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A phenotypic shift after initiation of IL-1 receptor blockade in a boy with systemic juvenile arthritis

Case presentation

A boy with systemic juvenile arthritis (sJIA) for 7 years, treated with prednisone 1 mg/kg/day, NSAIDS, Methotrexate and TNF-α antagonism, presented at age 15 with severe incapacitating back pain.

Clinically, a cachectic and stunted boy, weight and height SD scores were -5,05 and -3,65 respectively. He had fever, anaemia, splenomegaly and polyartrhitis. There was complete immobility and severe pain of his lumbar spine. X-rays revealed compression fractures of all lumbar vertebrae. Bone mass densitometry confirmed severe osteoporosis (Z-score -4.5 SD). ESR: 33, Hb: 7.4 g/dl, CRP 112 mg/dl.

IL-1 receptor antagonism resulted in a rapid major improvement. Within a few weeks, there was no fever, no arthritis, no splenomegaly, with normal CRP, ESR and Haemoglobin.

Body composition changed dramatically; weight increasing by 10 kg (SDS -2,9) and body fat fraction (DEXA) from 17% to 35%, despite weaning steroids from 1 to 0,3 mg/kg/day. Bone Z-score improved to -3,3 SD.


The present case illustrates the striking effectiveness of Il-1 receptor antagonism in a patient with severe refractory sJIA, and steroid dependency (with devastating side effects, esp on bone mineralization). IL1-ra has been associated with obesity in adults [1, 2]. IL1 receptor antagonism was associated with an important change of body composition in this patient. It remains to be clarified how the interplay between inflammation, Il-1 blockade and corticosteroid therapy impacts on body composition and fat metabolism.


  1. Di Renzo L: Interleukin-1 (IL-1) receptor antagonist gene polymorphism in normal weight obese syndrome: relationship to body composition and IL-1 alpha and beta plasma levels. Pharmacol Res. 2007, 55 (2): 131-8. 10.1016/j.phrs.2006.11.002.

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  2. Saltevo J: Levels of adiponectin, C-reactive protein and interleukin-1 receptor antagonist are associated with the relative change in body mass index between childhood and adulthood. Diab Vasc Dis Res. 2007, 4 (4): 328-31. 10.3132/dvdr.2007.060.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Scott, C., Wouters, C. & de Zegher, F. A phenotypic shift after initiation of IL-1 receptor blockade in a boy with systemic juvenile arthritis. Pediatr Rheumatol 6 (Suppl 1), P31 (2008).

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