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

[Risma, Kimberly]

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


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