Table 4.
Main studies reporting anakinra administration in pediatric FMF, TRAPS, HIDS and uAIDs.
| Type of study | Patients (n) | Dose OD (n) | CR* | PR** | Adverse events | Median follow up (months) | |
|---|---|---|---|---|---|---|---|
| FMF | |||||||
| Kuijk et al.56 | Case report | 1 | 100 mg/day | 100% | – | Mild urticarial rash | NA |
| Calligaris et al.57 | Case report | 1 | 1 mg/kg/day | 100% | – | Local injection site reaction | 18 |
| Roldan et al.55 | Case report | 1 | 1 mg/kg/day | 100% | – | None | 6 |
| Ozen et al.60 | Retrospective | 5 | 1–2 mg/kg/day | 60% (3/5) |
40% (2/5) |
NA | 9 (range 2–30) |
| Meinzer et al.58 | Retrospective | 4 | 1–2 mg/kg/day (1 OD) |
100% | – | Local injection site reaction | 6.5 (range 4–16) |
| Cetin et al.59 | Retrospective | 2 | 1–1.5 mg/kg/day | 100% | – | None | 9.5 (range 7–12) |
| Özçakar et al.61 | Retrospective | 7 | 1 mg/kg/day | 71% (5/7) |
19% (2/7) |
None | 15 (range 9–40) |
| TRAPS | |||||||
| Gattorno et al.62 | Retrospective | 4 | 1.5 mg/kg/day | 100% | – | Local injection site reaction | 11.4 (range 4–20) |
| Grimwood et al.63 | Case series | 2 | 2 mg/kg/day (2 OD) |
100% | – | Local injection site reaction | NA |
| MKD/HIDS | |||||||
| Rigante et al.64 | Case report | 1 | 1 mg/kg/day | – | 100% | Local injection site reaction | 18 |
| Nevyjel et al.65 | Case report | 1 | 30 mg/day | – | 100% | NA | 12 |
| Korppi et al.66 | Case report | 1 | 2 mg/kg/day | 100% | – | None | 6 |
| Bodar et al.67 | Prospective | 2 | 1–2 mg/kg/day (1 OD) |
50% (1/2) |
50% (1/2) |
Local injection site reaction | NA |
| Galeotti et al.68 | Retrospective | 5 | 1–5 mg/kg/day (1 OD) |
20%***
(1/5) |
80%***
(4/5) |
Local injection site reaction, bacterial pneumonia | 13***
(range 2–39) |
| Shendi et al.69 | Case report | 1 | 100 mg/day | 0% | 0% | Prolonged fever | 5 days |
| Levy et al.70 | Case series | 2 | 2–3 mg/kg/day | 50% (1/2) |
50% (1/2) |
NA | 6.5 (range 6–7) |
| Campanilho-Marques71 | Case series | 2 | 2–6 mg/kg/day | – | 100% | Herpes zoster infection | 12 (range 7–17) |
| Santos et al.72 | Case report | 1 | 2–5 mg/kg/day | – | 100% | None | 36 |
| Kostjukovits et al.73 | Case series | 3 | 2 mg/kg/day (1 OD) |
33% (1/3) |
67% (2/3) |
None | 36 (range 24–48) |
| uAIDs | |||||||
| Garg et al.74 | Retrospective | 22 | 2 mg/kg/day (range 2–6 mg/kg/day) |
36% (8/22) |
36% (8/22) |
Infection (8), neutropenia (7), local injection site reaction (5) | 35 (range 11–153) |
*
CR, no attacks and APRs, i.e. C-reactive protein, erythrocyte sedimentation rate and serum amyloid A within normal range.
**
PR, decreasing the attack rate/duration ⩾50% and/or APPs reducing ⩾50%.
***
Data from Kostjukovits et al.73
APPs, acute-phase proteins; APRs, acute-phase reactants; CR, complete response; FMF, familial Mediterranean fever; HIDS, hyperimmunoglobulinemia D syndrome; MKD, mevalonate kinase deficiency; NA, not available; OD, on demand; PR, partial response; TRAPS, tumor-necrosis-factor-receptor-associated periodic syndrome; uAIDs, undifferentiated autoinflammatory diseases.