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

[Riedl, Marc]

Mikko -

I've not seen primary PHT with CVID either.  Like you, have seen secondary
PHT due to advanced chronic lung disease from underlying PID and also
secondary hypogamm due to long-term immunosuppressive regimens used by our
pulmonary colleagues at the large ILD and PHT Lung Center here.

On 5/3/12 10:06 PM, "Seppänen Mikko" <Mikko.Seppanen at hus.fi> wrote:


>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?

>

>

>__________________________________________________

>-----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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