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