SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome comprises many features, but has no specific treatment.1 2 According to the target symptom (synovitis, skin, bone) non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying anti-rheumatic drugs (DMARDs), bisphosphonates3 and tumour necrosis factor (TNF) blockers4 may be proposed, but with variable results. Some patients do not exhibit improvement of symptoms despite use of several, sometimes combined, lines of treatment. An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist has recently been described5 under the name DIRA. It associates sterile multifocal osteomyelitis, periostitis and pustulosis, which looks like SAPHO, with good response to anakinra, an interleukin 1 (IL-1) receptor antagonist. We describe the …