Table 5 Treatment administrated and clinical response in the described DADA2 patients (1-14)
From: Monogenic polyarteritis: the lesson of ADA2 deficiency
Therapy | Case report (number of treated patients) | Response to treatment |
|---|---|---|
Steroids (orally or i.v.) | Navon et al. (17) | In 2 cases complete control of the disease with on demand steroidal therapy. In other patients steroid-dependence. |
Zhou et al. (9) | Partial control of diseases manifestations with high doses of corticosteroids | |
Van Eyck et al. (2) | Steroid-dependence | |
Belot et al. (2) | Steroid-dependence | |
Garg et al. (1) | Steroid-dependence | |
Van Montfrans et al. (6) | Partial response in 3 patients | |
Schepp et al. (1) | Partial response | |
cyclophosphamide (orally or i.v.) | Navon et al. (9) | Good response in 2 patients. |
Zhou et al. (7) | Not specified | |
Belot et al. (2) | Partial response | |
Garg et al. (1) | Poor response | |
Batu et al. (4) | Poor response | |
Azathioprine | Navon et al. (7) | No patients with complete response |
Van Eyck et al. (2) | Poor response | |
Belot et al. (1) | Good response in association to methotrexate | |
Batu et al. (3) | Poor response | |
Van Montfrans et al. (5) | Not specified | |
Methotrexate | Navon et al. (3) | Good response in association with other immunosuppressive and biologics |
Belot et al. (1) | Good response in association with azathioprine | |
Batu et al. (3) | Poor response | |
Schepp et al. (1) | Partial response | |
Cyclosporine | Van Eyck et al. (1) | Poor response |
Colchicine | Batu et al. (5) | Good response in one patient, none response in 4 patients |
Mycophenolate | Zhou et al. (2) | Not specified |
Van Eyck et al. (1) | Poor response | |
Belot et al. (1) | Partial response in association with cyclophosphamide | |
Batu et al. (2) | Good response in one patient, poor in the other | |
Sirolimus | Van Eyck et al. (2) | Good response in one patient Poor response in one patient |
Tacrolimus | Van Eyck et al. (2) | Good response in one patient Poor response in one patient |
I.v. immunoglobulins | Navon et al. (1) | Not specified |
Zhou et al. (5) | Not specified | |
Van Eyck et al. (2) | Prophylactic dosage | |
Belot et al. (1) | Prophylactic dosage | |
Schepp et al. (2) | Prophylactic dosage | |
Anakinra | Zhou et al. (5) | Not specified |
Garg et al. (1) | Initial partial response than relapse | |
Van Montfrans et al. (1) | Good response | |
Canakinumab | Garg et al. (1) | Initial partial response than relapse |
Etanercept | Navon et al. (5) | Complete response in 5 patients Partial response in 1 patient |
Zhou et al. (6) | Not specified | |
van Montfrans et al. (3) | Partial response in 1 pateint Complete response in 2 patients | |
Batu et al. (3) | Partial response in 2 patients, complete in 1 | |
Adalimumab | Navon et al. (3) | Complete response in 2 patients, exacerbation in 1 patient |
Infliximab | Navon et al. (2) | Complete response in 1 patient Partial response in 1 patient |
Tocilizumab | Zhou et al. (1) | Not specified |
Van Eyck et al. (1) | Complete response | |
Batu et al. (1) | Poor response | |
Rituximab | Zhou et al. (1) | Poor response |
Belot et al. (1) | Poor response |