[CIS-PAGID] Advice please

[Rohan Ameratunga (ADHB)]

Many thanks for the comments and advice.

He is on loratadine and nasal budesonide and undertakes daily nasal saline lavages.  I give my pts an ad hoc course of itraconazole in this situation but did not in this particular child.  

I agree with unsustained vaccine response being a possible marker of poor B cell memory, I did not measure it again in his case.

The other major improvement has been the drop in oral prednisone use from 6-8x per year to none.

thanks again

Rohan Ameratunga 
________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Richard Wasserman [drrichwasserman at gmail.com]
Sent: Saturday, May 19, 2012 3:44 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Advice please

The history you describe is, in my experience, very common. A couple of comments on your case:

 *   The response to H flu vaccine is only to the type b polysaccharide. H. flu b is responsible for meningitis and epiglotitis and maybe some pneumonia. Otitis and sinusitis are caused by types f and g and mostly the non-typables. Additionally H. flu vaccine is a protein polysaccharide conjugate that behaves like a protein antigen.
 *   You don't mention rhinitis treatment other than desensitization. It sounds like he would benefit from daily intranasal steroids and probably daily or as needed intranasal antihistamines in addition to saline nasal irrigations once or twice a day.
 *   The total IgG is near the lower limit of normal. Most immunologically normal individuals who have recurrent infection because of another susceptibility factor will have higher IgG concentrations.
 *   In our area the sinus CT would suggest allergic fungal sinusitis.
 *   I wonder if the response to Pneumovax has been sustained. Some patients like this one will have a great response measured at 4-5 weeks but revert to very low titers at 3 or 6 months.

Your clinical response is strong evidence that you have treated something real. I would continue IgG treatment.
Richard Wasserman
Dallas

On Fri, May 18, 2012 at 2:23 PM, Rohan Ameratunga (ADHB) <RohanA at adhb.govt.nz<mailto:RohanA at adhb.govt.nz>> wrote:
Dear Colleagues,
I would be most grateful for your opinions on this patient.

Case history

7 year old boy. Developed asthma early infancy. Good response to asthma prophylaxis.
Developed chronic sinus disease aged 5 including polyps.
Multiple and continuous upper and lower respiratory infections.
Atopic (dust mites, grasses etc) desensitised.- partial improvement.
Adenotonsillectomy- partial improvement
Placed on prophylactic antibiotics (Co-trimoxazole) - but continued to have breakthrough bacterial infections at least 1x per month. Usually H.influenzae in spite of vaccination and antibodies.

Investigations

CT sinuses pansinusitis/ nasal polyps
Normal Immunoglobulins 7 g/L (nr >7) and good response to HIB, dip-tet and pneumovax vaccines
Noted to have 8% double negative T cells (CD3+CD4-CD8-) Gamma-delta cells awaited.  No other features of ALPS.
Normal in vitro lymphocyte responses to lectins and antigens (diphtheria, tetanus, Candida)
Primary ciliary dyskinesia unlikely - normal ultrastructure and normal NO.
Normal sweat test.

Management

Underwent functional endoscopic sinus surgery- rapid recurrence of upper respiratory tract infections. Subsequently has balloon dilatation of nasal passages.  Regular sinus lavages.
Continued to have breakthrough upper and lower respiratory tract infections in spite of above.
Repeat CT sinuses- worsening sinusitis
I was concerned he was at high risk of bronchiectasis.  He had a chronic moist cough in spite of prophylactic antibiotics.

He was given a trial of subcutaneous immunoglobulin at the beginning of 2011.
There was a dramatic response to this.  His sinus disease settled, he no longer needs prophylactic antibiotics and has 1-2 bacterial infections per year while on subcut Ig.  His chest is clear.
Major improvement in QOL- was attending school/ daycare 50%- has only missed three weeks in the last year after starting Ig.  Can participate in soccer, watersports, gymnastics etc. Essentially normal busy life.
Previous weight gain 1.5 kg/y- has gained 4kg in 15 months since starting Ig.
CT sinuses not repeated as he is so well.

Questions

Has anyone had a similar experience?  Presumably this is an anti-inflammatory response rather than treating an undefined immune defect.
Interestingly, there is support for this approach in the literature.  A trial of IVIG seems to have shown objective markers of improvement in similar patients.
Any comments from any of the authors?  Not sure if Dr Ballow is on the list serve.
The question is how long to continue the scIg given the dramatic response.  One option is to stop in our summer (Nov) and review.  Any comments?

Ramesh S, Brodsky L, Afshani E, Pizzuto M, Ishman M, Helm J, Ballow M. Open trial of intravenous immune serum globulin for chronic sinusitis in children. Ann Allergy Asthma Immunol. 1997 Aug;79(2):119-24.

Thanks in advance

Rohan Ameratunga
Adult and paediatric immunologist
Auckland
New Zealand



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Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
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