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Table 1 Characteristics of patients with complement deficiency who developed invasive meningococcal disease in England

From: Invasive meningococcal disease in patients with complement deficiencies: a case series (2008–2017)

Case

Month + year of IMD

Age (years)

Detection method

Capsular group

PorA

Pen MIC

Pen

CtrA cycle number

ACWY Dose 1

ACWY Dose 2

4CMenB Dose 1

4CMenB Dose 2

Eculizumab

Underlying Condition

Antibiotic Prophylaxis

1

Jun 2011

9

Culture & PCR

Y

P1.5–1,10–1

0.125

S

20

    

No

Factor D

Penicillin after IMD

2a

May 2002

15

Culture & PCR

B

N

0.04

S

26

2011

   

No

C6 deficiency

No, rescue Amoxycillin

2b

Mar 2011

24

Culture & PCR

Y

P1.5–1,10–1

0.047

S

31

3a

Oct 1989

20

Culture only

Y

NTa

0.04

S

 

2008

   

No

C8 deficiency

Amoxycillin

3b

Nov 2011

42

Culture only

B

P1.5–2,10–1

0.047

S

 

4

Jun 2017

15

PCR only

NGb

No product

NA

NA

34

    

No

 

No

5a

Oct 2005

4

PCR only

B

ND

NA

NA

31

2016

   

No

C6 deficiency

No

5b

Mar 2018

16

Culture & PCR

Y

P1.5–1,10–1

0.064

S

36

6

Mar 2017

12

PCR

Y

P1.5–2,10–2

NA

NA

30

    

No

C7 deficiency

No

7

Mar 2013

17

Culture only

Y

P1.5–1,10–1

0.012

S

     

No

Hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis) on immunosuppression therapy (diagnosed in 2010)

No

8

Apr 2012

15

PCR only

Y

P1.5–1,10–1

NA

NA

28

2013

 

Oct 2014

Nov 2014

No

focal glomerulonephritis with persistently low complement C3/C4 (diagnosed in 2012)

Penicillin

9

Oct 2008

20

Culture & PCR

B

P1.17,16–3

0.047

S

34

Jul 2008

Dec 2011

Jul 2014

Aug 2014

Yes

PNH

Penicillin

10

Sep 2011

20

Culture only

B

P1.22,14

0.25

S

 

Aug 2011

 

Nov 2014

Jan 2015

Yes

PNH

Ciprofloxacin

11

Jan 2017

25

Culture only

Y

P1.18–1,3

0.094

S

 

May 2016

   

Yes

PNH

Penicillin

12

Oct 2009

40

Culture only

B

P1.22,14

0.064

S

     

Yes

PNH

No

13

Mar 2017

23

PCR only

W

P1.5,2

NA

NA

26

Dec 2016

   

Yes

PNH

Penicillin

14

Sep 2015

22

Culture only

NG (B) c

P1.22,14

0.50

R

 

Mar 2015

 

Apr 2015

May 2015

Yes (Mar 2015)

Renal

Penicillin

15

May 2016

20

Culture only

E

P1.5,2

0.094

S

 

Aug 2011

 

Feb 2015

Apr 2015

Yes (Oct 2013)

Renal

Not known

16a

Dec 2014

20

PCR only

NG (B)c

P1.7–1,1

NA

NA

CtrA not detected (but siaD B CT 30)

Apr 2014

 

Apr 2014

May 2014

Yes (Apr 2014)

Renal

Not known

16b

Mar 2017

22

PCR only

NG (B)c

P1.7–1,1

NA

NA

CtrA not detected (but siaD B CT 31)

  1. a NT. PorA phenotypically not typeable (before PorA sequencing was available); b NG. CtrA PCR positive, but negative for capsular groups A, B, C, W, & Y; c NG (B). Non-groupable because of interruption of ctrA gene but genotypically capsular group B;
  2. NA Not available, a culture is needed to determine the Penicillin MICs.; ND Not done