Osteitis

Abstract: Objective To define non-bacterial osteitis (NBO) as a clinical entity possibly associated with autoimmune manifestations. Patients with sterile osteitis were analysed to develop diagnostic criteria. Methods A total of 89 patients with non-bacterial inflammatory bone lesions were observed for a median of 49 months. History, diagnostic imaging, laboratory and histological data were obtained. Mutation analysis in the genes PSTPIP1 and PSTPIP2 was performed. Results Patients had an onset of disease at a median age of 10 yrs [interquartile range (IQR) 7.5-12] and suffered a median period of 21 (IQR 9-52) months with a median of three foci per patient. Twenty percent of all the patients demonstrated associated autoimmune disorders, particularly of the skin and bowel. The majority of bone lesions were located in the vertebrae and metaphyses. Slight-to-moderate elevation of inflammation values were found in all the patients and antinuclear antibodies were elevated in 30%. Non-steroidal anti-inflammatory drugs (NSAIDs) were effective in 85% of the patients. HLA-B27 and Human Leukocyte Antigen-DR (HLA-DR)-classification did not differ from the general population. Autoimmune diseases in 40% of all the families, multiply affected family members, linkage to 18q21 and mouse models strongly indicate a genetic basis for NBO. We observed three different courses of disease regarding the duration of complaints, rate of complications and associated autoimmune manifestations leading to a new classification of NBO. Conclusions Clinical analysis of our cohort leads us to define NBO as a distinct disease entity with three clinical presentations: acute NBO, chronic recurrent multifocal osteomyelitis or persistent chronic NBO. Diagnostic criteria were proposed to differentiate NBO from diseases with similar clinical presentation.

Abstract: The authors report the data collected by a national investigation organized by the French Society of Rheumatology, concerning the osteo-articular manifestations of severe acne, palmo-plantar pustulosis and primary thoracic and peripheral hyperostosis. This investigation collected 85 case-reports including 13 severe acne, 44 PPP and 28 hyperostosis without the dermatitis mentioned above. From this investigation, it appears that dermatological and osseous pictures described under various denominations, present common characteristics and transition forms justifying their common study under the acronym SAPHO (Syndrome Acne-Pustulosis-Hyperostosis-Osteitis). The bony involvement, especially anterior thoracic, but also vertebral and even peripheral seems to be the common denominator between these diseases. It realizes a true rheumatoid inflammatory osteitis, osseous counterpart of synovial and cartilagenous affections in inflammatory rheumatoid diseases. This group has rather loose connections with common psoriasis and slightly more definite relationships with primary ankylosing spondylarthritis. These clinical and immunogenetic connections occur also through bony involvement.