Cranio-osteoarthropathy associated with inflammatory arthritis

Results Case report Female child born at term to consanguineous parents from Asian ethnicity needed surgical ligation of a large patent ductus arteriosus (PDA) during the neonatal period. Finger clubbing was noted first from 6 weeks of age; over the next few years, despite detailed assessment, no secondary cause could be found. At the age of 7 years, she presented to the rheumatology team with swelling, pain and stiffness in her knees and ankles; although her joint symptoms dated back to since was 3 years old. She always had excessively sweaty hands and feet. On examination she had marked clubbing of her fingers and toes, with synovial effusions and thickening of knees and ankles. She also had long fingers and toes and prominent nose. Fingers were particularly hypermobile and she had prominent shoulders. Her initial synovial fluid analyses were consistent with primary inflammatory arthropathy, although subsequent synovial analysis has been of ‘non-inflammatory type’. Over the next few years she received multiple intraarticular joint steroid injections, with short lasting symptom relief. Plain radiographs from childhood had evidence of wormian bones. Radiographs also revealed periosteal reaction in all distal phalanges and femurs. Gadolinium enhanced MRI was consistent with inflammatory arthritis. Autoantibody screen has always been negative, persistently raised IgG and persistent mild elevation of angiotensin converting enzyme (ACE) levels. Her previous treatments for inflammatory joint disease have included methotrexate, etanercept and rituximab. She is seeing ophthalmologists for meiobian gland dysfunction, corneal neovascularisation and punctate epithelial erosion. She is waiting to see psychology for low mood.