[CIS PIDD] [cis-pidd] 62yo M with chronic infections, slightly abnormal labs

Fri Jul 11 13:14:22 EDT 2014

Marc and Yesim, 

Sorry for the delay in response, I was away from my office and could not access his records.
But, here are the answers to your questions:
PFTs (5/1/14):  FEV1     69%p (2.49 L),  FVC  68%p,  FEV1/FVC  0.76  (101%p), Interpretation:  Overall he has abnormal spirometry with no
evidence of airway obstruction for his age but with a restrictive pattern
instead.  
I also reviewed prior PFTs from a previous doctor and they also appear to have a moderate restrictive pattern, with his FEV1 and FVC have both been in the 60s%p
and his FEV1/FVC is normal at ~100%p.  This pattern at least dates back to July 2011, for which I have PFT
values available.
I do not have access to DLCO in my office, so I do not have this value.  I did refer him to pulmonology for further evaluation, so they may have done it, but I don't have the results as of now.
For what it's worth, his CXR was normal.  HRCT chest is pending.
CF has been ruled out, although I have not seen the documentation myself.

Shayna Burke, MD
Allergy and Asthma Family Care
928 Farmington Ave
West Hartford, CT 06107
860-233-6293

 From: "Riedl, Marc" <mriedl at ucsd.edu>
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org> 
Sent: Thursday, July 10, 2014 1:39 PM
Subject: Re: [cis-pidd] 62yo M with chronic infections, slightly abnormal labs

Curious what PFTs/DLCO and Chest CT look like. 

Marc Riedl, MD, MS 
Associate Professor of Medicine 
Division of Rheumatology, Allergy & Immunology 
University of California, San Diego

9500 Gilman Dr, Mail Code 0732 
La Jolla, CA  92093 
Tel 858.822.6766  Fax 858.642.3791    

 From: Shayna Burke <shaynaburke at yahoo.com>
Reply-To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Date: Thursday, July 10, 2014 6:49 AM
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Subject: Re:[cis-pidd] 62yo M with chronic infections, slightly abnormal labs

I recently saw this patient in my private practice office and was looking for any advice on an actual diagnosis, any further testing that should be done, and treatment suggestions.  Thanks in advance. 

He is a 62 year old gentleman who was diagnosed with asthma in his 30s (1983) after taking Inderal (propranolol) for an ocular migraine and within a few days of starting this medications suddenly developed severe asthma.  He has been hospitalized 2x for asthma, with 1x in the ICU.  He started prednisone in 1987 after he got a flu shot and then subsequently developed a severe asthma exacerbation, and has never been able to get off of the prednisone.  He used to require albuterol 4x/day, but now is much better controlled.  He is on numerous medications for his asthma, including prednisone 10mg daily, theophylline 300mg BID, Singulair 10mg daily, Dulera 200/5 mcg 2 puffs BID, Alvesco 160mcg 2 puffs BID, and Xolair 300mg SQ Q4weeks.    

He also has chronic otitis and sinus problems, including s/p bilateral mastoidectomies (right 1998, left 2010) and numerous myringotomy tubes, but with continued frequent otitis.  At the time that I saw him, he actually had new onset of green pus draining from his previously healed incision of his left mastoidectomy, which ended up growing pseudomonas.  In the past cultures of his ear have grown MRSA, for which he was treated with Bactrim x2months.     He has had pneumonia 2 times in the past (confirmed by CXR), 1 of which he was hospitalized for.  He also has a history of nasal polyps, s/p sinus/polyp surgery 3x (last in 1994).  He has not had any regrowth of the nasal polyps since remaining on fluticasone nasal spray after his last sinus surgery.   

Labs: 

	* normal CBC with dif aside from slightly high WBC at 11.9, slightly high neutrophil % at 88.4% and total neutrophil count at 10.5 k/UL, and low lymphocyte percent at 5.8% and absolute at 0.7,  
	* normal IgA at 105,  slightly low total IgM at 41 (nl 45-281), elevated total IgE at 417 (on Xolair) 
	* normal total IgG at 900, IgG1 at 438, IgG2 at 328, IgG3 at 41, and elevated IgG4 at 196, 
	* normal alpha-1-antitrypsin level at 141, 
	* protective diphtheria Ab at 0.46, protective tetanus Ab at 1.95 
	* “pre vaccination” (est last Pneumovax 5-10 years ago):  only 11 out of 23 protective, 
	* “post vaccination” with Pneummovax:  protective only to 14 out of 23 Pneumococcal serotypes with only 3-4 that had a 4fold increase and 7 serotypes actually went down from pre to post vaccination titers.   
	* normal complement level and normal C4 level, 
	* low total lymphocyte count (CD45) at 0.8 (nl 0.82-2.84) with normal Tcell and NK cell %s but low Bcell % at 3%, low total CD3 Tcell number at 529 (nl 550-2202), low Bcell number at 17 (nl 45-409), and low CD4 helper cells at 336 (nl 365-1437).   
	* Of note, there were 14% CD3+CD4+CD8+ (double positive) Tcells present.  

Given his chronic infections, despite his normal total IgG, I was planning on starting a trial of SCIG replacement. 

I also recently ordered an SPEP and HIV screen, which are pending.  He is having a ciliary biopsy by ENT this week as well. 

Any suggestions would be greatly appreciated.   Thanks in advance. 

Sincerely, 

Shayna Burke, MD 

Allergy and Asthma Family Care 

928 Farmington Ave 

West Hartford, CT 60107 

Phone:  860-233-6293 

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