title: Infliximab treatment in pachydermoperiostosis: a rare disease without an effective therapeutic option.
abstract: Hypertrophic osteoarthropathy (HOA) is characterized by periostitis of tubular bones, thickened skin, and digital clubbing. Its pathogenesis is unknown but an inflammatory factor and increased bone remodeling have been implicated. It is a very rare disease, usually diagnosed late with few therapeutic options. Bone and joint pains are secondary to periostitis and are usually difficult to control. Tumor necrosis factor-alpha is a cytokine that induces other inflammatory cytokine production, has an osteoclastogenic effect in different rheumatic diseases and probably also has an important role in periostitis and the systemic inflammatory manifestations in HOA. We describe the case of a patient with the primary form of HOA, who had refractory bone pain and arthritis that responded partially to infliximab treatment.
authors.affiliation: Rheumatology Division, University Hospital, Federal University of Santa Catarina, Florianópolis, Brazil.
authors.forename: Fernanda Vicente
authors.initials: FV
authors.lastname: da Costa
authors.forename: Sonia Cristina
authors.initials: SC
authors.lastname: de Magalhães Souza Fialho
authors.forename: Adriana F
authors.initials: AF
authors.lastname: Zimmermann
authors.forename: Fabrício S
authors.initials: FS
authors.lastname: Neves
authors.forename: Gláucio Ricardo
authors.initials: GR
authors.lastname: Werner de Castro
authors.forename: Ivânio Alves
authors.initials: IA
authors.lastname: Pereira
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D006801:Humans; D000069285:Infliximab; D008297:Male; D010004:Osteoarthropathy, Primary Hypertrophic / Q000188:drug therapy*; D035583:Rare Diseases; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
chemical_list: D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab