Dimitrios Daoussis1, Georgia Konstantopoulou2, Pantelis Kraniotis3, Lazaros Sakkas4, Stamatis-Nick Liossis2. 1. Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Patras, Greece. Electronic address: jimdaoussis@hotmail.com. 2. Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, University of Patras Medical School, Patras, Greece. 3. Department of Radiology, Patras University Hospital, Patras, Greece. 4. Department of Rheumatology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Larissa.
Abstract
BACKGROUND: The SAPHO syndrome is a relatively rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Biologics have been used in cases refractory to conventional treatment. METHODS: We present herein a patient with refractory to treatment SAPHO syndrome who exhibited a dramatic and fast response to IL-17 blockade. Additionally, we performed a systematic review of all cases of patients with SAPHO syndrome treated with biologics to date. RESULTS: We identified 66 cases treated with biologics (45 with TNF blockers, 7 with IL-1 blockers, 13 with biologics targeting the IL-23/IL-17 axis, and 1 with tocilizumab). Data support a positive effect of anti-TNF treatment in SAPHO with a response rate in bone and joint manifestations of 93.3%. Skin disease also improved in 21/29 cases (72.4%). Data related to IL-1 inhibition in SAPHO are encouraging with most patients exhibiting a significant response in musculoskeletal manifestations (6/7, 85.7%). However, IL-1 inhibition is not effective in skin manifestations. Ustekinumab seems to have some efficacy with 2/4 patients responding in skin and 3/5 in bone/joint manifestations. Data related to IL-17 blockade indicate efficacy in skin disease with 4/7 patients responding (57.1%). Joint/bone manifestations improved in 3/8 patients (37.5%). CONCLUSIONS: In SAPHO patients not responding to conventional treatment, TNF blockers appear to be the first choice. In patients failing TNF blockers, IL-1 inhibitors and biologics targeting the IL-17/IL-23 axis could be used.
BACKGROUND: The SAPHO syndrome is a relatively rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Biologics have been used in cases refractory to conventional treatment. METHODS: We present herein a patient with refractory to treatment SAPHO syndrome who exhibited a dramatic and fast response to IL-17 blockade. Additionally, we performed a systematic review of all cases of patients with SAPHO syndrome treated with biologics to date. RESULTS: We identified 66 cases treated with biologics (45 with TNF blockers, 7 with IL-1 blockers, 13 with biologics targeting the IL-23/IL-17 axis, and 1 with tocilizumab). Data support a positive effect of anti-TNF treatment in SAPHO with a response rate in bone and joint manifestations of 93.3%. Skin disease also improved in 21/29 cases (72.4%). Data related to IL-1 inhibition in SAPHO are encouraging with most patients exhibiting a significant response in musculoskeletal manifestations (6/7, 85.7%). However, IL-1 inhibition is not effective in skin manifestations. Ustekinumab seems to have some efficacy with 2/4 patients responding in skin and 3/5 in bone/joint manifestations. Data related to IL-17 blockade indicate efficacy in skin disease with 4/7 patients responding (57.1%). Joint/bone manifestations improved in 3/8 patients (37.5%). CONCLUSIONS: In SAPHO patients not responding to conventional treatment, TNF blockers appear to be the first choice. In patients failing TNF blockers, IL-1 inhibitors and biologics targeting the IL-17/IL-23 axis could be used.