Background/Purpose: SAPHO Syndrome is a chronic disease with bone, joint and skin involvement characterized by synovitis, acne, pustulosis, hyperostosis, osteitis. Treatment decisions of SAPHO syndrome are generally based on experts’ opinions. Our aim is to evaluate treatment options and response of patients with SAPHO syndrome in the referral outpatient clinic

Methods: All patients with SAPHO syndrome diagnosed since February 2014 have been registered and monitored prospectively with a standard form. All patients met the Benhamou criteria (1) for SAPHO syndrome. At baseline and follow-up visits, patients were assessed regarding acute phase reactants, BASDAI, BASFI, number of swollen and tender joints (28 joints), patient global assessment (particularly assessed for skin and joint) and overall assessment of patient. Baseline and follow-up treatment choices were decided by expert opinions. There were 25 patients with SAPHO syndrome and 22 patients had complete demographic and treatment data, who were analyzed.  

Results: Twenty-two patients (12 (54.5%) female) with SAPHO syndrome were evaluated. Mean age of diagnosis was 40.1±13.2, the mean follow-up duration was 17.6±24.5 months. The evaluation of the disease activity at initial and final visits were shown in figure 1. Initial and maintenance treatment regimens were given in table 1. At baseline, distribution of each particular treatment choices were pamidronate in 10 patients (45.4%), methotrexate in 9 patients (40.9%), sulphasalazine in 6 patients (27.2%), corticosteroids in 3 patients (13.6%), cyclosporine, anti-TNF and surgery each in 1 particular patient (4.5%).  During follow-up period, 3 patients (13.6%) were switched to anti-TNF treatments due to inefficacy of other treatments. At the final visit, all patients achieved remission according to physician assessment. We did not find any treatment-related side effects during follow-up period.

Conclusion: Randomized controlled trials have not been performed to evaluate treatment options for rare rheumatologic disorders such as SAPHO syndrome. In our case series, pamidronate seems to be an effective treatment option against both osteoarthritic and skin manifestations, on the other hand anti-TNF treatments should be kept in mind on the resistant patients.

Reference

1. Benhamou CL. Rev Rhum Mal Osteoartic. 1984

Table 1. Distribution of drugs administered at baseline and for the maintenance