Table 3.
Clinical characteristics of 11 cases of TAFRO syndrome with kidney biopsy
| Case no./Ref. | Age/sex | UP | Alb (g/dL) | Cr (mg/dL) | CRP (mg/dL) | VEGF/IL-6 (pg/mL) | 1st Therapy/ response | 2nd Therapy/ response | Kidney biopsy findings | |||||||||
| Thrombi | Endothelial swelling | Endocapillary proliferation | Mesangiolysis | Mesangial proliferation | Double contours of GBM | Glomerular Ig deposition by IF | Subendothelial lesions by EM | Electron dense deposits | Diagnosis by authors | |||||||||
| 1/ [4] | 38 / M | n.d. | 3 | 2.59 | 11.18 | 4420 / n.d. | PSL / good | - | n.d. | n.d. | n.d. | n.d. | Yes | Yes | Yes | n.d. | n.d. | MPGN-like |
| 2/ [5] | 55 / F | 0.54 (g/g・Cr) | 2.7 | 2.1 | 4.1 | 464 / 11.7 | Pulse steroid / good | - | n.d. | n.d. | n.d. | n.d. | Yes | Yes | Yes | n.d. | No | MPGN-like |
| 3/ [6] | 76 / F | 0.30 (g/day) | 1.2 | 3.02 | 16 | 1350 / 49.2 | PSL /good | - | n.d. | n.d. | Yes | n.d. | Yes | Yes | No | Yes | No | MPGN-like |
| 4/ [7] | 79 / F | 2.65 (g/g・Cr) | 2.4 | 1.85 | 3.9 | 15.6 / 3.76 | Pulse steroid, PE / bad | RTX / good | n.d. | n.d. | No | Yes | No | Yes | No | Yes | n.d. | TMA-like |
| 5/ [8] | 70 / M | 0.33 (g/day) | 2.5 | 1.28 | 9.85 | 126 / 33 | Pulse steroid / good | - | No | Yes | No | Yes | No | Yes | No | Yes | No | MPGN-like |
| 6/ [9] | 61 / F | n.d. | 2.8 | 2.14 | 23.12 | n.d. / 722.6 | Pulse steroid, TCZ, RTX / good | - | n.d. | Yes | n.d. | Yes | Yes | Yes | No | n.d. | n.d. | MPGN-like |
| 7/ [10] | 80 / F | 0.41 (g/day) | 2.8 | 1.17 | 7.3 | 454 / 21.6 | PSL /bad | TCZ / good | No | Yes | Yes | No | No | Yes | Yes | Yes | No | TMA-like |
| 8/ [11] | 51 / F | 0.52 (g/g・Cr) | 2.5 | 1.03 | 4.58 | 198 / 21.2 | Pulse steroid / good | - | n.d. | Yes | No | Yes | No | Yes | No | n.d. | n.d. | TMA-like |
| 9/ [12] | 84 / M | 0.30 (g/day) | 2.4 | 2.31 | 8.3 | 177 / 12.3 | PSL, PE /bad | TCZ / good | No | Yes | Yes | Yes | No | No | No | Yes | No | TMA-like |
| 10/ [13] | 54 / F | 3.2 (g/g・Cr) | 2.4 | 1.11 | 7 | n.d. / 8.2 | PSL /good | - | No | Yes | No | No | No | No | No | Yes | No | Endothelial injury |
| our case | 48 / F | 1.57 (g/g・Cr) | 2.6 | 1.32 | 18.33 | 494 / 166 | Pulse steroid /bad | TCZ / good | No | Yes | Yes | Yes | No | Yes | No | Yes | No | TMA-like |
Abbreviations: Alb Albumin; Cr Creatinine, CRP C-reactive protein, EM Electron microscopy, GBM Glomerular basement membrane, IF Immunofluorescence, Ig Immunoglobulin, IL Interleukin, MPGN Membranoproliferative glomerulonephritis, n.d. not data, PE Plasma exchange, PSL Prednisolone, RTX rituximab, TCZ tocilizumab, TMA Thrombotic microangiopathy, UP Urinary protein, VEGF Vascular endothelial growth factor