Authors | Year | Patients, n | Age | M/F, n/n | Serum MIg, n | Haematological diagnosis, n | Evaluation of factors affecting CAP, n | Electronic microscopy findings of renal biopsy, n | Treatment | Renal outcomes, n |
---|---|---|---|---|---|---|---|---|---|---|
Meri [19] | 1992 | 1 | 57 | 0/1 | λ LC: 1 | MGRS | λ LC dimer could act as a mini-autoantibody against CFH | Subendothelial and intramembranous dense deposits in the GBM (consistent with DDD) | NA | NA |
Jokiranta [20] | 1999 | |||||||||
Sethi [9] | 2010 | 10 | 60 (49–77) | 2/8 | IgGκ: 6 IgGλ: 3 IgA & IgGλ: 1 | No patient had progressed beyond MGUS | FHAA and CFH H402 allele: 11 | DDD: 10 | No specific therapy | ESRD: 4; chronic kidney failure: 2; recent diagnosis: 4 |
Bridoux [8] | 2011 | 6 | 67.5 (40–74) | 3/3 | IgGκ: 4 | MGUS: 5 | FHAA: 1 | EM (performed in 5 patients): nonextensiveamorphous electron-dense deposits, or deposits of intermediate density, with a “sausage-shaped” appearance, were observed within the lamina densa (consistent with DDD) | No treatment: 2; Dex: 1; Mel+ Dex: 1; CYC + Dex: 1; Bortezomib+Dex: 1 | ESRD: 5 (despite chemotherapy in 4 patients); chronic kidney failure: 1 |
IgGλ: 2 | Smouldering MM: 1 | CFH H402 allele: 2 | ||||||||
Zand [10] | 2013 | 10 | 61.5 (22–69) | 7/3 | IgGκ: 6 | MGUS:9 CLL: 1 | C3NeF: 2 | C3GN: 10 | Conservative: 4; Rituximab+CYC + vincristine+prednisone: 1; Dex + bortezomib: 1; immunosuppressive therapy: 4 | ESRD: 3; relatively stable renal function: 6; improved renal funcion: 1 |
IgGλ: 2 | CFH H402 allele: 3 | |||||||||
IgAλ: 1 | ||||||||||
IgMλ: 1 | ||||||||||
Lloyd [5] | 2016 | 10 | 63.5 (52–90) | 9/3 | IgGκ: 8 | MGRS: 5 | NA | DDD: 3; C3GN: 7 | Chemotherapy (including bortezomib+DEX): 4 patients with MM | ESRD: 5 (three patients with MM); relatively stable renal function: 3; improved renal funcion: 2 (one patient underwent SCT) |
IgGλ: 1 | MM: 4 | |||||||||
IgAκ: 1 | Polyclonal plasmacytosis: 1 | |||||||||
Yin [17] | 2016 | 1 | 64 | 0/1 | IgGλ: 1 | MM | NA | C3GN | Thalidomide+DEX | recent diagnosis |
Daccueil [14] | 2017 | 1 | 61 | 1/0 | IgGλ: 1 | MGRS | NA | DDD | Rituximab+steroids | Improved renal function. |
Hamzi [21] | 2017 | 1 | 32 | 1/0 | λ LC: 1 | MM | Not done | Electronic microscopy was not performed | Chemotherapy (CYC + DEX + thalidomide) → SCT | Improved renal function. |
Chauvet [12] | 2017 | 50 | 65 (38–82) | 33/17 | IgGκ: 36 | MGRS: 30 | C3NeF: 3; | Electron microscopy was available in 25 cases. DDD: 1; C3GN: 24 | Conservative: 13; immunosuppressive therapy: 8; clone-directed chemotherapy: 29 (including bortezomib in 22 patients) | ESRD: 25 (9 in the chemotherapy group) |
IgGλ: 11 | Smouldering MM: 15 | FHAA: 9 | ||||||||
IgAκ: 1 | Symptomatic MM: 2 | a rare variant of undetermined significance (p. D130N in CFH and p. E548Q in CFI): 2 | ||||||||
IgAλ: 1 | CLL: 3 | |||||||||
λ LC only: 1 | ||||||||||
Timmermans [15] | 2018 | 2 | 75.5 (75–76) | 1/1 | IgGκ: 1 | MGUS: 2 | C3NeF and FHAA were not found | DDD: 1; C3GN: 1 | Conservative: 2 | Relatively stable renal function: 1; chronic kidney failure: 1 |
IgGλ: 1 | ||||||||||
Chauvet [13] | 2018 | 41 | 63 (36–83) | 25/16 | IgGκ: 27 | MGRS: 28 | C3NeF: 3; | Electron microscopy was available in 25 cases. DDD: 1; C3GN: 24 | NA | NA |
IgGλ: 11 | other (MM, CLL): 13 | FHAA: 9; | ||||||||
IgAκ: 2 | FIAA: 2; | |||||||||
κ LC only: 1 | Anti-CR1 Abs: 11 | |||||||||
a rare variant of undetermined significance (p. D130N in CFH and p. E548Q in CFI): 2 | ||||||||||
Ravindran [11] | 2018 | 36 | 60 (20–85) | 25/11 | IgG: 31 | MGRS: 26 | C3NeF: 11/24; | DDD: 4; C3GN: 32 | Conservative: 3; non-targeted therapy: 17; MIg-targeted therapy: 16 | Complete or partial renal response or stable renal function: 44% of patient with MIg-targeted therapy and 41% of patients with non-targeted therapy. |
IgM: 3 | Smouldering MM: 2 | FHAA: 2/24; | ||||||||
IgA: 1 | Symptomatic MM: 5 | FBAA: 1/24; | ||||||||
κ: 26 | CLL: 1 | CFH H402 and/or V62 allele: 13/21 | ||||||||
λ: 10 | Cryoglobulinemia (type 1): 1 | other variants/mutations of unknown C3G pathogenicity: 4/212 | ||||||||
Cryoglobulinemia (type 2): 1 | ||||||||||
Hirashio [22] | 2018 | 1 | 52 | 1/0 | λ LC: 1 | MGRS | FHAA | DDD | Bortezomib+DEX | Improved renal function and histological resolution of DDD. |
Ramirez [23] | 2018 | 1 | 70 | 1/0 | IgGκ: 1 | MGUS | Not done | NA | Intravenous methylprednisolone→PEX → MMF | Improved renal function |
Lepori [24] | 2018 | 1 | 55 | 1/0 | IgGκ: 1 | MGRS | Not done | C3GN | Mel→SCT | Relatively stable renal function |
Moog [25] | 2018 | 1 | 59 | 1/0 | IgGλ: 1 | Smouldering MM | Not done | DDD | Eculizumab, chemotherapy (bortezomib+DEX), PEX, immunosuppressive therapy | Relatively stable renal function |