Background
Case presentation
Clinical history and initial laboratory data
Parameter | Value (reference range) | Parameter | Value (reference range) |
---|---|---|---|
Hematology | Urine BJP | negative | |
WBC count, /μL | 10,160 (3500–9100) | Immunology | |
Hemoglobin, g/dL | 12.6 (11.3–15.2) | IgG, mg/dL | 842 (820–1740) |
Platelet count, 104/μL | 20.5 (13.0–36.9) | IgG4, mg/dL | 28 (5–117) |
PT, % | 65.5 (80–100) | IgA, mg/dL | 166 (90–400) |
APTT, seconds | 40.7 (24–39) | IgM, mg/dL | 100 (52–270) |
FDP, μg/mL | 26 (< 5) | IgE, IU/mL | 23 (< 170) |
Blood chemistry | C3, mg/mL | 1.62 (0.86–1.6) | |
Cr, mg/dL | 1.32 (0.46–0.79) | C4, mg/mL | 0.35 (0.17–0.45) |
eGFR, mL/min/1.73m2 | 34.84 | ANA | negative |
SUN, mg/dL | 21 (9–20) | MPO-ANCA | negative |
Total protein, g/dL | 5.9 (6.7–8.3) | PR3-ANCA | negative |
Albumin, g/dL | 2.6 (3.8–5.1) | Anti-GBM antibody | negative |
AST, U/L | 19 (10–40) | Anti-SS-a/b antibody | negative |
ALT, U/L | 9 (5–45) | RF | negative |
LDH, U/L | 229 (120–240) | Cryoglobulin | negative |
ALP, U/L | 1845 (104–338) | sIL-2R, U/mL | 986 (122–496) |
γ-GTP, U/L | 569 (0–42) | Anti-CL IgG | negative |
T-Cho, mg/dL | 156 (150–219) | Anti-CLβ2GPI complex | negative |
TG, mg/dL | 159 (50–150) | ADAMTS13 activity, IU/mL | 0.633 (0.780~1.570) |
Glucose, mg/dL | 124 (75–110) | IL-6, pg/mL | 166 (< 8) |
HbA1c, % | 6.1 (4.6–6.2) | VEGF, pg/mL | 494 (< 38.3) |
CRP, mg/dL | 18.33 (0–0.29) | HBV surface antigen | negative |
Urinalysis | HCV antibody | negative | |
Urine dipstick protein | 3+ | HIV antibody | negative |
Urine occult blood | ± | HHV-8 DNA | negative |
Spot Urine PCR, g/g | 1.57 | T-SPOT assay | negative |
Kidney biopsy findings
Light microscopy
Immunofluorescence microscopy
Electron microscopy (Fig. 1c)
VEGF-A, CD34, and D2–40 staining
Control | Case | P-value | |
---|---|---|---|
VEGF-A positive area in glomeruli (%) | 1.6 ± 0.40 | 2.0 ± 0.55 | 0.63 |
VEGF-A positive area in cortex (%) | 0.32 ± 0.072 | 1.6 ± 0.31 | < 0.0001 |
CD34 positive area in glomeruli (%) | 22 ± 1.2 | 5.4 ± 0.48 | < 0.0001 |
CD34 positive area in cortex (%) | 7.1 ± 0.29 | 2.5 ± 0.23 | < 0.0001 |
D2–40 positive area in cortex (%) | 0.22 ± 0.091 | 0.072 ± 0.030 | 0.39 |
Clinical course
Discussion and conclusions
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 |