Table 1 (abstract P401). Rate of HO flares in 4 FOP patients before and during anti-IL1 treatment
From: Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022)
Patient number and gender | Age at diagnosis | Age at starting anti-IL1 treatment | Flare rate before anti-IL1 | Flare rate during anti-IL1 treatment | Decrease |
|---|---|---|---|---|---|
1. Male | 13.5 years | 15 years (ANA) 15.4 years (CKB) | 40/13=3.07/month (prior to ANA) | ANA 100mg/day – 3/4=0.75/month. CKB ~ 2.7mg/kg monthly – 5/4=1.25/month. Split CKB 1.35mg/kg twice monthly – 13/16=0.81/month. After augmenting CKB dose to >2mg/kg twice monthly – 3/11=0.27/month Weighted mean of flares under anti-IL1 Tx – 0.68/month | 91% |
2. Female | 3 years | 5.7 years | 23/18=1.28/month | CKB 4mg/kg monthly – 6/22=0.27/month, of which 3/6=0.5 were not treated with steroids, at least 5/6=0.83 were within known sites and 3/6=0.5 were within surgical site (anterior right neck) | 79% |
3. Female | 16 months | 23 months | 4/4=1/month | CKB 4mg/kg monthly – 3/11=0.27/month, of which all were post-traumatic forehead swellings, all were partially treated with steroids (1-2 doses) and all became non-palpable over time | 73% |
4. Female | 3 years | 14.3 years (ANA) 14.5 years (CKB) | 9/13=0.69 /month (before ANA) | ANA 100 mg daily, followed by CKB 3.5 mg/kg/month – 5/18=0.27/month, with significantly decreased pain and induration at flare sites | 61% |