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PACK syndrome: A case series and review
B Collins1, D Dillon1, R M Silver1
1Department of Medicine, Medical University of South Carolina, Charleston, SC, USA; Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, USA.
The American Journal of the Medical Sciences
|December 19, 2022
Summary
PACK syndrome, a rare overlap of primary biliary cholangitis (PBC) and CREST syndrome, affects mostly White women over 65. Early recognition is crucial for managing this condition.
Area of Science:
- Rheumatology
- Hepatology
- Autoimmune Diseases
Background:
- PACK syndrome is a rare overlap between CREST syndrome and primary biliary cholangitis (PBC).
- It is characterized by primary biliary cholangitis, anticentromere antibodies, CREST syndrome, and keratoconjunctivitis sicca.
- Understanding this overlap is essential for patient management.
Purpose of the Study:
- To describe the clinical characteristics of patients with PACK syndrome.
- To present one of the largest case series of this rare condition.
- To raise awareness among clinicians regarding this potential overlap syndrome.
Main Methods:
- Retrospective cohort analysis and review of patients meeting diagnostic criteria for PACK syndrome.
- Analysis of demographic data, disease progression, and associated conditions.
- Prevalence calculation within a primary biliary cholangitis cohort.
Main Results:
- Fourteen patients diagnosed with PACK syndrome were identified, all female, predominantly White (86%), with an average age of 66.7 years.
- The prevalence of PACK syndrome in the PBC cohort was 5.08%.
- CREST syndrome preceded PBC diagnosis in 58% of patients; limited pulmonary and renal involvement were noted.
Conclusions:
- PACK syndrome is a rare but important overlap between CREST and PBC.
- Clinicians should consider PACK syndrome in patients with either CREST or PBC.
- Awareness of this syndrome's insidious development is vital for timely diagnosis and management.
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