camptodactyly-arthropathy-coxa vara-pericarditis syndrome (Jacobs syndrome)

Pathology

• noninflammatory synoviocyte hyperplasia
• subintimal fibrosis of synovial capsule

Genetics

• autosomal recessive
• associated with defects in PRG4

Clinical manifestations

• normal appearing joints at birth
• with advancing age, develop joint failure (see pathology)

More general terms

• genetic syndrome (multisystem disorder)

Database

• OMIM: https://mirror.omim.org/entry/208250