SAPHO syndrome: A long-term follow-up study of 120 cases

Objective: To assess the long-term outcome of the synovitis, acne, pustulos is, hyperostosis, osteitis (SAPHO) syndrome. Methods: All patients with the SAPHO syndrome seen at our unit between 1974 and 1997 were identified. Follow-up was prospective from 1992 to 1997. Dat a before 1992 were analyzed retrospectively. Clinical symptoms, treatments and biological data, including erythrocyte sedimentation rate and C-reactiv e protein, were recorded at least yearly. When available, radiological data , HLA B27 status, and findings from bone or skin biopsy specimens were reco rded. For each drug, an efficacy index (EI) was determined as follows: "0" for less than 30% improvement, as judged by the patient, on horizontal visu al analog scale, "0.5" for partial efficacy, and "1" for more than 60% impr ovement. Results: We identified 120 patients with the SAPHO syndrome (50 men, 70 wom en), of whom 102 patients were followed-up prospectively after 1992; 3 of t hese 102 patients were lost to follow-up. Six patients also had Crohn's dis ease, and three had ulcerative colitis. Except for a significant associatio n of palmoplantar pustulosis (PPP) or psoriasis vulgaris (PV) with axial os teitis (P = .007), the dermatologic presentation had no significant influen ce on rheumatic symptoms (ie, osteitis or arthritis, peripheral or axial). The HLA B27 antigen was not significantly associated with a particular patt ern of distribution of arthritis or osteitis. No severe or disabling compli cations were noted. In the 47 patients followed-up for more than 5 years (m ean, 9.5; range, 5 to 23), the mean number of osteitis or arthritis foci In creased during follow-up from 1.57 to 1.91 and from 2.68 to 3.11, respectiv ely. Nonsteroidal antiinflammatory drugs (NSAIDs) were prescribed in 113 of 120 (94%) patients, with a mean Fl of 0.67 (+/-0.39). Corticosteroid ICS) therapy was used in 23 patients, with a mean El of 0.67 (+/-0.42). Colchici ne and sulfasalazine had a mean El of 0.36 (+/-0.44) and 0.16 (+/-0.30), in 28 and 18 patients, respectively. Methotrexate was given to 10 patients (6 with peripheral arthritis), with a mean El of 0.64 (+/-0.48). Doxycyclin ( 100 mg twice daily) was used in 20 patients, usually to treat osteitis, wit h a mean El of 0.26 (+/-0.42). Intraarticular injections of a CS or osmic a cid were used in 27 patients, with a mean Fl of 0.77 (+/-0.35). Conclusions: SAPHO syndrome is a relevant and stable entity, with a good lo ng-term prognosis. NSAIDs and intraarticular injections (CS or osmic acid) most often alleviate rheumatic symptoms, but prednisone or methotrexate are sometimes necessary and appear globally helpful. Copyright @ by W.B. Saund ers Company.