Table 1.
Characteristics of 134 new cases of SCLS reported between 2010–2016
| Age at diagnosis* | 48.3 years (newborn-85 years)± |
| Sex (female/male) | 71/63 (52.2%) |
| Identifiable precipitating trigger for attacks | 44% |
| MGUS+ | 68% (adults only) |
| M-spike concentration (g/L)*# | 3.5 (1–19) |
| Breakthrough episodes on theophylline-based regimen | 84% (47/56) |
| Median annual attack frequency on theophylline-based regimen | 2.25 (0–20) |
| Breakthrough episodes on IVIG/SCIG | 25% (18/73) |
| Median annual attack frequency on IVIG/SCIG | 0 (0–3.3) |
| Overall mortality rate | 14%& |
±
75th percentile = 49.8 years; 25th percentile = 16 years;
*
Median (range) of monoclonal paraprotein (M-spike) concentration by immunofixation electrophoresis;
#
(based on 41 cases excluding European registry [concentrations not reported]);
&
includes two non-SCLS related deaths.