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. 2020 Nov 11;40(7):2973–2977. doi: 10.1007/s10067-020-05501-w

Table 3.

Literature review of reported cases of Schnitzler’s Syndrome without monoclonal gammopathy and described case comparison

Author, yr Age (yrs) Sex Monoclonal gammopathy (quantity) (FU) Treatment(s) IL-1 blockade treatment(s) Outcome (timing) AEs
Gladue HS, 2014 58 M IgM k (20 months) CS, MTX,ETN, IFX

Anakinra

Canakinumab

Initial response

Severe site injection reaction (anakinra)

Death (ventricular tachycardia)

44 F No (N/A) Any

Anakinra

Canakinumab

Remission Severe site injection reaction (anakinra)
Husak R, 2000 57 M Total IgM elevation (6.5 g/L) (24 months) Antihistamine, CS, trofosfamide, AZA, colchicine, PEX, IFN-α No
Varella TCN, 2005 36 M Total IgM elevation (3.7 g/L) (N/A) Thalidomide,antihistamine, NSAIDs, IFN-α No
Urbanski M, 2016 62 M No (14 months) MTX, AZA, dapsone, ADA, colchicine Anakinra Remission (24 h) No
Mulla E, 2015 71 M Total IgM elevation (4.7 g/L) monoclonal IgM k (48 months) CS, colchicine Anakinra Remission (days) No
Ahn MJ, 2018 69 M No (3 months) ETN, antihistamine, CS, colchicine, CsA, dapsone, MTX Anakinra Remission (24 h) Mild site injection reaction
described case 21 F Total IgM elevation (2,57 g/L) (24 months) NSAIDs, CS, CsA, omalizumab Anakinra Remission (48 h) Neutropenia

yrs. years, CsA cyclosporine, MTX methotrexate, AE adverse events, CS corticosteroids, NSAIDs non steroidal anti-inflammatory drugs, ETN etanercept, ADA adalimumab, AZA azathioprine, PEX plasma exchange, IFX infliximab, IFN-α interferon alfa, FU follow-up, N/A not available