[CIS PIDD] [cis-pidd] recurrent pneumonia
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Thu Jun 2 08:25:45 EDT 2016
Thank you for your interesting suggestions.
Lymphocyte phenotyping normal.
Repeat pneumococcal antibody titers four years after vaccination 16/23
titers well maintained
Interval chest x-ray normal, HRCT not done, Pneumonias not localized. No
episodes of sinusitis or other infection in the past four years. No
organism was isolated during her episodes of pneumonia.
IgG subclasses, CF testing not done. CH50 normal so C2 deficiency unlikely.
I doubt cyclic neutropenia in the absence periodic fevers and because of
the long interval between infections. Wouldn't ELA2 deficiency result in
more frequent infections and persistent neutropenia?
I also doubt ABPA with a normal interval chest x ray, no symptoms and IgE
=2. None of the episodes have been treated with steroids making
eosinophilic pneumonia and BOOP unlikely.
I will certainly image her chest and look for a CF mutation.
Given the low frequency of these pneumonias, how do people feel about
prophylactic antibiotics or IG treatment?
Richard
Dallas
On Thu, Jun 2, 2016 at 6:58 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:
> Mikko's excellent list should be given to every trainee.
>
> I would add foreign body. More common in kids but I have an adult who was
> in a hurricane and inhaled something that keeps reseeding fungus in her.
>
> Kate Sullivan
> Sent from my iPhone
>
> On Jun 2, 2016, at 3:19 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
> Hi!
>
>
>
> (again someone commented w/o signing in this thread, please remember to
> sign!)
>
>
>
> This is one of the most common problems an adult CI meets. In parenthesis
> what we have found from tertiary hospital:
>
> a) earlier missed CF? In adults, sweat test often poor, gene test
> needed (occasional) +/- ABPA
>
> b) earlier missed IC (and ultrastructural and measures to directly
> assessed ciliary motility needed), usually always recurrent sinusitis as
> well (occasional)
>
> c) structural causes (HRCT?):
> 1) bronchiectasis (7-15% of BE patients have PID, additional similar
> percentages CF/IC)? (common)
> 2) in children at least bronchomalacia has been shown to increase risk,
> seen 3 adults where nothing else was found
> 3) pulmonary sequestration (pneumonia always at the same lobe)
>
> d) HIV (common)
>
> e) SAD/SPAD (which of course in this patient is not the case) +/-
> IgG2 deficiency. (occasional)
>
> f) Symptomatic, isolated IgG2D is very rare w/o SAD, and then IgG2
> is usually VERY low and I would then look for APDS type I/II (PIK3CD/R1) or
> Lynch syndromes, take a very careful and broad family history first. (rare)
>
> g) recurrent pneumonias due to non-CF ABPA and bronchiectasis
> combined (not that rare)
>
> h) chronic pulmonary aspergillosis (CCPA, CNPA and others) (not that
> rare)
>
> i) long-term active smoker, COPD (tobacco smoke actually not only
> causes secondary IC but also secondary MHCII deficiency) (common)
>
> j) GERD (RR only 1.15, treatment problematic) (occasional)
>
> k) recurrent eosinophilic pneumonia (occasional)
>
> l) recurrent COP/BOOP and an immunologic disease (rare)
>
> m) C2 deficiency (rare)
>
> n) late onset CGD (only in literature not found any yet)
>
> o) cyclic neutropenia, either secondary or late-onset ELANE
> (described rarely in several other SCN genes, occasional/rare)
>
> p) and I have always wondered whether there is a hypomorphic SGD
> around, thus I do check blood smear in problematic cases, if hyposegmented
> neutrophils are to be found, not found it yet)
>
>
>
> When it comes *to MBL/MASP2 deficiency*, I am a skeptic:
> Depending on the definition what MBL deficiency is (whether only minute
> amounts of MBL are sufficient to be called MBLD), Finland has MBL
> deficiency in 9-13% of individuals (I think 3rd highest in the world).
> Thus, in a complete workup, if CH50A, CH50C, CH50L are done simultaneously,
> you will eventually find MBL deficiency without causal relationship (and I
> have not seen any more than what I would expect if thus studied).
> Population-based studies do not suggest cause-effect relationship. The NEJM
> case report on MASP2 def, if you read NEJM 2003;349:554-60 carefully, notes
> that the patient had SAD/SPAD also! MBL2/MASP2 def is undoubtedly a risk
> factor for more frequent pneumonias and bronchiectasis in antibody
> deficient patients, I personally doubt if anything else.
>
>
>
> q) Occasional pneumonias in AERD/Samter patient and in severe asthma
> are also seen sometimes, but not in the frequency you state.
>
>
>
> r) And after complete work up, I still have no cause for an
> occasional patient, if the pneumonias are highly frequent I do test the
> effect of IVIg for 9-12 months and if it does help I am - and the patient
> is - happy. I wonder if these people could have a SPAD/SAD –form where
> anti-PnP antibodies would be in the wrong subclass and non-opsonizing
> (there is scarce old literature to suggest this).
>
>
>
> Hope this helps,
>
>
>
> Mikko
>
>
>
>
>
> oyl Mikko Seppänen
>
> Harvinaissairauksien yksikkö (HAKE), HUS
>
>
>
> Mikko Seppänen, MD, PhD, Associate professor
>
> Specialist in Internal Medicine and Infectious Diseases
>
> Head, Rare Disease Center, Helsinki University Hospital (HUH)
>
> Children’s Hospital, P.O.Box 280
>
> FI-00029 HUS
>
> FINLAND
>
> &
>
> Senior Consultant (PIDD)
>
> Adult Immunodeficiency Unit
>
> Inflammation Center, HUH
>
>
>
> phone +358 9 47180201
>
> GSM +358 50 4279606
>
> fax +358 9 47174703
>
>
>
>
>
>
>
> *Lähettäjä:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org
> <cis-pidd at lists.clinimmsoc.org>]
> *Lähetetty:* 2. kesäkuuta 2016 0:44
> *Vastaanottaja:* CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Aihe:* Re: [cis-pidd] recurrent pneumonia
>
>
>
> Geez this patient sound more than vaguely familiar...getting blood drawn
> for Wahi in 2 weeks if you'd like to tag on...
>
> Sent from my iPhone
>
>
> On Jun 1, 2016, at 2:18 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
> Agree with the previous suggestions. I’ve seen 2 similar adult patients
> that had long histories of serious recurrent pneumonia with subsequent
> development of structural lung disease. After exhaustive immunodeficiency
> and pulmonary evaluations, one was diagnosed only with complete MBL
> deficiency and the other with immotile-cilia syndrome by ultrastructural
> studies. The former patient has just started on a trial of IVIG due to
> frequent hospitalizations, bronchiectasis, and the lack of any other
> specific therapy. Too early to say whether this is of any benefit. The
> latter patient was lost to f/u.
>
>
>
> Marc
>
>
>
> Marc Riedl, MD, MS
>
> Professor of Medicine
>
> Adult Primary Immunodeficiency Program
>
> Division of Rheumatology, Allergy & Immunology
>
> University of California, San Diego
>
> 8899 University Center Lane, Suite 230
> San Diego, CA 92122
> Tel 858.657.5350 Fax 858.657.5375
>
>
>
> *From: *CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> *Reply-To: *CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Date: *Wednesday, June 1, 2016 at 1:33 PM
> *To: *CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Subject: *RE: [cis-pidd] recurrent pneumonia
>
>
>
> The observation by Dr. Meyts brings to mind an adult female patient who
> carried a Dx of an antibody deficiency syndrome who had recurrent Staph and
> pseudomonas pneumonias who was found to have normal Ig function, but who
> also totally lacked mannose binding lectin and was a heterozygote for
> cystic fibrosis.
>
>
>
> Which organisms were responsible for the current patient’s infections?
>
>
>
> Jim Jones
>
>
>
> James.Jones at childrenscolorado.org
>
>
>
> *From:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org
> <cis-pidd at lists.clinimmsoc.org>]
> *Sent:* Wednesday, June 01, 2016 2:10 PM
> *To:* CIS-PIDD
> *Subject:* RE: [cis-pidd] recurrent pneumonia
>
>
>
> Interesting.
>
> Looking at it from a different angle if as said lymphocytes etc are fine
> and before looking deeper into PID:
>
> may sound stupid but did the patient have sweat test / nasal potential
> measurement / CFTR mutation analysis performed? We have seen atypical CF
> presenting like this.
>
> No otitis so probably not a ciliary problem.
>
>
>
> Best,
>
> Isabelle meyts uz leuven belgium
>
>
>
>
>
> *Van:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org
> <cis-pidd at lists.clinimmsoc.org>]
> *Verzonden:* woensdag 1 juni 2016 14:06
> *Aan:* CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Onderwerp:* [cis-pidd] recurrent pneumonia
>
>
>
> Colleagues:
>
> I would like suggestions on a 46 yo female anesthesiologist with recurrent
> pneumonia. She has had five episodes of pneumonia over the last 15 years.
> Two requiring hospitalization and one ICU admission. Her most recent
> pneumonia admission occurred despite beginning levofloxacin within three
> hours of the onset of symptoms. Her infection history includes recurrent
> sinusitis when young that has become a much less frequent problem, a large
> cellulitis after a stingray envenomation and MRSA sinusitis following
> surgery. She is a non-smoker. Her most recent pneumonia occurred while on
> vacation and was not hospital acquired.
>
> IgG 1070 IgA 132 IgM 125
>
> Responded to 17/23 pneumococcal serotypes, adequate response to DT and H.
> flu. CH50 and AH50 normal.
>
> Mannose binding lectin undetectable.
>
> Suggestions for additional studies?
>
> Given the fact that she averages less than on infection per year, I have
> been unenthusiastic about antibiotic prophylaxis. Any management
> recommendations?
>
> Thank you,
>
> Richard Wasserman
>
> Dallas
>
>
>
> --
>
> Richard L. Wasserman, MD, PhD
> Allergy Partners of North Texas
> 7777 Forest Lane, Suite B-332
> Dallas, Texas 75230
> Office (972) 566-7788
> Fax (972) 566-8837
> Cell (214) 697-7211
>
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--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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