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Nodular Regenerative Hyperplasia of the Liver: A Rare Vascular Complication in Systemic Sclerosis
Laura Graf1,2, Rucsandra Dobrota1,2, Suzana Jordan1,2
1From the Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
Objective:
To investigate nodular regenerative hyperplasia (NRH) as a vascular complication of systemic sclerosis (SSc) with microvasculopathy as a common denominator.
Methods:
Cases of SSc-NRH were identified by systematic literature review and by screening the Zurich cohort. NRH had to be diagnosed by liver biopsy.
Results:
Literature review retrieved 22 cases. In our cohort, 1.4% of patients with SSc were diagnosed with NRH. Most had vasculopathy, were positive for anticentromere antibodies, had elevated alkaline phosphatase and gamma-glutamyl transferase levels, normal liver morphology on ultrasound yet increased stiffness on ultrasound elastography, and had portal hypertension.
Conclusion:
NRH might represent a rare yet potentially life-threatening vascular complication in SSc.
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