Table 2.
Nine patients with IgA-dominant MPGN pattern associated with cirrhosis
| Patients | Age (yr)/Sex | Concomitant or recent infection | Renal features |
Immunological findings |
Histological features |
Treatment | Outcomes/Follow-up | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ScR (mg/dL) | uPCR (g/g) | Hur | C3 g/l (0.9–1.8) | C4 g/l (0.1–0.4) | LM | IF |
||||||
| Mg | Cw | |||||||||||
| Pt. 1 | 71/M | No | 4 | 2.2 | + | 0.89 | 0.29 | MPGN | IgA +++ | IgA +++ | Steroids | Infectious arthritis leading to the discontinuation of steroids and MMF 2 months after initial KB. ScR 2.22 mg/dl and uPCR 2 g/g/5 mo. |
| ATN | C3 +++ | C3 +++ | MMF | Death (decompensated cirrhosis) | ||||||||
| Pt. 2 | 53/F | No | 3.75 | 3 | + | “normal” | 0.12 | MPGN | IgA + | IgA ++ | ACE inhibitor only | ScR 0.96 mg/dl and uPCR 0.4g/g /10 yr |
| ATN | C3 +++ | |||||||||||
| IgG,IgM traces | ||||||||||||
| Pt. 3 | 59/F | Yes, Staphylococcus aureus bacteraemia | 6.16 | 8 | + | 0.55 | 0.1 | MPGN | IgA +++ | IgA +++ | Steroids | ScR 1mg/dl/5 yr |
| Dialysis | Crescent (20%) | C3 +++ | C3 +++ | |||||||||
| Severe ATN | IgG,IgM, traces | IgG ++ | ||||||||||
| TIN (25%) | C1Q trace | |||||||||||
| Pt. 4 | 73/F | No | 5.47 | 7.68 | + | 1.11 | 0.22 | MPGN | None | IgA +++ | Steroids | Dialysis, death 1 mo after initial KB from sigmoiditis/1 mo |
| Dialysis | ATN | C3 ++ | ||||||||||
| Crescent (20%) | ||||||||||||
| Pt. 5 | 52/M | No | 7.96 | 2.98 | + | 0.9 | 0.24 | MPGN | IgA +++ | IgA +++ | Dual blockade of renin- angiotensin system | Dialysis, death 5.7 yr after KB from traffic accident |
| ATN | C3 ++ | C3 xyd++ | ||||||||||
| Pt. 6 | 66/M | No | 6.2 | 9.36 | +a | NA | NA | MPGN | IgA ++ | IgA ++ | Steroids 40mg/d | Dialysis, death 2 yr after KB from lung malignancy |
| Dialysis | ATN | C3 ++ | C3 ++ | |||||||||
| IgM trace | ||||||||||||
| Pt. 7 | 62/M | No | 1.2 | 8.97 | + | 1.25 | 0.28 | MPGN | IgA +++ | IgA +++ | Steroids 80mg/d | Death 4.5 mo from KB from Staphylococcus aureus bacteriemia, ScR 5.1 mg/dl |
| ATN | C3 ++ | C3 ++ | + | |||||||||
| ACE inhibitor | ||||||||||||
| Pt. 8 | 81/M | No | 6.25 | 4 | + | NA | NA | MPGN | IgA ++ | IgA ++ | Steroids | Dialysis, death 11.5 mo after KB from exacerbation of COPD |
| Crescent (30%) | C3+ | C3 + | ||||||||||
| ATN | ||||||||||||
| Pt. 9 | 70/M | No | 2.46 | 13 | + | 0.85 | 0.2 | MPGN | IgA +++ | IgA +++ | Steroids | Death 1.6 mo after KB from decompensated cirrhosis, last ScR 1.58 mg/dl, uPCR 4.6 g/g |
| C3 +++ | C3 +++ | + | ||||||||||
| IgM + | IgM + | ACE inhibitor | ||||||||||
ACE, angiotensin-converting enzyme; ATN, acute tubular necrosis; COPD, chronic obstructive pulmonary disease; Cw, capillary wall; F, female; Hur, hematuria; IF, immunofluorescence; KB, kidney biopsy; LM, light microscopy; M, male; Mg, mesangium; MMF, mycophenolate mofetil; MPGN, membranoproliferative glomerulonephritis; Pt., patient; ScR, serum creatinine; uPCR, urinary protein- to- creatinine ratio.
a
Macroscopic hematuria.