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2017 ; 37
(3
): 287-294
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Gastrointestinal Manifestations in X-linked Agammaglobulinemia
#MMPMID28236219
Barmettler S
; Otani IM
; Minhas J
; Abraham RS
; Chang Y
; Dorsey MJ
; Ballas ZK
; Bonilla FA
; Ochs HD
; Walter JE
J Clin Immunol
2017[Apr]; 37
(3
): 287-294
PMID28236219
show ga
PURPOSE: X-linked agammaglobulinemia is a primary humoral immunodeficiency
characterized by hypogammaglobulinemia and increased susceptibility to infection.
Although there is increased awareness of autoimmune and inflammatory
complications in X-linked agammaglobulinemia (XLA), the spectrum of
gastrointestinal manifestations has not previously been fully explored. METHODS:
We present a case report of a family with two affected patients with XLA. Given
the gastrointestinal involvement of the grandfather in this family, we performed
a retrospective descriptive analysis of XLA patients with reported diagnoses of
GI manifestations and inflammatory bowel disease (IBD) or enteritis registered at
the United States Immunodeficiency Network, a national registry of primary
immunodeficiencies. RESULTS: In this cohort of patients with XLA, we found that
up to 35% had concurrent gastrointestinal manifestations, and 10% had reported
diagnoses of IBD or enteritis. The most commonly reported mutations were
missense, which have been associated with a less severe XLA phenotype in the
literature. The severity of symptoms were wide ranging, and management strategies
were diverse and mainly experimental. CONCLUSIONS: Patients with XLA may require
close monitoring with particular attention for GI manifestations including IBD
and infectious enteritis. Further studies are needed to improve diagnosis and
management of GI conditions in XLA patients.
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