[CIS PIDD] [cis-pidd] recurrent pneumonia

Wed Jun 1 17:44:29 EDT 2016

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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