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A phenotypic shift after initiation of IL-1 receptor blockade in a boy with systemic juvenile arthritis
Pediatric Rheumatology volume 6, Article number: P31 (2008)
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.
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.
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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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, P31 (2008). https://doi.org/10.1186/1546-0096-6-S1-P31
- Body Composition
- Compression Fracture
- Severe Osteoporosis