RT Journal Article
SR Electronic
T1 Pain improvement in Camurati-Engelmann disease after anti-TNFα therapy
JF BMJ Case Reports
FD BMJ Publishing Group Ltd
SP bcr-2017-221760
DO 10.1136/bcr-2017-221760
VO 2017
A1 Sónia Moreira
A1 Bernardo Cunha
A1 Nelson Pedro Jesus
A1 Lèlita Santos
YR 2017
UL http://casereports.bmj.com/content/2017/bcr-2017-221760.abstract
AB Camurati-Engelmann disease (CED) is a rare disorder included in the group of craniotubular hyperostosis diseases. Corticosteroids are used for pain management in CED, but in refractory or corticosteroid-non-tolerant patients, pain management is limited. We report the case of a woman with CED diagnosed in early infancy whose initial complaints included persistent bone pain associated with progressive functional disability. She was treated with steroids but over time became dependent on higher doses with only mild pain relief. In her third decade, she was diagnosed with ulcerative colitis (UC) and was treated with mesalazine, azathioprine and prednisolone. Due to recurrent exacerbations of UC, treatment was changed to infliximab, an antitumour necrosis factor-alpha (TNFα). Remission of UC was achieved and CED-associated pain also improved with infliximab. This is the first report showing a possible role of anti-TNFα in pain management in CED with unsatisfactory response to steroids.