[CIS PIDD] [cis-pidd] 20 year old female with low IgA and autoimmune gastritis

Fri Feb 13 13:29:57 EST 2015

Hi Kim,
I have no answer to the question you are asking, but instead maybe a suggestion for an alternative to immunosuppressive treatment.
You did not specify what exactly her symptoms were, but I wonder whether she might respond to octreotide. Unfortunately, my experience with this condition is from my fellowship days when I worked on an animal model of organ-specific autoimmunity in mice after neonatal thymectomy. I have not had cared for any patients with this diagnosis, but have a patient who is on octreotide for intestinal lymphangiectasia, and he has done substantially better on it.
Best wishes,Soheil
Soheil Chegini, M.D.
Exton Allergy & Asthma Associates
656 West Lincoln Hwy.
Exton, PA 19341
Phone: (610) 269-3066
Fax: (610) 269-8615
      From: "Risma, Kimberly" <Kimberly.Risma at cchmc.org>
 To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org> 
 Sent: Friday, February 13, 2015 10:31 AM
 Subject: [cis-pidd] 20 year old female with low IgA and autoimmune gastritis

Hello colleagues,    I have followed a young lady for a few years who presented for immune evaluation at 19 years of age with debilitating abdominal distress secondary to documented active autoimmune gastritis. She had elevated gastrin levels, (+) antiparietal Antibodies, and anti thyroperoxidase antibodies suggesting  a variant of autoimmune polyendocrinopathy. Her symptomatology responded to oral steroid therapy and she was transitioned successfully to sirolimus as she did not tolerate a “no treatment” approach. Over the last 3 years of therapy her gastrin levels have fallen nicely although her antiparietal Ab remains strongly (+). She becomes symptomatic again when she stops sirolimus for a week. Her immune evaluation was essentially normal except for a mildly decreased IgA. She has no history of recurrent infections. She reportedly developed Herpes Zoster once on sirolimus, but it resolved successfully without antivirals.    My question for the group-  She is wanting to have a baby and would like to stop her medications to do so (I don’t have any experience with pregnancy and sirolimus). Are there any other options other than trying to find a low dose steroid approach?    Thanks in advance, Kim    Kimberly Risma MD PhD Assistant Professor Director, Allergy/Immunology Fellowship Division of Allergy/Immunology Cincinnati Children’s Hospital Medical Center     ---  The CIS-PIDD listserv is supported by:  
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