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Table 5 Efficacy of therapies tried in Schnitzler’s syndrome

From: Schnitzler’s syndrome: lessons from 281 cases

   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
  1. 1only against urticaria partially effective.
  2. 2only against bone pain partially effective.
  3. 3etanercept, adalimumab, and infliximab were tried.
  4. *exacerbation in one case.