Chronic non-bacterial (Sterile) osteomyelitis in the practice of pediatric rheumatologist, the contemporary diagnostic and treatment approaches: Literature review and own data analysis

Abstract

The article presents the current data on the pathogenesis, features of clinical manifestations, and diagnostics of different variants of chronic non-bacterial (sterile) osteomyelitis (CNO). The inherited forms of the disease, such as syndromes of deficiency of the interleukin-1 receptor antagonist (DIRA), sterile pyarthrosis with pyoderma gangrenosum and acne (PAPA-syndrome), dyserythropoietic anemia with chronic non-bacterial osteomyelitis (Majeed-syndrome) are described. The pathogenesis of CNO and related diseases is based on hyperproduction of proinflammatory cytokines, particularly of interleukin-1β and tumor necrosis factor-α. Based on the literature data and our own observations, the recommendations for diagnostics and treatment of CNO are presented.