[CIS PIDD] [cis-pidd] 40 year-old lady with recurrent severe bacterial infections, selective IgG deficiency, & discordant polysaccheride responses

[Stan Ress]

Colleagues,

I was consulted about a 40 year-old female patient with Asthma, currently well controlled on Singulair 10mgs daily, Foxair accuhaler 50/500 bd and Ventolin prn.  Her last exacerbation was in May, 2013 associated with a community acquired pneumonia. She has chronic allergic rhinitis complicated by recurrent sinusitis and postnasal drip, &  has had previous upper airway endoscopy and functional endoscopic sinus surgery.  Other chronic problems include severe persistent reflux, controlled on Nexiam 40mgs bd, Hypothyroidism on Eltroxin thyroid replacement, and Addison’s following severe sepsis with DIC and intra-adrenal haemorrhage bilaterally. She is currently on 10mg hydrocortisone replacement therapy. She also has chronic eczema on topical corticosteroids that is complicated by recurrent cellulitis, requiring antibiotics.

She has been under the care of a pulmonologist in another city who reports that she is prone to recurrent severe bacterial infections complicated by bacteraemia septic shock and, on occasion, multi-organ failure.   The source of sepsis has been cellulitis and lower respiratory tract infections.   She, fortunately, has always responded to aggressive supportive care with ventilation, dialysis, inotropic support and appropriate antibiotics. She had been to see numerous specialists but no one had been able to pinpoint any specific immune deficiency.

Investigations that I suggested showed reduced IgG – 6.65 g/l (7-16) with associated reduction in  IgG2 +3 subclasses, normal  IgA of 0.97 (0.7-4.0) & IgM of 0.43 (0.4-2.3), normal C3 and C4, normal classical & alternative pathway, normal MBL, normal immune phenotyping (CD3, CD4, CD8, B-cells & NK cells). CD27+IgD- class switched memory B cells 14.3% (9.2-18.9), CD27+IgD+ non-class switched memory 11.8 (13.4-21.4). Initial baseline vaccination IgG ELIZA indicated low diphtheria & H. Influenza Abs. with normal Tetanus 0.23 (>0.10) & S. Pneumonia 44.2 (>35). Following vaccination with tetanus-conjugated H. influenza (no pure H. Influenza vaccine available) there was  a good response with increase in Tetanus Abs - 3.16 and H. Influenza Abs  >9.0.

I would greatly appreciate your thoughts regarding the following questions:


1.       Can the mild selective reduction in IgG account for the recurrent infections? I have no data regarding any organisms ever being isolated. She had received steroids in May which could also cause a reduction in  serum IgG as a confounding variable.

2.       How to interpret discordant IgG levels against H. Influenza & S. Pneumonia. While she responded to Tetanus-conjugated vaccination, what would the in-vivo situation be, would she be vulnerable to other polysaccharide bacteria?

3.       Given the chronicity & severity of her infections, is she a candidate for IVIG?

Thanks & Regards,

Stan
--
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-(0)865173095
Cell: 0833115482
email: stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>


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