The SAPHO Syndrome

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

doi:10.1016/j.semarthrit.2012.05.006

Cited by 266 publications

(290 citation statements)

References 93 publications

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“…In this case, given the patient's diffuse bone involvement and robust dermatologic findings, SAPHO syndrome was considered. Dermatologic findings of SAPHO syndrome appear as severe acne or pustules that most commonly present on the palms or soles [7,11]. However, our patient had excoriations secondary to pruritus but lacked acneiform lesions or palmoplantar pustulosis as typically would be seen with SAPHO syndrome.…”

Section: Discussion and Treatmentmentioning

confidence: 51%

“…As noted earlier, SAPHO syndrome, consisting of synovitis, acne, pustulosis, hyperostosis, and osteitis, is an inflammatory bone condition that often involves skin and has some overlapping features with spondyloarthropathies [11]. This rare condition often is unrecognized because of the wide variability in its musculoskeletal and cutaneous manifestations [11].…”

Section: Discussion and Treatmentmentioning

confidence: 97%

“…This rare condition often is unrecognized because of the wide variability in its musculoskeletal and cutaneous manifestations [11]. This condition has been described in the literature as chronic recurrent multifocal osteomyelitis (CRMO) and various other names [7,11].…”

Section: Discussion and Treatmentmentioning

confidence: 99%

“…Lymphoma with Secondary Bone Involvement (Stage IV) SAPHO syndrome (previously known as chronic recurrent multifocal osteomyelitis, but more recently renamed to reflect the features that often accompany it, specifically synovitis, acne, pustulosis, hyperostosis, and osteitis [11])…”

Section: Differential Diagnosismentioning

confidence: 99%

See 3 more Smart Citations

A 26-year-old Woman With Bilateral Leg Pain and Pruritus

Morell

Gardner

Suva

et al. 2015

Clinical Orthopaedics &Amp; Related Research

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Section: Discussion and Treatmentmentioning

confidence: 51%

Section: Discussion and Treatmentmentioning

confidence: 97%

Section: Discussion and Treatmentmentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

See 2 more Smart Citations

A 26-year-old Woman With Bilateral Leg Pain and Pruritus

Morell

Gardner

Suva

et al. 2015

Clinical Orthopaedics &Amp; Related Research

View full text Add to dashboard Cite

“…The association of CRMO with arthritis and pyogenic skin manifestations identifies a distinct clinical entity called SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome, usually observed in young adults and characterized, at least in few anecdotal cases, by the same positive response to anti-IL-1 treatment 10 .…”

Section: Rheumatologymentioning

confidence: 99%

Osteolytic Lesion in PAPA Syndrome Responding to Anti-interleukin 1 Treatment

et al. 2014

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Tofacitinib for the Treatment of Nail Lesions and Palmoplantar Pustulosis in Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome

Cao

et al. 2021

JAMA Dermatol

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IMPORTANCENail involvement is common in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, which has a strong association with quality of life in patients with SAPHO. Tofacitinib is an oral Janus kinase inhibitor that has been previously shown to be effective for nail psoriasis.OBJECTIVE To assess the efficacy and safety of tofacitinib for the treatment of nail involvement in SAPHO syndrome. INTERVENTIONS Participants received tofacitinib, 5 mg, twice daily, for 12 weeks. DESIGN, SETTING, AND PARTICIPANTS This open-label, single-arm, prospective pilot study included 13 patients with SAPHO syndrome accompanied by nail lesions and active palmoplantar pustulosis who were recruited from Peking Union Medical College Hospital from September 2019 to December 2019. Follow-up was completed in March 2020. Analysis began March 2020. MAIN OUTCOMES AND MEASURESThe primary end point was the percentage of the change from baseline in Nail Psoriasis Severity Index scores at week 12. Secondary end points included the percentage of the change from baseline in Palmoplantar Psoriasis Area and Severity Index scores, change from baseline in Visual Analogue Scale scores in global osteoarticular pain, Dermatology Life Quality Index scores, and inflammatory markers. Adverse events were recorded throughout the study. RESULTSThirteen female Asian patients (means [SD] age, 39.7 [12.3] years) were included, all of whom completed the study. At week 12, significant improvements were observed in Nail Psoriasis Severity Index scores (median, −67% [interquartile range (IQR), −56% to −77%]; P < .001) and Palmoplantar Psoriasis Area and Severity Index scores (median, −71% [IQR, −58% to −78%]; P < .001). Significant improvement was also noted in Dermatology Life Quality Index scores (median, −12 [IQR, −8.5 to −15]; P < .001) at week 12. A significant decrease in Visual Analogue Scale scores in global osteoarticular pain was observed at week 8 (median, −4 [IQR, 0 to −5]; P = .02) but was not significant at week 12. Inflammatory marker levels were decreased, as indicated by erythrocyte sedimentation rate (median, −8 mm/h [IQR, −4 mm/h to −11 mm/h]; P < .001) and high-sensitivity C-reactive protein levels (median, −1.6 [IQR, −0.3 to −4.1]; P = .01). No severe adverse events were observed. CONCLUSIONS AND RELEVANCEIn this pilot study, tofacitinib yielded significant remission of nail lesions and palmoplantar psoriasis accompanied by an improvement in quality of life in patients with SAPHO syndrome. Additional follow-up studies to evaluate the long-term efficacy and safety of tofacitinib for nail involvement in SAPHO syndrome are warranted.

show abstract

The SAPHO Syndrome

Abstract: 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.

Cited by 266 publications

References 93 publications

A 26-year-old Woman With Bilateral Leg Pain and Pruritus

A 26-year-old Woman With Bilateral Leg Pain and Pruritus

Osteolytic Lesion in PAPA Syndrome Responding to Anti-interleukin 1 Treatment

Tofacitinib for the Treatment of Nail Lesions and Palmoplantar Pustulosis in Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome

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