|
| Author | No. of pts | Therapeutic regimen | Duration of treatment (months) | Clinical/serological effects | Adverse events |
|
| Kraetsch et al. (2001) [52] | 6 AOSD pts | Infliximab + DMARDs + CSs | 5–28 | Resolution of systemic features/Normalization of inflammatory markers | Infusion reactions |
| Husni et al. (2002) [53] | 12 AOSD pts | Etanercept + MTX + CSs ± NSAIDs | 6 | Two pts with systemic features withdrew (flare).
Approximately two thirds improvement of arthritic complaints | Injection-site reactions, upper respiratory tract illness, rash, diarrhea, sinusitis |
| Dechant et al. (2004) [54] | 8 AOSD pts | Infliximab + DMARDs + CSs | 1–5 | Improvement of systemic features and serological markers | Infusion reactions |
| Kokkinos et al. (2004) [55] | 4 AOSD pts | Infliximab + MTX + CSs | 3.5–18 | Remission of systemic features Normalization of inflammatory markers and liver function tests | None reported |
| Fautrel et al. (2005) [56] | 20 AOSD pts | Infliximab and/or etanercept + MTX + CSs | 11 for etanercept/9 for infliximab | Remission: 5 pts/Failure: 4 pts/the rest: partial response | Recurrent bronchitis, lupus rash, optic neuritis, cardiac failure, thigh abscess, rash |
| Fitzgerald et al. (2005) [57] | 4 AOSD pts | Anakinra + MTX + CSs | 6–19 | Rapid resolution of clinical and inflammatory markers in all pts | Viral pneumonia, idiopathic pulmonary hypertension, shingles, flu-like syndrome |
| Woo et al. (2005) [58] | 18 SJIA pts | Tocilizumab + CSs ± MTX | 1-2 | Eleven patients achieved ACR 30 responses, eight achieved ≥50% ACR responses | Oral herpes simple, low lymphocytic levels, and transient increases in ALT |
| Kötter et al. (2007) [28] | 4 AOSD pts | Anakinra + CSs + DMARDs | 12–44 | Rapid resolution of clinical and inflammatory markers in all pts within days. Tapering of CS therapy could not be achieved. | Injection-site erythema that improved within 6 weeks of therapy |
| Kalliolias et al. (2007) [59] | 4 AOSD pts | Anakinra ± CSs | 5–17 | Normalization of clinical (within hours) and inflammatory markers (within 2–4 weeks) along with liver enzymes (within 3 weeks) in all pts. Rapid tapering of CS therapy. | Self-limited injection-site erythema |
| Lequerré et al. (2008) [60] | 15 AOSD and 20 SJIA pts | Anakinra + CSs ± DMARDs | 11–27 | AOSD → CR:9 pts, PR:2 pts, NR:2 pts, Intolerance: 2 pts. Anakinra was stopped in 2 pts due to adverse skin reactions SJIA → CR: 6 pts, PR: 4 pts NR: 10 pts (2 at 2 months). 1 patient who initially achieved a CR developed visceral leishmaniasis and anakinra was stopped. | AOSD: bronchitis, uncomplicated hepatitis A, varicella, cutaneous infections, osteonecrosis of the femoral hip (attributed to CS treatment), local pain and injection-site reactions. SJIA: rhinopharyngitis nonextensive labial herpes and visceral leishmaniasis |
| Laskari et al. (2011) [61] | 25 AOSD pts | Anakinra + DMARDs | 1.5–71 | Complete clinical response in 84% of pts, partial in 12%/Complete laboratory response in 80% of pts | Severe urticarial reaction, various infections, local injection reaction |
| Nigrovic et al. (2011) [62] | 46 SJIA pts | Anakinra + DMARDs + CSs | 14.5 | Systemic features resolved within 1 month in >95% of pts/persistence of active arthritis in 11%. CRP and ferritin normalized within 1 month ( >80% of pts) | Injection site reactions, eosinophilic hepatitis, mild asymptomatic neutropenia, and elevation of liver enzymes |
| Puéchal et al. (2011) [63] | 14 AOSD pts | Tocilizumab + DMARDs + CSs | 6 | Good EULAR response in 64% of pts at 3 months/EULAR remission in 57% at 6 months/Inflammatory markers improved | Necrotizing angiodermatitis, chest pain, mild hyperlipidemia, elevation of liver enzymes |
| Ruperto et al. (2012) [64] | 23 SJIA pts | Canakinumab + CSs | 24 | 60% responders according to the adapted ACR Pediatric 50 criteria and 4 patients inactive by day 15 | Two severe possibly related to the study drug: Epstein-Barr virus infection and hematoma, prolonged activated partial thromboplastin time, gastroenteritis, and syncope |
| Kontzias and Efthimiou (2012) [65] | 2 AOSD pts | Canakinumab + CSs ± MTX | 6–12 | Normalization of inflammatory markers and remission of both systemic and arthritic manifestations | None reported |
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