[CIS PIDD] [cis-pidd] Sub-protective pneumococcal titers in a CVID patient on therapeutic doses of IgSQ and recurrent bronchitis

[Seppänen Mikko]

Dear Soheil,


a)      I would also try to culture sputum, though as an ID doctor I always tell young doctors and medical students that “there is no bacterial bronchitis, at least in non COPD-patients”,  this - like Ravishankar noted - is not exactly true in a subset of CVIDs…


b)      if available for You/his insurance: check for respiratory viruses by PCR (see Kainulainen L et al.)


c)       I would aim at higher IgG at least if there are pneumonias, other proven bacterial infections, signs of chronic viral infections, deteriorating diffusion in serial follow up (we take 1x/year, if in doubt 2x/y) or like. It is not rare that in pan-hypogammaglobulinemia + SAD (CVID/XLA/…)or HIGM3  I need a  trough >10-11.


d)      Below, You use AAAAI criteria to define a “protective level”, this is somewhat arguable and has not been formally proven, the only formal proof comes from WHO on >0.35 vs. invasive infections.
Since IgG does not contain mucosal IgA/ IgM and only contains IgG1+ IgG3 that through neonatal FcR do - but only to an extent - reach airways, we actually have no clue about how to interpret serum levels vs. airway/gut or other levels?
Thus individualized dosing seems even more reasonable, like Richard and others had noted already.
-------------------------------

e)      Outside this specific question, the fact that CVIDs often have bronchiectasis and need per oral /i.v. antibiotic courses based on sputum samples + physiotherapy/exercise seems to be far too often forgotten..
And diffusion is in our experience one of the very best follow up tests in CVID!



Hope this helps further?



Mikko


dos Mikko Seppänen, LKT
Immuunipuutosv-o, HYKS

Mikko Seppänen, MD, PhD, Associate professor/Senior Lecturer
Specialist in Internal Medicine and Infectious Diseases
Senior Consultant, Physician in charge (PIDD)
Immunodeficiency Unit
Division of Infectious Diseases
Department of Medicine
Helsinki University Central Hospital
Hospital District of Helsinki and Uusimaa
Aurora Hospital, Ward 4-2 and Outpatient Clinic
P.O.Box 348
FI-00029 HUS, Helsinki
FINLAND
phone +358 9 47175923, fax +358 9 47175945




Lähettäjä: Soheil Chegini [mailto:schegini at yahoo.com]
Lähetetty: 22. tammikuuta 2015 21:00
Vastaanottaja: CIS-PIDD
Aihe: [cis-pidd] Sub-protective pneumococcal titers in a CVID patient on therapeutic doses of IgSQ and recurrent bronchitis

Dear all,

I am wondering what others would suggest to reduce the frequency of bronchitis in a 54 y.o. man with CVID who is on Hizentra 10 g SQ weekly and has an excellent IgG level (814 in 6/14 and 916 1/15). He has responded well to oral antibiotics (Ceftin and Augmentin x 10 each) for 2 episodes of bronchitis that he has had this fall with resolution of his symptoms. He is also treated for asthma with Symbicort 160/4.5 and his spirometry is stable at his baseline with some irreversible airflow obstruction. No bronchiectasis.

Here are his 14-serotype pneumococcal titers:
Type 1                   0.9
Type 3                   0.9
Type 4                   <0.3
Type 5                   1.4
Type 8                   1.0
Type 9                   0.7
Type 12                 0.4
Type 14                 3.3
Type 19                 1.4
Type 23                 0.9
Type 26                 1.1
Type 51                 1.3
Type 56                 1.2
Type 68                 0.7

These titers suggest adequate protection only against 4 of the 14 tested serotypes, but I am not inclined to increase his dose of Hizentra given his excellent total IgG. He does not produce any Ig (IgA,7 and IgM 11), and his pre-Treatment IgG was 151. He is tolerating the infusions well and I have no other excuse to change the Ig preparation.

I would very much appreciate your help and advice. Thank you very much in advance.

Best regards,
sc

Soheil Chegini, M.D.
Exton Allergy & Asthma Associates
656 West Lincoln Hwy.
Exton, PA 19341
Phone: (610) 269-3066
Fax: (610) 269-8615



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