Table 5 Efficacy of therapies tried in Schnitzler’s syndrome
Efficacy % | Efficacy, number of cases | Reported | |||||
|---|---|---|---|---|---|---|---|
High | Partial | High | Partial | None | #cases | ||
Highly effective | anti-IL-1Ra (anakinra) | 94% | 2% | 81 | 2 | 3* | 86 |
anti-IL-1β antibodies (canakinumab) | 91% | 9% | 10 | 1 | 0 | 11 | |
anti-IL-6 antibodies (tocilizumab) | 75% | 25% | 3 | 1 | 0 | 4 | |
fusion protein IL-1R (rilonacept) | 50% | 38% | 4 | 3 | 1 | 8 | |
Moderately effective | anti-CD20 rituximab | 21% | 16% | 4 | 3 | 12 | 19 |
IFNα | 20% | 35% | 4 | 7 | 9 | 20 | |
corticosteroids | 18% | 46% | 33 | 86 | 66 | 185 | |
thalidomide | 19% | 25% | 3 | 4 | 9 | 16 | |
colchicine | 14% | 6% | 7 | 3 | 41 | 51 | |
pefloxacin | 13% | 63% | 2 | 10 | 4 | 16 | |
cyclosporin | 10% | 14% | 3 | 4 | 22 | 29 | |
Hardly effective | PUV-A1 | 8% | 62% | 1 | 8 | 4 | 13 |
alkylating agents | 7% | 20% | 4 | 12 | 44 | 60 | |
COX inhibitors | 6% | 33% | 6 | 31 | 57 | 94 | |
hydroxychloroquine | 7% | 7% | 1 | 1 | 13 | 15 | |
dapsone | 5% | 5% | 2 | 2 | 35 | 39 | |
histone deacetylase inhibitor (ITF2357) | 0% | 75% | 0 | 3 | 1 | 4 | |
doxepin | 0% | 50% | 0 | 3 | 3 | 6 | |
bisphosphonates2 | 0% | 33% | 0 | 3 | 6 | 9 | |
I.v. immunoglobulins | 0% | 25% | 0 | 2 | 6 | 8 | |
psoralen | 0% | 25% | 0 | 1 | 3 | 4 | |
UVB phototherapy | 0% | 25% | 0 | 1 | 3 | 4 | |
H1 antihistamine1 | 0% | 10% | 0 | 15 | 132 | 147 | |
plasmapheresis | 0% | 7% | 0 | 1 | 13 | 14 | |
e.c. immunoadsorption | 33% | 0% | 1 | 0 | 2 | 3 | |
bortezomib | 0% | 100% | 0 | 1 | 0 | 1 | |
dihydroergotamine | 0% | 100% | 0 | 1 | 0 | 1 | |
Not effective | azathioprine | 0% | 0% | 0 | 0 | 26 | 26 |
anti-TNF3 | 0% | 0% | 0 | 0 | 10* | 10 | |
chloroquine | 0% | 0% | 0 | 0 | 6 | 6 | |
sulfasalazine | 0% | 0% | 0 | 0 | 3 | 3 | |
fludarabine | 0% | 0% | 0 | 0 | 1 | 1 | |
UVA phototherapy | 0% | 0% | 0 | 0 | 1 | 1 | |
sulphones | 0% | 0% | 0 | 0 | 1 | 1 | |
leflunomide | 0% | 0% | 0 | 0 | 1 | 1 | |