the hip. MRI studies of the lumbar spine showed evidence of facet joint arthropathy and facet joint effusion, a synovial cyst in both cases and synovial enhancement in post-contrast images with no associated facet joint ankylosis. Conclusion The findings of the two cases confirm the possible axial affection in the CACP syndrome in the form of facet joint disease as a new finding in this rare syndrome. Spinal involvement should be screened in all cases, as it may have consequences for diagnosis and treatment.

Original languageEnglish
Pages (from-to)195-198
Number of pages4
JournalEgyptian rheumatologist
Volume39
Issue number3
DOIs
Publication statusPublished - 1 Jul 2017

Keywords

  • Axial joints affection
  • Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome
  • Facet joint ankylosis
  • Facet joint arthropathy

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