POS1305 PEDIATRIC SAPHO SYNDROME: SINGLE ENTITY OR COMBINATION OF DIFFERENT DISEASES?

Background SAPHO is a heterogeneous autoinflammatory disease, characterized by bone and joint involvement wide variety of dermatologic manifestations, including palmo-plantar pustulosis (PPP), acne, hidradenitis suppurativa (HS), pyoderma gangrenosum (PG), psoriasis, Snedd-Wilkinson disease Sweet syndrome. In children, the osteo-articular manifestations are chronic recurrent multifocal osteomyelitis (CRMO), involving metaepiphysis long bones axial skeleton, as in adult form. The treatment still today challenge, no therapeutic guidelines available children: NSAIDs frequently used first line for CRMO lesions, followed bisphosphonates, methotrexate biologic therapies. literature there many case reports but only few series 1-3 , which this largest Europe (29 cases reported China 4 ). Objectives to report clinical radiological features response therapy children. Methods data (serological, imaging therapy) 13 patients, between 2001 2021 at Unit Autoinflammatory diseases Gaslini Hospital were reviewed. Results 7/13 patients male. At onset, acne-HS was present manifestation 8/13 while PPP concomitant or subsequent manifestations. skin characterised acne (comedonic 3 cases, nodulo-cystic cases), (5), HS (3), PG (1). Multifocal inflammatory lesions with both 92% whereas sternoclavicolar observed 85% patients. onset all treated NSAIDS 4, also PPP, achieved remission. Methotrexate salazopirin used, 2/13 severe painful bisphosphonates successfully employed. 5 cutaneous mild did not need treatment. 9/13 required upgrade biologics: Etanercept partial result, Adalimumab 6 had an optimal response, 1 even if requiring increased dose (80 mg per week) swapped dapsone. Anakinra ustekinumab secukinumab (2) efficacious. It note that comedonic refractory treatments needed multiple cycling achieve remission only. Conclusion Our well known Despite CRMO, any This may suggest identify phenotypes different treatments. References [1]Luzzati M, Ital J Pediatr. 2020;46(1):169. [2]Tlougan BE, Pediatr Dermatol. 2009;26(5):497 [3]Beretta-Piccoli BC, Eur 2000;159(8):594 [4]Wu N, Clin Rheumatol. 2021;40(4):1487 Disclosure Interests Caterina Matucci-Cerinic: None declared, Gianmaria Viglizzo: Roberta Caorsi Speakers bureau: Sobi, Consultant of: Novartis, Eli Lilly, Stefano Volpi: Clara Malattia: Marco Gattorno SOBI