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

[Seppänen Mikko]

Dear Alan,

no need for me to write a lengthy story, since therapy wise Marc wrote it ALL for me...(nice to notice that some agree in so many points). 

I would however, like Marc and Melvin, stress that HRCT at start of follow up, and PFT w DLCO are in my experience as well often aberrant (i.e occult pulmonary problems are common) or that especially DLCO may very insidiously deteriorate during follow up (minimum at start every 6 months, if stable, once 12 months and information that "help is one call away").As an ID specialist, I tend to dislike long-term prophylactic antibiotics, but they do get antibiotics courses to home to be used when needed. 
 
Once You commence therapy, if You have not just followed too long, PFTs usually correct themselves within next 6-12 months (and the patient becomes extremely compliant)... 
Most of my patients are now on therapy, due to PFT deterioration and/or bronchiectasis/COP, occasional ones have curiously developed mildish emphysema before therapy (nonsmokers, antitrypsin normal)? At least one was found to have LIP as well in this scenario, otherwise quite common in symptomatic CVIDs in Finland, and very many seem to radiologically have follicular bronchiolitis (nonsmokers, even w/o asthma), respiratory bronchiolitis (smokers, quitting mandatory or the patient can expect a very quick descent), mediastinal lymphadenopathy or like. And if HRCT shows bronchiectasis, start! And HRCT often shows occult large spleen as well and does find the occasional Good, like Klaus suggested, but do tell the poor pulmonary radiologist all that You are looking for... ;=)

BTW, in my patients, in a scenario like this, CD4 are often low/lowish, NK(CD16+56+) often low, B-cells might be low and all the three mix into various combinations, and smBs very often low or at least borderline (close to 0.4%, cf. with EUROClass).

It is not always easy to start therapy right away, since it is cumbersome and if the patient feels subjectively well, You often need objective evidence to convince the patient (and others as well). 



mikko

Helsinki, Finland

PS. The most mysterious patient similar to this in my career was a young lady, who had almost no measurable IgG,A,M and severe, non-therapy responsive, primary, fulminant pulmonary hypertension, no (other?) autoimmunity, absolutely no infections and she died very quickly. I lost the patient ID and have ever since regretted that I did not publish it back then, but I was very young and unexperienced. I have never seen primary PHT in CVIDs since, some secondary to severely damaged lungs - yes. Have You?


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-----Alkuperäinen viesti-----
Lähettäjä: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Riedl, Marc
Lähetetty: 3. toukokuuta 2012 20:51
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: Re: [CIS-PAGID] CVID? Diagnosed Incidentally in Healthy Patient, What Do I Do?

We follow a number of similar patients at our center.  We attempt to gather as much immunologic data as we can including switched memory B-cell profile and vaccine responses.  However, at the end of the day, this is probable CVID and it's a matter of discussing the risks and benefits of Ig replacement therapy with the patient.  I typically get chest CT and PFTs with DLCO to help guide the strength of that recommendation.  At these levels and with a history of pneumonia, I strongly encourage patients to start therapy given the potential risk of the next infection being serious and rapidly progressive.  That said, we have patients that with understanding of the risk elect to forgo IVIG ("I've done fine to this point") and we monitor them clinically over time.  Some have in fact done well for many years without evidence of recurrent infection or pulmonary consequences.  It would tremendously useful to have methods of risk-stratification for these patients, but aside from the above diagnostics, I think we're making only educated guesses about the optimal treatment course.

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

       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
cell (404) 593-33338
fax (404) 355-0079





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