Abstract
A rare overlap syndrome between CREST (calcinosis, Raynaud phenomenon, esophageal
dysmotility, sclerodactyly, and telangiectasia) syndrome and primary biliary cholangitis
(PBC) is described as PACK syndrome, an acronym for primary biliary cholangitis, anticentromere antibodies, CREST syndrome, and keratoconjunctivitis sicca. In this retrospective cohort analysis and review, we present
fourteen patients who meet diagnostic criteria for PACK syndrome in one of the largest
case series of this group. All patients were female, 86% of whom were White with an
average age of 66.7 years (range 39-78 years). The prevalence was 5.08% in our PBC
cohort (n=256) similar to previous findings. CREST syndrome was diagnosed prior to
PBC in 58% of our patients and limited pulmonary and renal involvement were observed.
This syndrome is rare, but given its insidious development, clinicians should be aware
of this potential overlap in CREST-only and PBC-only patients.
Keywords
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Article info
Publication history
Published online: December 16, 2022
Accepted:
December 10,
2022
Received:
January 27,
2022
Identification
Copyright
© 2022 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
