The SAPHO Syndrome

Minhchau Thi Nguyen, Andrea Borchers, Carlo Selmi, Stanley M. Naguwa, Gurtej Cheema, M. Eric Gershwin

Research output: Contribution to journalArticle

105 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)254-265
Number of pages12
JournalSeminars in Arthritis and Rheumatism
Volume42
Issue number3
DOIs
Publication statusPublished - Dec 2012

Fingerprint

Acquired Hyperostosis Syndrome
Osteitis
Hyperostosis
Skin Manifestations
Pain
Antirheumatic Agents
Synovitis
Acne Vulgaris
Diphosphonates
Non-Steroidal Anti-Inflammatory Agents
Drug Delivery Systems
Clinical Protocols
Interleukin-1
Skin Diseases
Early Diagnosis
Adrenal Cortex Hormones
Epidemiology
Therapeutics
Tumor Necrosis Factor-alpha
Anti-Bacterial Agents

Keywords

  • Acne
  • Hyperostosis
  • Osteitis
  • Pustulosis
  • Synovitis

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine

Cite this

Nguyen, M. T., Borchers, A., Selmi, C., Naguwa, S. M., Cheema, G., & Gershwin, M. E. (2012). The SAPHO Syndrome. Seminars in Arthritis and Rheumatism, 42(3), 254-265. https://doi.org/10.1016/j.semarthrit.2012.05.006

The SAPHO Syndrome. / Nguyen, Minhchau Thi; Borchers, Andrea; Selmi, Carlo; Naguwa, Stanley M.; Cheema, Gurtej; Gershwin, M. Eric.

In: Seminars in Arthritis and Rheumatism, Vol. 42, No. 3, 12.2012, p. 254-265.

Research output: Contribution to journalArticle

Nguyen, MT, Borchers, A, Selmi, C, Naguwa, SM, Cheema, G & Gershwin, ME 2012, 'The SAPHO Syndrome', Seminars in Arthritis and Rheumatism, vol. 42, no. 3, pp. 254-265. https://doi.org/10.1016/j.semarthrit.2012.05.006
Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME. The SAPHO Syndrome. Seminars in Arthritis and Rheumatism. 2012 Dec;42(3):254-265. https://doi.org/10.1016/j.semarthrit.2012.05.006
Nguyen, Minhchau Thi ; Borchers, Andrea ; Selmi, Carlo ; Naguwa, Stanley M. ; Cheema, Gurtej ; Gershwin, M. Eric. / The SAPHO Syndrome. In: Seminars in Arthritis and Rheumatism. 2012 ; Vol. 42, No. 3. pp. 254-265.
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