[CIS PIDD] [cis-pidd] IgG2 subclass deficiency with recurrent infections

[Stan Ress]

Dear Colleagues,
 
I've been referred a 54 year-old lady for evaluation of Ig deficiency.
She gave a history of recurrent sore throats & especially sinus
infections during childhood, with repeated courses of antibiotics. These
continued into adulthood, and in her 20's she had 2 episodes of viral
meningitis. She gives a history of "encephalitis" with fever and was
hospitalized twice for this diagnosis, in 2010 & 2012. She was treated
with IV antibiotics, but says it was "felt to be viral".  Since
September 2010 here have been recurrent sore throats, sore ears, and
sinusitis treated with antibiotics. She has been under an ENT & a sinus
scan was apparently clear. In June 2012 serum Ig results showed normal
IgA, IgM 0.45 (0.5-2.5 G/L), IgG 6.89 G/L (7-16 G/L) IgG1 4.66
(4.05-10.11) IgG2 1.24 (1.69 -7.86) IgG3 0.12 (0.11 - 0.85) IgG4 0.09
(0.03-2.01). She was given intramuscular Ig for 2 months. In August 2012
there was an apparent "PUO" and after oral antibiotics she was treated
with IV antibiotics for 10 days. In January 2013 IgG2 was again
subnormal 1.53 G/L. Flow cytometric absolute counts: CD19 B-cells 346
(200-400), CD3 1835 (11-1700), CD4 1350 (700-1100), CD8 490 (500 -900),
CD4/8 ratio 2.76, NK cells 196 (200-400). C3 normal, C4 0.19 (0.2 -0.5).
CT chest - NAD.
 
She was given a course of IVIG in October 2012 and again on 7 February
2013. After this in February 2013 she had 3 courses of antibiotics for
recurrent sinusitis. In January 2013 while off IVIG, IgG ELIZA baseline
vaccination status was determined: Tetanus toxoid was 0.66 (sufficient
protection), H. Influenza  was 0.57 (>1.5 for adults), Strept. Pneumonia
was low at 32.82.
 
I first saw the patient & repeated her Ig levels in April 2013, 2
months after her last IVIG injection. IgG 7.42, IgM 0.66, IgG subclasses
again isolated reduction in IgG2 1.58 (1.69 -7.86). Repeat of
pneumococcal IgG ELIZA 3 weeks after pneumovax 24 vaccine, was >270
indicative of a good response (although we are unable to measure
individual pneumococcal serotypes in our setting). I will also give her
H. Influenza vaccine, it is currently out of stock. 
 
She may be evolving into CVH & the plan will be to monitor & carefully
document all infections, with isolation of organisms if possible. Most
of her infections seem to be viral without an acute phase response
(raised CRP, etc). 
 
I would appreciate advise regarding the following questions:
 
1. She travels extensively for her work and is about to embark on a 7
week trip to Morocco, Japan & USA. Aside from normal hygiene measures,
she asks what protective measures she could take for this trip? Would
there be a role for empirical IM immunoglobulin prior to departure &
perhaps again after 4 weeks while abroad? Any role for prophylactic
antibiotics?
 
2. Given her clinical picture, and the reduced IgG2 with total IgG
either reduced or on lower limit of normal, is there an indication for
Ig replacement? (perhaps SC weekly "push" IG would be easier to motivate
for insurance cover, than monthly IVIG). It's difficult because of her
good response to pneumovax 24, although of course this conclusion is
tempered by lack of information on individual serotypes, and the
response to H. Influenza still needs to be done . 
 
Any advice on these questions & further investigation and/or management
of the patient would be appreciated.
 
Thanks & Regards,
 
Stan Ress
 
 
--
Stanley Ress
Associate Professor of Medicine
Head: Division of Clinical Immunology
Department of Medicine
H47 Old Main Building-room 26
Groote Schuur Hospital and UCT
Observatory 7925
Cape Town
South Africa
TEL:INTERN. + 2721-4066201 or 4066197
FAX:   "    + 2721-4486815
Cell: 0833115482 
email: stan.ress at uct.ac.za




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