| Anakinra (Kineret) | Competitive binding to IL-1R1 thus inhibiting action of IL-1α and IL-1β | Adult: 100 mg/d Children: 1-2 mg/kg/d Variable: 0.5-2 mg/kg/d are sufficient to suppress disease activity in FCAS patients, but 6-8 mg/kg/d may be needed to treat CINCA/NOMIDa* Half-life: 4-6 h | Injection site reactions, infections, neutropenia, malignancies, hypersensitivity, immunogenicity | Before treatment initiation exclusion of latent tuberculosis, hepatitis, or other infectious diseases | CAPS Schnitzler DIRA FMF MKD/HIDS PAPA SAPHO DITRA TRAPS HA20 BS/EOS Majeed | IIA1, IV2,3 IV4 IV5 IB6, IV7 IV8 V9 V10 V11 IV12 V13 V14 V15 |
| Rilonacept (Arcalyst) | Soluble decoy receptor that binds IL-1β > IL-1α > IL-1Ra | Adult: 160 mg/wk Children: 2.2 mg/kg/wk Mean half-life: 8.6 days | Injection site reactions, infections, neutropenia, immunogenicity, reduced lipid level | Exclusion of serious infections before treatment initiation | CAPSa Schnitzler DIRA FMF | IB16 IV17 IV18 IB19 |
| Canakinumab (Ilaris) | Human monoclonal antibody specific for IL-1β | Adult: 150 mg/8 wk Children: 2 mg/kg/8 wk Mean half-life: 26 d | Injection site reactions, infections, vertigo, hypersensitivity, immunogenicity | Exclusion of serious infections before treatment initiation | CAPSa Schnitzler FMFa MKD/HIDSa TRAPSa BS/EOS Majeed | IB20, IV21 IB22 IB23 IB23 IB23 V24 V25 |
| Colchicine | Multiple mechanisms of action (prevention of microtubule assembly) | Adult: 1.2-2.4 mg/d Children: 0.5-1.5 mg/d | Elderly patients and those with renal impairment treated with colchicine may develop myopathy or neuropathy | None | FMFa HA20 | V26 V13 |
| TNF-α inhibitors | Monoclonal antibody/Soluble decoy receptor that binds TNF-α | Etanercept: 50 mg/wk Adalimumab: 40 mg/2 wk Infliximab: 3-5 mg/2-8 wk No published data for other TNF inhibitors | Injection site reactions, infusion-related reactions, infections, autoimmunity, malignancies, heart failure, hepatotoxicity | Tuberculosis and infections during treatment, exclusion of latent tuberculosis before treatment | FMF PAPA TRAPS HA20 ORAS BS/EOS | V27 V28 III29 V30 V31 V32 |
| Tocilizumab (Actemra) | Competitive inhibition of binding of IL-6 to IL-6 receptor | Adult/children: 4–8 mg/kg/4 wk | Infusion reactions, infections, gastrointestinal perforations, anaphylaxis, neutropenia, thrombocytopenia, elevated liver enzymes and lipids, malignancies, immunogenicity | Tuberculosis and infections during treatment, exclusion of latent tuberculosis before treatment | Schnitzler TRAPS | IV33 V34 |
| Ustekinumab (Stelara) | Monoclonal antibody that binds p40 subunit of IL-12 and IL-23, thus inhibiting interaction with IL-12Rβ1 | ...