TY - JOUR
T1 - Spinal involvement in Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome in two Yemeni sisters
AU - Emad, Yasser
AU - Ragab, Yasser
AU - Ibrahim, Osama
AU - Khalifa, Maher
AU - Dawood, Ahmed
AU - Rasker, Johannes J.
PY - 2017/7/1
Y1 - 2017/7/1
N2 - 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.
AB - 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.
KW - Axial joints affection
KW - Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome
KW - Facet joint ankylosis
KW - Facet joint arthropathy
UR - http://www.scopus.com/inward/record.url?scp=85006276847&partnerID=8YFLogxK
U2 - 10.1016/j.ejr.2016.10.001
DO - 10.1016/j.ejr.2016.10.001
M3 - Article
AN - SCOPUS:85006276847
VL - 39
SP - 195
EP - 198
JO - Egyptian rheumatologist
JF - Egyptian rheumatologist
SN - 1110-1164
IS - 3
ER -