Main demographic, clinical, and biological data at time of kidney biopsy and at onset of treatment
| . | All patients (N = 50) . | Chemotherapy (N = 29) . | IS therapy (N = 8) . | Conservative treatment (N = 13) . | P value . |
|---|---|---|---|---|---|
| Age, y | 65 (38-82) | 69 (36-83) | 64 (57-70) | 60 (40-77) | .58 |
| Male, n (%) | 33 (66%) | 18 (62%) | 5 (75%) | 10 (77%) | .62 |
| Renal manifestations at diagnosis | |||||
| Serum creatinine, µmol/L | 158 (68-990) | 158 (68-990) | 168 (135-280) | 150 (70-317) | .59 |
| eGFR, ml/min/1.73 m2 | 37 (3-100) | 37 (3-94) | 32 (20-49) | 39 (16-100) | .51 |
| CKD stage 1-2 | 8/49 (16%) | 10/28 (36%) | 0 (0%) | 2 (15%) | .07 |
| CKD stage 3 | 15/49 (31%) | 5 /28 (18%) | 3 (37%) | 7 (54%) | .06 |
| CKD stage 4-5 | 27/49 (55%) | 13/28 (46%) | 5 (63%) | 4 (31%) | .35 |
| Nephrotic syndrome, n (%) | 20/46 (43%) | 14/22 (63%) | 3 (38%) | 3/11 (27%) | .11 |
| 24-h proteinuria, g | 3.15 (0.1-14) | 4 (0.7-14) | 1.7 (0.4-10) | 1.8 (0.1-10) | .06 |
| Hematuria, n (%) | 33/39 (84%) | 19/24 (79%) | 6/6 (100%) | 8/9 (89%) | .41 |
| Renal manifestations at onset of treatment | |||||
| Serum creatinine, mmol/L | — | 202 (68-990) | 198 (70-450) | — | .47 |
| eGFR, ml/min/1.73 m2 | — | 28 (4-100) | 29 (11-100) | — | .32 |
| CKD stage 1-2 | — | 6 (21%) | 0 (0%) | — | .71 |
| CKD stage 3 | — | 9 (31%) | 1 (13%) | — | .76 |
| CKD stage 4-5 | — | 14 (48%) | 7 (87%) | — | 1 |
| Nephrotic syndrome, n (%) | — | 17 (58%) | 5 (65%) | — | .37 |
| 24-h proteinuria, g | — | 3.55 (0.7-13) | 2.91 (0.6-10) | — | .06 |
| Hematological findings | |||||
| Serum monoclonal Ig, n (%) | |||||
| IgG κ | 36 (71%) | 24 (80%) | 5 (67%) | 7 (54%) | .12 |
| IgG λ | 11 (22%) | 3 (10%) | 3 (38%) | 5 (38%) | .06 |
| IgA κ | 1 (2%) | 0 (0%) | 0 (0%) | 1 (8%) | 1 |
| IgA λ | 1 (2%) | 1 (3%) | 0 (0%) | 0 (0%) | 1 |
| λ LC only | 1 (2%) | 1 (3%) | 0 (0%) | 0 (0%) | 1 |
| Abnormal FLC, n (%) | 17/32 (53%) | 12/22 (55%) | 2/3 (67%) | 3/7 (43%) | .76 |
| dFLC, mg/L | 79 (2-20800) | 79 (6-20800) | 114 (15-959) | 22 (2-820) | .55 |
| MIg concentration, g/L | 10.2 (2-38) | 11 (2-38) | 6 (2-15) | 9 (3-30) | |
| Underlying hematological disorder, n (%) | |||||
| MGRS | 30 (60%) | 12 (41%) | 7 (88%) | 11 (84%) | .006 |
| Smoldering MM | 15 (30%) | 13 (43%) | 1 (14%) | 1 (8%) | .02 |
| Symptomatic MM | 2 (4%) | 2 (7%) | 0 (0%) | 0 (0%) | .47 |
| CLL | 3 (6%) | 2 (7%) | 0 (0%) | 1 (8%) | 1 |
| Extra renal manifestations, n (%) | 5 (10%) | 5 (17%)* | 0 (0%) | 0 (0%) | .13 |
| Biological thrombotic microangiopathy, n (%) | 3 (6%) | 3 (10%) | 0 (0%) | 0 (0%) | .31 |
| Immunological findings | |||||
| C3 level, mg/L | 746 (78-1130) | 689 (78-1220) | 866 (410-1120) | 682 (473-1130) | .95 |
| Low C3 level, n (%) | 22 (43%) | 14 (48%) | 3 (38%) | 7 (46%) | .76 |
| C4 level, mg/L | 250 (104-575) | 243 (109-575) | 265 (193-321) | 282 (104-376) | .82 |
| sC5b-9, ng/mL | 734 (164-2370) | 961 (164-2370) | 705 (269-1035) | 535 (266-960) | .17 |
| Elevated sC5b-9, upper 420 ng/mL | 27/34 (79%) | 15/18 (83%) | 6/7 (86%) | 6/9 (67%) | .42 |
| C3Nef, n (%) | 3 (6%) | 1 (3%) | 1 (14%) | 1 (8%) | .6 |
| Anti-FH antibody, n (%) | 9 (19%) | 4 (14%) | 3 (38%) | 2 (15%) | .29 |
| Pattern of glomerular disease by electron microscopy | |||||
| C3GN | 24/25 (96%) | 18/19 (95%) | — | 6/6 (100%) | 1 |
| DDD | 1/25 (4%) | 1/19 (5%) | — | 0 (0%) | 1 |
| . | All patients (N = 50) . | Chemotherapy (N = 29) . | IS therapy (N = 8) . | Conservative treatment (N = 13) . | P value . |
|---|---|---|---|---|---|
| Age, y | 65 (38-82) | 69 (36-83) | 64 (57-70) | 60 (40-77) | .58 |
| Male, n (%) | 33 (66%) | 18 (62%) | 5 (75%) | 10 (77%) | .62 |
| Renal manifestations at diagnosis | |||||
| Serum creatinine, µmol/L | 158 (68-990) | 158 (68-990) | 168 (135-280) | 150 (70-317) | .59 |
| eGFR, ml/min/1.73 m2 | 37 (3-100) | 37 (3-94) | 32 (20-49) | 39 (16-100) | .51 |
| CKD stage 1-2 | 8/49 (16%) | 10/28 (36%) | 0 (0%) | 2 (15%) | .07 |
| CKD stage 3 | 15/49 (31%) | 5 /28 (18%) | 3 (37%) | 7 (54%) | .06 |
| CKD stage 4-5 | 27/49 (55%) | 13/28 (46%) | 5 (63%) | 4 (31%) | .35 |
| Nephrotic syndrome, n (%) | 20/46 (43%) | 14/22 (63%) | 3 (38%) | 3/11 (27%) | .11 |
| 24-h proteinuria, g | 3.15 (0.1-14) | 4 (0.7-14) | 1.7 (0.4-10) | 1.8 (0.1-10) | .06 |
| Hematuria, n (%) | 33/39 (84%) | 19/24 (79%) | 6/6 (100%) | 8/9 (89%) | .41 |
| Renal manifestations at onset of treatment | |||||
| Serum creatinine, mmol/L | — | 202 (68-990) | 198 (70-450) | — | .47 |
| eGFR, ml/min/1.73 m2 | — | 28 (4-100) | 29 (11-100) | — | .32 |
| CKD stage 1-2 | — | 6 (21%) | 0 (0%) | — | .71 |
| CKD stage 3 | — | 9 (31%) | 1 (13%) | — | .76 |
| CKD stage 4-5 | — | 14 (48%) | 7 (87%) | — | 1 |
| Nephrotic syndrome, n (%) | — | 17 (58%) | 5 (65%) | — | .37 |
| 24-h proteinuria, g | — | 3.55 (0.7-13) | 2.91 (0.6-10) | — | .06 |
| Hematological findings | |||||
| Serum monoclonal Ig, n (%) | |||||
| IgG κ | 36 (71%) | 24 (80%) | 5 (67%) | 7 (54%) | .12 |
| IgG λ | 11 (22%) | 3 (10%) | 3 (38%) | 5 (38%) | .06 |
| IgA κ | 1 (2%) | 0 (0%) | 0 (0%) | 1 (8%) | 1 |
| IgA λ | 1 (2%) | 1 (3%) | 0 (0%) | 0 (0%) | 1 |
| λ LC only | 1 (2%) | 1 (3%) | 0 (0%) | 0 (0%) | 1 |
| Abnormal FLC, n (%) | 17/32 (53%) | 12/22 (55%) | 2/3 (67%) | 3/7 (43%) | .76 |
| dFLC, mg/L | 79 (2-20800) | 79 (6-20800) | 114 (15-959) | 22 (2-820) | .55 |
| MIg concentration, g/L | 10.2 (2-38) | 11 (2-38) | 6 (2-15) | 9 (3-30) | |
| Underlying hematological disorder, n (%) | |||||
| MGRS | 30 (60%) | 12 (41%) | 7 (88%) | 11 (84%) | .006 |
| Smoldering MM | 15 (30%) | 13 (43%) | 1 (14%) | 1 (8%) | .02 |
| Symptomatic MM | 2 (4%) | 2 (7%) | 0 (0%) | 0 (0%) | .47 |
| CLL | 3 (6%) | 2 (7%) | 0 (0%) | 1 (8%) | 1 |
| Extra renal manifestations, n (%) | 5 (10%) | 5 (17%)* | 0 (0%) | 0 (0%) | .13 |
| Biological thrombotic microangiopathy, n (%) | 3 (6%) | 3 (10%) | 0 (0%) | 0 (0%) | .31 |
| Immunological findings | |||||
| C3 level, mg/L | 746 (78-1130) | 689 (78-1220) | 866 (410-1120) | 682 (473-1130) | .95 |
| Low C3 level, n (%) | 22 (43%) | 14 (48%) | 3 (38%) | 7 (46%) | .76 |
| C4 level, mg/L | 250 (104-575) | 243 (109-575) | 265 (193-321) | 282 (104-376) | .82 |
| sC5b-9, ng/mL | 734 (164-2370) | 961 (164-2370) | 705 (269-1035) | 535 (266-960) | .17 |
| Elevated sC5b-9, upper 420 ng/mL | 27/34 (79%) | 15/18 (83%) | 6/7 (86%) | 6/9 (67%) | .42 |
| C3Nef, n (%) | 3 (6%) | 1 (3%) | 1 (14%) | 1 (8%) | .6 |
| Anti-FH antibody, n (%) | 9 (19%) | 4 (14%) | 3 (38%) | 2 (15%) | .29 |
| Pattern of glomerular disease by electron microscopy | |||||
| C3GN | 24/25 (96%) | 18/19 (95%) | — | 6/6 (100%) | 1 |
| DDD | 1/25 (4%) | 1/19 (5%) | — | 0 (0%) | 1 |
Values for continuous variables are expressed as median [range], and values for categorical variables, as percentage. Statistical analysis: for contingency, chi2 test, for comparison, Kruskal-Wallis test.
C3Nef, C3 nephritic factor; IS, immunosuppressive.
Diffuse mucinosis (n = 1), digital ischemia (n = 2), skin purpuric lesions (n = 1), and capillary leak syndrome (n = 1).