[CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

[Seppänen Mikko]

Dear all,

there is an Italian proof of concept study, Chest. 2011 Dec;140(6):1581-9, on use of MRI in this setting, and there will be another soon published from Turku, Finland (Kainulainen L et al).

I have been trying to convince our pulmonary radiologists to start MRI here as well, w/o luck... It certainly is promising and would potentially solve a lot of issues with follow up, maybe should also be studied if dose titration would become easier with it? I would warmly welcome (and participate in) a large multicentre study if someone is interested in running such a study!

mikko
__________________________________________________
Mikko Seppänen, MD, PhD
Specialist in Internal Medicine and Infectious Diseases
Senior Consultant, Physician in charge (PIDD)
EM(E)A Expert, PIDDs and Intravenous Immunoglobulin Therapy

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
_________________________________________


-----Alkuperäinen viesti-----
Lähettäjä: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Bleesing, Jacob
Lähetetty: 7. toukokuuta 2012 21:53
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: Re: [CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

a quick note on imaging and patients with immunodeficiency disorders (concerns for radiation).

having to deal with 2 sibs with Ligase IV deficiency, I learned from our radiologists that MRI might be an alternative to HRCT scans. Got good data from MRI, but since there was no significant bronchiectasis, hard to make a case for MRI as a replacement

see URL:

http://www.ncbi.nlm.nih.gov/pubmed/21622550

Perhaps an opportunity for us to find out if this true or not.

JB



________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Riedl, Marc [MRiedl at mednet.ucla.edu]
Sent: Monday, May 07, 2012 2:33 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

Certainly this highlights the importance of discussing the treatment options including IgG replacement with patients.  I always discuss the "worst case scenario" with them such that they clearly hear the potential risks of deferring therapy.    I would recommend the HRCT if not done previously.  I've been surprised occasionally at the disconnect between CT and PFT findings, I.e. Normal PFTS don't necessarily r/o bronchiectatic changes.  I personally don't do HRCT at regularly scheduled intervals, but I have a low threshold for repeating if there is any notable and persistent change in respiratory clinical status of PID patients.

Best,

Marc

Marc Riedl, M.D., M.S.
Associate Professor of Medicine
Section Head, Clinical Immunology and Allergy
UCLA - David Geffen School of Medicine
10833 Le Conte Ave, 37-131 CHS
Los Angeles, CA  90095-1680
Tel 310.206.4345  Fax 310.267.009


From: Alan Redding <aredding99 at gmail.com<mailto:aredding99 at gmail.com>>
Reply-To: pagid listserve <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>
To: pagid listserve <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>
Subject: Re: [CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

Dear Dr. Grimbacher/all,

   Yes, I think, from a medico-legals standpoint, we should offer this women immunoglobulin replacement, despite the fact that her infections history has been relatively mild.  Now, from a purely medical standpoint, do I need to perform a high-resolution CT scan on her, as many of have suggested?  Or, if her PFT's are completely normal, can I just do a chest X-ray?
   I have even read that patients with CVID should have CT chests at regular intervals.  Is this plan better than ordering chest X-rays and full PFT's at regular intervals?  Since CT chests deliver much more radiation than chest X-rays, I do not want to irradiate anyone more than necessary.
   I appreciate all of the wonderful advice that I have received from around the world.

Sincerely,
 Alan

On Sun, May 6, 2012 at 5:54 AM, Grimbacher, Bodo <b.grimbacher at ucl.ac.uk<mailto:b.grimbacher at ucl.ac.uk>> wrote:
Dear all,

What is the medico-legal situation with a patient with < 4g/L IgG in the
US and such an infection profile?

I was told of a court-trial in Hamburg in which the Hospital lost as they
failed offering a your women IVIG treatment who went on developing
debilitating bronchiectasis.
So should we at least offer these type of patients IgG replacement?

Prof. B. Grimbacher
Director
CCI - Centre of Chronic Immunodeficiencies
Freiburg, Germany

Am 03.05.12 18:44 schrieb "Alan Redding" unter <aredding99 at gmail.com<mailto:aredding99 at gmail.com>>:


>       Recently, an internist referred a 54 yo F to me because her total

>protein (TP) level was low (5.8 g/dL) and her gamma globulin fraction was

>low (0.2 g/dL).  Bloodwork was done as part of a routine physical.  In

>her twenties, while pregnant, she says that she was hospitalized for

>pneumonia (patient doesn't know details of this infection).  Since then,

>she says that she has been treated for pneumonia twice as an outpatient,

>but she cannot recall having a CXR on either occasion.  This is her only

>infectious history.  Other than hypercholesterolemia, she is healthy.

>She feels perfectly fine.  No history of recurrent sinusitis, bronchitis,

>cough, etc.  She even asked me "Why am I here?"

>       On further workup, total Ig A was undectectable (<4 mg/dL), IgM

>was low at 23 mg/dL, and IgG was low at 240 mg/dL.  She had protective

>levels to tetanus (0.45 IU/mL) and diptheria (0.07 IU/mL), which

>increased after Tdap vaccination to 1.85 mg/dL and 0.20 mg/dL,

>respectively.  She also had protective antibody levels to Varicella

>Zoster virus.  She did not respond to the first dose of hepatitis A virus

>vaccine, but did show "reactive" antibody levels after receiving the

>second dose of hepatitis A virus vaccine.  However, she showed zero

>response to Pneumovax vaccine, the H. flu vaccine, or the meningoccal

>polysaccharide vaccine.

>     In summation, it appears that she can mount an immune response to

>protein antigens, both new and old.   However, since she did not respond

>to the H. flu conjugate vaccine, and, she did not respond to the

>hepatitis A vaccine until after the second dose, the response may be

>sluggish.   And, she cannot respond to new polysaccharide vaccines.

>          I have never seen a patient like this, before.  Could it be

>that I have just caught CVID, and she is just lucky that she has not had

>a serious infection?  Or, might one say, "Well, she does have low

>antibody levels.  But, something must be working right, because she is

>54, and hasn't had frequent or severe infections.   It may be difficult

>to talk her into starting immunoglobulin replacement when she feels

>normal, and has hardly been sick.   However, I want to recommend the

>safest course of action, both for her sake, and, for mine.  I would

>appreciate any recommendations, especially, if anyone has ever had

>personal experience with patients such as this.

>

>Sincerely,

>Alan Redding, M.D.

>Redding Allergy and Asthma Center

>3193 Howell Mill Rd. NW, Ste 102

>Atlanta, GA 30327

>direct line (404) 941-1183<tel:%28404%29%20941-1183>

>cell (404) 593-33338

>fax (404) 355-0079<tel:%28404%29%20355-0079>

>

>

>

>





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