Clinical heterogeneity of SAPHO syndrome: challenging diagnose and treatment

Francesco Cianci, Angelo Zoli, Elisa Gremese, Gianfranco Ferraccioli

Research output: Contribution to journalArticle

24 Citations (Scopus)


Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare disease which is often misdiagnosed and under-recognized, because of its peculiar and heterogeneous clinical presentation. Its main features consist of cutaneous and osteoarticular manifestations, the latter affecting more often the anterior chest wall and having typical radiologic findings. There are no validated diagnostic criteria for SAPHO and no guidelines for treatment, due mainly to its rarity; as a consequence, therapy is empirical and aimed to control pain and modifying inflammatory process. To date, the use of anti-TNF agents has been proved to be a valid alternative for patients unresponsive to conventional treatments, such as NSAIDs, corticosteroids, DMARDs and biphosphonates. The clinical heterogeneity of the disease, possibly due to differences in pathogenic mechanism of different manifestations, is challenging for both diagnosis and treatment, which should aim to control both skin and bone involvement in different clinical subsets. Here, we summarize the current status of knowledge about the SAPHO syndrome and present two cases of patients with very different disease manifestations, suggesting the need for personalized treatment.
Original languageEnglish
Pages (from-to)2151-2158
Number of pages8
JournalClinical Rheumatology
Publication statusPublished - 2017


  • Acquired Hyperostosis Syndrome
  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents, Non-Steroidal
  • Biologic drugs
  • Diphosphonates
  • Female
  • Humans
  • Hyperostosis
  • Middle Aged
  • Osteitis
  • Pain
  • Pain Management
  • Skin
  • Skin Diseases
  • Treatment
  • Tumor Necrosis Factor-alpha


Dive into the research topics of 'Clinical heterogeneity of SAPHO syndrome: challenging diagnose and treatment'. Together they form a unique fingerprint.

Cite this