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SP0038 Chronic nonbacterial osteomyelitis
  1. H. Girschick
  2. for the Eurofever consortium*
  1. Pediatrics, Vivantes Childrens Hospital, Berlin, Germany

Abstract

Chronic nonbacterial osteomyelitis CNO is an inflammatory disorder of the musculoskeletal system with yet unknown etiology. In addition to bone inflammation, patients may present with inflammation of other tissues including e.g. the joints, the skin or the gut. Differential diagnosis is done by exclusion. Recently, the search for a causative etiology has identified mutations in the alkaline phosphatase gene or the interleukin-1 receptor antagonist (IL1RN) gene in a few patients presenting with a clinical picture resembling CNO. In addition, monocytes from CNO patients in general exhibited a reduced IL-10 expression in response to LPS stimulation that is independent of IL10 promoter polymorphisms. Thus, impaired MAPK signaling in CNO monocytes in response to LPS stimulation has been described as a potential causative mechanism. Of note, for the majority of patients no monogenetic or metabolic cause can be identified.

So far the published cohorts of patients were mainly based on local pediatric rheumatology centers or national cohorts. Thus, the Eurofever consortium tried to build up an international database gathering and describing the clinical and laboratory findings of 329 CNO patients. The history, clinical symptoms, laboratory and imaging data were reviewed blindly by the CNO EUROFEVER faculty. In 327 patients the diagnosis was confirmed, in 2 the diagnosis seemed likely. To our knowledge, this is by far the largest CNO group reported in the literature. Cohort description: 263 patients below the age of 18 and 66 patients above 18 were registered. 113 were male and 216 female. 311 patients were caucasian, 10 of other origin. 14 countries participated in the study. The age at disease onset was 11.8 years (STDEV 7.9 a), the age at diagnosis was 12.9 years (mean; STDEV 8,1 a). The CNO EUROFEVER data will be introduced to the EULAR PReS audience. In addition, therapeutic strategies based on a long-term follow up cohort will be presented.

*The Eurofever consortium: Hermann Girschick, Tobias Schwarz, Christine Beck, Annette Jansson, Susanne Schalm, Henner Morbach, Aldo Naselli, Isabelle Koné-Paut, Antonella Insalaco, Susan Nielsen, Troels Herlin, Sulaiman M. Al-Mayouf, Michael Hofer, Pierre Quartier, Christina Boros, Jasmin Kuemmerle-Deschner, Rosa Merino, Loredana Lepore, Silvana Martino, Tamas Constantin, Marija Jelusic-Drazic, Irina Nikishina, Marco Gattorno, Nicolino Ruperto.

Disclosure of Interest None Declared

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