Table 5.

Outcomes after plasma treatment in aHUS patients with mutations in CFH, CFI, C3, THBD, MCP, or CFH autoantibodies and in patients without mutations

MutationCFHCFIC3THBDMCPCFH AntibodiesNone
Plasma treated episodes90 (52 patients)8 (7 patients)14 (10 patients)8 (6 patients)29 (14 patients)12 (7 patients)103 (84 patients)
    Remission57 (63%)2 (25%)a8 (57%)a7 (88%)28 (97%)b9 (75%)71 (69%)a
        Complete remission5 (5%)1 (12.5%)a6 (43%)a,b5 (62%)b26 (90%)b3 (25%)30 (29%)a,b
        Partial remission52 (58%)1 (12.5%)2 (14%)b2 (25%)2 (7%)b6 (50%)a41 (40%)a
    ESRF–death33 (37%)6 (75%)a6 (43%)a1 (13%)1 (3%)b3 (25%)32 (31%)a
        ESRF25 (28%)6 (75%)a6 (43%)a1 (3%)3 (25%)31 (30%)
        Death8 (9%)1 (13%)1 (1%)
  • Complete remission is defined as normalization of both hematologic parameters and of renal function (see Table 3). Partial remission is defined as normalization of hematologic parameters with renal sequelae (see Table 3). In this table, we included only the episodes for which information about plasma treatment was available. CFH group includes two patients with CFH mutations and CFH autoantibodies and three patients with the CFH/CFHR-1 hybrid gene.

  • a P < 0.0024 after Bonferroni correction compared with the group with MCP mutations.

  • b P < 0.0024 after Bonferroni correction compared with the group with CFH mutations.