[CIS PIDD] [cis-pidd] recurrent pneumonia

Thu Jun 2 07:58:57 EDT 2016

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<mailto: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]
Lähetetty: 2. kesäkuuta 2016 0:44
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net<http://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<mailto: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<mailto:cis-pidd at lists.clinimmsoc.org>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Wednesday, June 1, 2016 at 1:33 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto: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<mailto:James.Jones at childrenscolorado.org>

From: CIS-PIDD [mailto: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]
Verzonden: woensdag 1 juni 2016 14:06
Aan: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto: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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