Table 2.
Cases of Schnitzler-like syndrome with delayed or absent monoclonal gammopathy. Summary of published cases of a Schnitzler-like syndrome including the two cases reported in this case series that displayed delayed or absent monoclonal gammopathy.
| Cases reported in this case series | ||||
|---|---|---|---|---|
| Age | Sex | Hypergammaglobulinemia | Treatment with IL-1 blockade | Response |
| 47 | F | Delayed IgM κ after 5 years | Anakinra | + |
| 62 | F | Polyclonal IgA | Anakinra | + |
| Previously reported cases (table updated and modified, see reference 5 for full citation links)5 | ||||
| 58 | F | Polyclonal IgA and IgG | Anakinra | + |
| 54 | F | Polyclonal IgA | Anakinra | + |
| 36 | M | Polyclonal IgG | NAa | NA |
| 64 | M | Polyclonal IgA | Anakinra | + |
| 21 | F | Polyclonal IgM | Anakinra | + |
| 57 | M | Polyclonal IgM and IgE | NA | NA |
| 63 | M | Biclonal IgM κ, λ | NA | NA |
| 71 | M | Delayed IgM κ after | Anakinra | + |
| 58 | M | Delayed IgM after 20 months | Canakinumab | =b |
| 51 | M | Delayed IgM after 4 years | Anakinra | + |
| 44 | F | None | Anakinra & Canakinumab | + |
| 62 | M | None | Anakinra | + |
| 52 | F | None | Anakinra | + |
| 69 | M | None | Anakinra | + |
| 43 | F | None | Canakinumab | +8 |
| 75 | M | None | Anakinra | +7 |
a
NA refers to not available.
b
= equates to partial response