title: The SAPHO syndrome.
abstract: OBJECTIVE
  To review the epidemiology, presentation, diagnosis, treatment, pathogenesis, and genetics of the syndrome known under the acronym of SAPHO for Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis to heighten awareness of this entity.
  
  
  METHODS
  We conducted a Medline search using SAPHO syndrome, chronic recurrent multifocal osteitis/osteomyelitis, and related terms as keywords and extracted further relevant articles from the retrieved references.
  
  
  RESULTS
  The SAHPO acronym identifies a syndrome encompassing a variety of osteoarticular disorders that are frequently accompanied by dermatoses characterized by neutrophilic pseudoabscesses, but can also occur in isolation. SAPHO syndrome is rare, although probably underrecognized because its diagnosis may be challenging because of the wide variability in its musculoskeletal and cutaneous manifestations. This is especially true when atypical sites are involved and when specific skin lesions are absent. There are no standardized treatment protocols available. Current treatments are empirical and have the objective of providing relief from the at times debilitating pain associated with SAPHO syndrome. They include nonsteroidal anti-inflammatory drugs and analgesics as first-line agents. Systemic corticosteroids, disease-modifying anti-rheumatic drugs, biologicals targeting tumor necrosis factor alpha and interleukin-1, and bisphosphonates have all been beneficial in some patients, but ineffective in others. This suggests that the pathogenesis of SAPHO syndrome is multifactorial, but this aspect remains poorly explored, although bacteria and immunological dysfunction are hypothesized to play a role.
  
  
  CONCLUSIONS
  The early recognition, diagnosis, and prompt treatment of SAPHO syndrome can prevent the unnecessary use of long-term antibiotics or invasive procedures, while rapidly alleviating pain in a majority of affected patients.
authors.affiliation: Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, CA 95616, USA.
authors.forename: Minhchau Thi
authors.initials: MT
authors.lastname: Nguyen
authors.forename: Andrea
authors.initials: A
authors.lastname: Borchers
authors.forename: Carlo
authors.initials: C
authors.lastname: Selmi
authors.forename: Stanley M
authors.initials: SM
authors.lastname: Naguwa
authors.forename: Gurtej
authors.initials: G
authors.lastname: Cheema
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
mesh_terms: D020083:Acquired Hyperostosis Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D000293:Adolescent; D000328:Adult; D000368:Aged; D000700:Analgesics / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D015984:Causality; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000188:drug therapy; D059408:Pain Management; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000473:pathology; D055815:Young Adult
chemical_list: D000700:Analgesics; D000894:Anti-Inflammatory Agents, Non-Steroidal