We report a case of 23-year-old Nepalese gentleman with a seropositive RA and very unusual X-ray findings. Our case has bilateral symmetrical inflammatory arthritis involving knees, ankles and wrists. The unusual things about our RA case were related to the facts that there were no hands or feet joints involvement, the non-inflammatory bilateral knees effusion as per the synovial fluid analysis and the very abnormal X-rays findings. Also the patient has diffuse enlargement of the whole hand and feet with no joints tenderness and with some fingers and toes clubbing. 
His inflammatory markers were significantly elevated (ESR 51 and CRP 56), with positive Anti CCP antibody and negative rheumatoid factor. He was started on oral methotrexate with folic acid in addition to oral prednisolone 5 mg daily with modest response.  His X-ray showed diffuse irregular cortical bone thickening and mild fusiform bony enlargement in the diaphyseal region of long bones, with sparing of the epiphyses area. Bone scan: showed isolated symmetrically increased radiotracer uptake involving the diaphyseal region only. 
 
On reviewing his case with MSS radiologist, we diagnosed a very rare autosomal dominant bone disease; Camurati‑Engelmann disease or Progressive Diaphyseal Dysplasia. This is the first case to be reported in our area. These patients usually present with limb pain and easy fatigability. Our case presentation was bilateral symmetrical polyarthritis preserving hands and feet. We report this to increase our rheumatology colleagues’ awareness about such a diagnosis as the treatment is totally different.

Seropositive Rheumatoid Arthritis with very unusual X-ray findings