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Table 1 Reports of C3G patients combined with monoclonal gammopathy in the literature

From: Monoclonal immunoglobulin mediates complement activation in monoclonal gammopathy associated-C3 glomerulonephritis

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

  1. Abbreviations: C3G C3 glomerulopathy, C3NeF C3 nephritic factor, CAP Complement alternative pathway, CFH Complement factor H, CLL Chronic lymphocytic leukaemia, FHAA Factor H autoantibody, LC Light chain, MGRS Monoclonal gammopathy of renal significance, MGUS Monoclonal gammopathy of undetermined significance, MIg Monoclonal immunoglobulin, MM Multiple myeloma, MPGN Membranoproliferative glomerulonephritis, ESRD End-stage renal disease, SCT Stem cell transplantation, Dex High-dose dexamethasone, Mel Melphalan, CYC Cyclophosphamide, PEX Plasma exchange, MMF Mofetil mycophenolate, NA Not available
  2. 1Evaluation was not performed in the remaining 9 patients
  3. 2Variants/mutations of unknown C3G pathogenicity, including APCS, C1QA, F5, DGK, FCN1, and PLG