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SAT0269 The Clinical Features of Sapho Syndrome in Japanese Patients: A Single Center Cohort Study
  1. Y. Akiyama,
  2. T. Sato,
  3. S. Hanai,
  4. Y. Kamata,
  5. K. Nagatani,
  6. M. Iwamoto,
  7. H. Okazaki,
  8. S. Minota
  1. Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, shimotsuke-shi, Japan

Abstract

Background SAPHO syndrome is a rare syndrome characterized by osteoarticular and cutaneous involvements. Some drugs including non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, antibiotics, synthetic disease modifying antirheumatic drugs (sDMARDs) and bisphosphonates have been used for the treatment of SAPHO syndrome, but there are no randomized trials about the treatment. Recently, some cases who responded to anti-TNF agents have been reported.

Objectives We investigated the clinical features and treatment courses of SAPHO syndrome in our division.

Methods We retrospectively reviewed the clinical data of patients with the diagnosis of SAPHO syndrome who were hospitalized in our division from 2003 to 2014. We used Hayem's criteria as the diagnostic criteria.

Results Twenty patients (6 males and 14 females) were included in this study. The median age at the time of diagnosis was 53 years (22-64 years). Thirteen patients (65%) had palmoplantar pustulosis (PPP), one (5%) had sterile pustulosis, and six (30%) had no sign of cutaneous manifestation. Osteoarticular lesions involved the anterior chest wall (65%), the spine (45%), sacroiliac joint (30%), and osteitis (20%). Axial arthritis, peripheral arthritis, and enthesitis developed in 80%, 45%, and 20% of the patients, respectively. Although PPP developed at the same time of bone involvement in five patients (25%), five patients (25%) had PPP after the onset of bone symptoms and four patients (20%) had PPP before bone symptoms. There was no association with PPP and peripheral arthritis, sternoclavicular arthritis, or spondylitis. Comorbidities included metabolic syndrome in 25% of the patients and autoimmune diseases in 20%. Rheumatoid factor examined in 19 patients was positive in 2 patients and one of them was also positive for anti-citrullinated protein antibody. The elevation of C-reactive protein or erythrocyte sedimentation rate was observed in 15 of 19 patients. None of 10 patients tested for HLA was positive for HLA-B27. Various kinds of drugs were used in these patients. NSAIDs were medicated in all the patients. NSAIDs, glucocorticoids, sDMARDs, bisphosphonates, or anti-TNF agents were administered as monotherapy, or combination therapy with two or more drugs. Among five patients who received anti-TNF agent, two patients (40%) responded to the treatment. Finally, the results of the treatments were partial or complete response in 17 patients and no response in 3 patients.

Conclusions In our experience, the frequency of osteitis and axial involvements was higher compared with past studies. The effect of anti-TNF agents was reported in some case series, but the effect was dependent on the patients in our study. Since there are several patients refractory to treatment, the treatment for SAPHO syndrome needs further exploration.

References

  1. Hayem G et al. Semin Arthritis Rheum 1999;29:159-171

  2. Jurik AG, et al. J Pediatr Orthop 1998;8:49-58

  3. Colia M, et al. Arthritis Rheum 2009;61:813-821

  4. Grosjean C, et al. J Rheumatol 2010;37:639-643

  5. Hurtado-Nedelec M, et al. J Rheumatol 2010;37:401-409

  6. Sueli C, et al. Rheum Dis Clin 2013;39:401-418

Disclosure of Interest None declared

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