[CIS PIDD] [cis-pidd] 60+ yo man with wasting complicating Good Syndrome

Tue Jan 26 19:30:32 EST 2016

Joe,

I’ve seen this sort of concerning progressive weight loss in several adult CVID patients, though not in any of the Good Syndrome patients I follow.  Have conducted exhaustive infectious, malignancy, and nutritional evaluations and in each case the weight loss has been attributed to what our GI docs call “CVID enteropathy”, which means small and/or large bowel biopsies show inflammation and abnormal architecture, but not fitting into the typical patterns of IBD, celiac, etc.  In some cases, there’s been a remarkable lack of clinical bowel symptoms despite the abnormal biopsy results.  Stool studies for malabsorption have sometimes been abnormal.   I’d re-look closely at the lower GI tract.

Best,

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: Tuesday, January 26, 2016 at 12:41 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: RE:[cis-pidd] 60+ yo man with wasting complicating Good Syndrome

Hello Joe,
What is the stool alpha 1 antitrypsin clearance?
Also, have you ruled out  strongyloides and Whipple’s disease?

How is his cardiac function?
What are his B cell numbers?

I have seen a few older adult patients ( n=2) with intestinal lymphangiectasia as the only cause for wasting and have responded to Entocort ( budesonide).

Thanks,
Avni

Avni Joshi
Mayo Clinic

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, January 26, 2016 1:45 PM
To: CIS-PIDD
Subject: [cis-pidd] 60+ yo man with wasting complicating Good Syndrome

Colleagues:

Below is the history of a man I saw today.  Any insights you may have for the management of his progressive wasting would be much appreciated.  Thanks.

Joe Church

Children’s Hospital Los Angeles

Chief Complaint

Good Syndrome complicated by marked weight loss

History of Present Illness

This 60+yo man was referred for evaluation of immune deficiency and marked weight loss.

In October 2011 he was diagnosed ashaving common variable immune deficiency (CVID) based upon very low IgG (253, 329), IgA (<10, 11) and IgM (13,mg/dL) levels, and very poor responses to Pneumovax (+0/23 to +1/23) and tetanus vaccines (0.12, 0.16) on 10/21/11 and 12/12/20/11, respectively. He was started on Ig replacement and since early 2012 he has been self- infusing Hizentra weekly since November 2012.  Per patient report his IgG was 917 on 1/4/16.  Getting 14 grams per week.  Importantly, pt never had typical infections (respiratory) characteristic of CVID.

Lichen planus:  first noted oral, biopsied (2009, 2011), skin of back (Sept 2014), skin of right elbow (June 2014).  Skin involvement much improved with cyclosporine; oral/tongue involvement 'slightly improved.'  Currently oral lichen planus lesion looks terrible to me.

In February 2013 he was diagnosed with thymoma (noted serendipitously when abdominal CT spotted the tumor), Type AB, Masroka Stage III.  With the prior diagnosis of CVID this now is diagnostic of Good syndrome At this time a RUL lung nodule was noted and a needle biopsy showed granuloma only;   Excision of the 986 gram tumor was done on March 26, 2013.  However, the pericardial margin was still positive for tumor, and he received radiation therapy (no chemo).   Getting Chest CT ~ q 6 months; so far so good.

On October 1, 2015 he was hospitalized for a Hemoglobin of 5.7.  At time he felt very weak and had swelling of legs.  He received "at least 10 units of blood.  Initially diagnosed at xxxxxxx   with myelodysplasia, he obtained a second opinion where a diagnosis of pure red blood cell aplasia was made.  He was started on cyclosporine and has had a good response with his latest Hgb at 13.2 on 1/19/16.

Patient’s primary concern at this time is progressive weight loss.  In mid-2012 he weighed ~150 lbs. and began losing weight often 5-10 lbs. over short periods of time, then plateauing for a while before losing again.  At the time of his thymectomy March 26, 2013 he weighed 130 lbs.  He has tried multiple weight gain techniques:  small frequent meals, supplements, high calorie foods etc.  Nothing has helped.  He has had multiple blood and stool tests and 3 upper endoscopies (Jan 2013, Sept 2013, Sept 2015) that did not identify an etiology for his weight loss.  He was shown to have "mild gastritis, a 1 cm hiatal hernia with minimal yeast" (Jan 2013); "chronic gastric inflammation" (Sept 2013); and a small stomch polyp that was removed (benign) September 2015.  He was given an empiric trial of Rifaximin at xxxxxxx (Jan 2016); a pill cam was just done; results are pending.   His reported weight on 1/21/16  and at today’s visit was 110 lbs.

GI related issues in the past have included

   -  Constipation of relatively sudden onset (2011).  A colonoscopy was done (?~2005) and was normal.

   -  Hemorrhoids:  Had for years

   -  E. Coli  AGE after trip to Europe ~ April 2015

   -  Large stools without diarrhea:  noted earlier Jan 2016; re-started on Creon and seems improved.

Laboratory Results (selected):

   -  CBCs:  innumerable all normal until onset of pure red cell aplasia.

   -  Comp metabolic panels:  innumerable all normal except decreased albumin 2.6 (10/3/15); 2.9 recently.

   -  See CVID for immunoglobulin levels and pneumococcal antibody response

   -  10/21/2011 CD3 85% (2017), CD4 40% (958), CD8 38% (909);  HIV Ab negative;  CH50 >60 (normal);  ANA + !:80;  celiac panel negative;  SPEP low gamm, but no spike.

   -  12/20/11  anti-IgA "<113 U/mL (negative)

GI Laboratory Results (selected):

  -  Dec 2012  O+P, Giardia Ag, cryptosporidium, C. diff toxin, culture all negative;  IBD panel negative

  - July 2013 stool elastase 377 elevated

   -  May 2015   IBD panel (extended) negative;  GI pathogen PCR panel (includes bacteria, parasites and viruses adeno, rota, noro etc.) negative

   -  Sept 2015  GI Pathogen PCR panel  negative

   -  Oct 15, 2015   CBC normal;  EPO level 1120 (normal 4.3-18 mIU/mL).

FINALLY the current plan is to place a PICC and begin TPN while the search for an etiology of the wasting is continued.

.

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