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

[Keller, Michael D]

Hi Stan,

Innate immunodeficiencies are rare but could explain severe invasive viral infections.  Do you have the ability to look at her NK function?

Best regards,
Mike
________________________________________
From: Stan Ress [Stan.Ress at uct.ac.za]
Sent: Monday, April 29, 2013 5:15 PM
To: CIS-PIDD
Subject: [cis-pidd] IgG2 subclass deficiency with recurrent infections

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<mailto:stan.ress at uct.ac.za>

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