[CIS-PAGID] Advice please

[Rohan Ameratunga (ADHB)]

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