Sustained remission of SAPHO syndrome with pamidronate: a follow-up of fourteen cases and a review of the literature

Abstract

Objective: To evaluate the efficacy of intravenous (i.v.) pamidronate in patients with SAPHO syndrome refractory to first line treatments and to review the available literature on pamidronate for this indication.

Methods: We report 14 cases of SAPHO syndrome refractory to non-steroideal anti-inflammatory drugs (NSAIDs), glucocorticoids and disease modifying anti-rheumatic drugs (DMARDs) treated with i.v. pamidronate. All patients received i.v. 60 mg pamidronate/day for 3 consecutive days. The primary evaluation criterion was the disappearance of bone pain, considered as the reduction in the visual analogic scale for pain (VAS) greater than 50%.

Results: Ten patients were females and 4 were males. The mean age at onset was 40.4 years old. Ten patients presented a relapsing-remitting course, while in 4 cases the disease followed a prolonged course. In all cases anterior chest wall involvement occurred early in the disease. In 2 cases there was also a peripheral monoarthritis. Eleven patients experienced several flares of palmo-plantar pustulosis, while severe acne was present in 2. In one case there was no cutaneous involvement. Twelve of the 14 patients had a good response after 3 infusions and in 8 of these patients a sustained remission was observed. The recurrence of skin manifestations does not seem to be influenced by pamidronate.

Conclusions: Pamidronate appears to be an effective treatment in the osteo-articular manifestations of SAPHO syndrome. As far as cutaneous lesions are concerned, evidence of efficacy is not so impressive.