Table 1.
Clinical and pathologic findings in PCDH7-associated MN (Mayo Clinic cohort patients 1–10 and Belgian cohort patients 10–14)
| Patient | Age, yr | Sex | Urinary Protein, g/24 h | Serum Cr, mg/dl | Urinary Protein, g/24 h | Serum Cr, mg/dl | Sclerosed/Total Glomeruli | IFTA, % | IF | IgG | EM |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 73 | M | 3.2 | 1.1 | 1.3 (36 mo) | 1.2 (36 mo) | 0/12 | 0 | IgG 3+; C1q 1+; C3 ± | IgG1 2+; IgG3 1+; IgG4 1+ | II |
| 2 | 66 | M | 9.6 | 1.3 | 6 (36 mo) | 1.0 (36 mo) | 3/16 | 10 | IgG 2+ | IgG1 1+; IgG4 2+ | II, TRI rare |
| 3 | 68 | M | NA | 1.1 | NA | NA | 2/18 | 10 | IgG 2+ | IgG4 2+ | II |
| 4 | 59 | W | 3 | 1.1 | 1.3 (6 mo) | 1.5 (6 mo) | 21/51 | 25 | IgG 2+; C3 ± to 1+ | IgG1 1+; IgG2 2+; IgG3 2+; IgG4 3+ | II, TRI |
| 5 | 61 | M | 7 | 1.9 | Recent diagnosis (<6 mo) | Recent diagnosis (<6 mo) | 3/11 | 10 | IgG 3+; C1q 1+; C3 1+ | IgG1 2+; IgG3 2+ | II |
| 6 | 38 | M | 3 | 1 | 1 (6 mo) | 0.9 (6 mo) | 2/33 | 0 | IgG 1+; C1q/C3 ± | No glomeruli | II |
| 7 | 37 | M | 1.4 | 1.76 | Recent diagnosis (<6 mo) | Recent diagnosis (<6 mo) | 2/34 | 25 | IgG 3+; C3 ± | IgG4 1+ | III, IV |
| 8 | 67 | M | 4.3 | 1.2 | 1.3 (24 mo) | 1.4 (24 mo) | 19/22 | 30 | IgG 3+; C3 1+ | Not done | III |
| 9 | 75 | W | 7 | 1.0 | 1.4 (96 mo) | 1.3 (96 mo) | 2/13 | 10 | IgG 3+; C3 1+ | Not done | II |
| 10 | 70 | W | 1 | 1.6 | 1.4 (18 mo) | 1.6 (18 mo) | 7/30 | 15 | IgG 3+; C3 1+ | Not done | II |
| 11 | 64 | W | 8.4 | 1.2 | 0.9 (20 mo) | 0.9 (20 mo) | 3/15 | <25 | IgG 3+; C3 3+; C1q 1+ | IgG3 3+; IgG2 2+; IgG1/4 trace | I |
| 12 | 61 | M | 3.9 | 1.0 | 1.0 (19 mo) | 1.5 (19 mo) | 2/17 | <25 | IgG 3+ | IgG4 2+; IgG2 1+ | I |
| 13 | 66 | W | 23.3 | 3.8 | 0.7 (23 mo) | 1.8 (23 mo) | 1/6 | <25 | IgG 3+; IgA 2+; C3 3+ | IgG4 2+; IgG2 2+ | I |
| 14 | 72 | W | 21 | 1.3 | NA | NA | 2/5 | <25 | IgG 1+ | Not done | I |
Follow-up is in months. Cr, creatinine; IFTA, interstitial fibrosis and tubular atrophy; M, man; TRI, tubuloreticular inclusion; NA, not available; W, woman.