Abstract
The camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is a
rare autosomal recessive disease characterized by congenital or early-onset
camptodactyly, childhood-onset non-inflammatory arthropathy associated with
synovial hyperplasia, constrictive pericarditis and coxa vara. This rare disease is
caused by mutations in PRG4 gene which encodes for lubricin, a protein present
in synovial fluid responsible for lubrication.
The case we are reporting is of 9 and half years old boy who presented to us in
pediatric out patient department with early onset camptodactyly and noninflammatory arthropathy. Synovial biopsy shows synovial hyperplasia with
numerous CD68 positive multinucleated giant cells. He was discharged and
physiotherapy was advised with regular follow ups.
Shahzadi Sumbal Ghazi, Muhammad Umar Nisar, Samer Sikander, Jai Krishin. (2018) FAMILIAL CAMPTODACTYLY COXA VARA PERICARDITIS SYNDROME MASQUARADING AS JUVUNILE IDIOPATHIC ARTHRITIS, KHYBER MEDICAL UNIVERSITY JOURNAL, Volume 10, Issue 4.
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