Table 2.
Evidence for treatment of Cogan's syndrome with biological agents.
| Biological agent | Immune mechanism | Number of CS patients reported to be treated with biotherapy | Age (years)/gender/clinical presentation | Biologic dosage and regimen | Number of CS patients that responded to biotherapy | Study design | Country of publication | Ref |
|---|---|---|---|---|---|---|---|---|
| Infliximab | Chimeric anti-TNF-α monoclonal antibody | 2 | 33/M/AVD, IK | 300 mg × 1/month | 2 | CR | Switzerland | [42] |
| 49/M/SNHL | 300 mg × 6/week | |||||||
| 3 | 30/F/AVD, IK | 3 mg/Kg at weeks 0, 2, 6, 8, and then every 8 weeks | 3 | CR | Italy | [40] | ||
| 29/M/AVD, IK | NA∗ | |||||||
| 35/M/SNHL, scleritis | NA | |||||||
| 2 | 37/F/AVD, IK | 3 mg/kg at 0, 2, and 6 weeks | 1 | CR | USA | [39] | ||
| 36/F/AVD, IK | 3 mg/kg for 4 months | |||||||
| 1 | 16/M/TINU, SNHL, BRAO, glaucoma, and uveitis | 900 mg at 0, 3, and 5 weeks | 1 | CR | USA | [41] | ||
| 1 | 48/F/AVD | 3 mg/kg at 0 and 3 weeks and then every 8 weeks | 1 | CR | Spain | [43] | ||
| 1 | 51/F/SNHL | 3 mg/kg every 8 weeks for 3 years∗∗ | 1 | CR | Israel | [6] | ||
| 1 | NA/NA/AVD, scleritis | NA | 1 | CR | Switzerland | [74] | ||
| 1 | 67/F/AVD | NA | 1 | CR | Greece | [45] | ||
|
| ||||||||
| Etanercept | TNF-α receptor fusion protein | 3 | NA | 25 mg × 2/week for 24 weeks | 2 | CR | USA | [46] |
|
| ||||||||
| Adalimumab | Anti-TNF-α monoclonal antibody | 1 | 69/M/SNHL, iritis, aortitis, meningitis, panniculitis, and seronegative arthritis | 40 mg × 1/week for 2 weeks | 0 | CR | Japan | [50] |
| 1 | 25/F/AVD, conjunctivitis, IK | 40 mg × 1/week for 6 months | 0 | CR | Italy | [48] | ||
|
| ||||||||
| Rituximab | Anti-CD20 monoclonal antibody | 1 | 25/F/AVD, conjunctivitis, IK | 500 mg × 1/week for 4 weeks | 1 | CR | Italy | [48] |
| 1 | 67/F/AVD | NA | 0 | CR | Greece | [45] | ||
| 1 | 43/F/AVD, IK | 375 mg/m2 × 1/week for 4 weeks | 0 | CR | USA | [49] | ||
|
| ||||||||
| Tocilizumab | Humanized anti-IL-6 receptor monoclonal antibody | 1 | 69/M/SNHL, iritis, aortitis, meningitis, panniculitis, and seronegative arthritis | 8 mg/kg × 1/month | 1 | CR | Japan | [50] |
| 1 | 59/M/SNHL, anterior uveitis | 162 mg × 1/week for 2 weeks | 0 | CR | USA | [47] | ||
CS: Cogan's syndrome; Ref: references; M: male; F: female; AVD: audiovestibulary dysfunction; SNHL: sensorineural hearing loss; IK: interstitial keratitis; TNF-α: tumor necrosis factor-α; CD: cluster of differentiation; IL: interleukin; CR: case report; NA: data is not available; TINU: tubulointerstitial nephritis and uveitis syndrome; BRAO: branch retinal artery occlusion. ∗Dosage is noted as given according to “international protocol.” ∗∗Unpublished data.