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

[Bodo Grimbacher]

Dear Alan,
Some of the CVID lung pathology cannot be seen in a regular X-ray.
(You may argue that in that case they are also not worthy for treatment.)
However, to have a baseline of this possible "end-organ" damage is useful
in the future management of any of these patients.
So we (in London and in Freiburg) do an initial baseline CT scan of the
lungs (you may ask for a low radiation protocol if your radiology team
offers this). The (regular) follow-up is clearly driven by the clinical
presentation of each individual CVID patient.
Hope this helps,
Yours, Bodo

Am 07.05.12 16:00 schrieb "Alan Redding" unter <aredding99 at gmail.com>:


>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>

>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>:

>

>>       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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