Therapy
Ilium Osteitis as the Main Manifestation of the SAPHO Syndrome: Response to Infliximab Therapy and Review of the Literature

https://doi.org/10.1016/j.semarthrit.2007.08.004Get rights and content

Objective

To analyze the clinical efficacy of anti-tumor necrosis factor (TNF)-α therapy in the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. We describe 2 new cases with ilium osteitis as the main SAPHO syndrome feature and review reported cases treated with anti-TNF-α.

Methods

A literature search of SAPHO syndrome cases treated with TNF-α blocking therapy with special emphasis on osteoarticular and skin responses was performed.

Results

Eighteen cases were identified: 17 SAPHO syndrome and 1 chronic recurrent multifocal osteomyelitis, a juvenile variant of SAPHO syndrome. Sixteen were reported cases and 2 were nonreported cases seen in our arthritis unit. Sixteen patients received infliximab and 2 received etanercept, with an early, sustained clinical improvement in most cases.

Conclusions

Anti-TNF-α therapies are effective treatment for patients with refractory SAPHO syndrome, not only for cutaneous lesions but also for persistent bone lesions such as osteitis.

Section snippets

Methods

We report 2 patients with the SAPHO syndrome treated with infliximab and retrospectively reviewed patients seen in our department between 2000 and 2007. Additionally, a computer-assisted (MEDLINE, National Library of Medicine, Bethesda, MD) literature search was accomplished to locate all reports of SAPHO syndrome treated with TNF-α blockers published in English since 1999, when TNF-α blockers became commercially available. Cases reported in abstracts from European League Against Rheumatism and

Case Report 1

A 45-year-old woman with no other relevant medical history complained of pain in the right buttock, accompanied by occasional fever and asthenia at 21 years of age. Radiographs showed right sacroiliitis and bone scintigraphy showed mild uptake in the right sacroiliac joint. The patient was HLA-B27 negative. Mild, pustular skin lesions on the palms of the hands appeared several months after the first visit (November 1989). An initial diagnosis of psoriatic arthritis was made based on the

Results

Eighteen patients were identified in the literature search (13 women and 5 men) including 16 reported cases (5, 6, 7, 8, 9, 10, 11, 12) and 2 unpublished cases from our unit. Seventeen patients had SAPHO syndrome and 1 patient had chronic recurrent multifocal osteomyelitis. The clinical characteristics are shown in Table 1.

The mean age at introduction of anti-TNF-α therapy was 42.3 ± 14 years (range, 16-67 years). All patients had osteoarticular features, including chest wall involvement in 16

Discussion

We describe 2 cases of the SAPHO syndrome the main manifestation of which was extensive osteitis of the ilium that initially presented as sacroiliitis without involvement of the anterior chest wall or skin lesions. This unusual presentation of the SAPHO syndrome led us to make a differential diagnosis with various bone and joint disorders including spondyloarthropathy, osteomyelitis, primary bone malignancy, hematological conditions such as lymphoma, and even Paget’s disease of the bone.

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