PT - JOURNAL ARTICLE AU - Laura Graf AU - Rucsandra Dobrota AU - Suzana Jordan AU - Lukas Martin Wildi AU - Oliver Distler AU - Britta Maurer TI - Nodular Regenerative Hyperplasia of the Liver: A Rare Vascular Complication in Systemic Sclerosis AID - 10.3899/jrheum.170292 DP - 2018 Jan 01 TA - The Journal of Rheumatology PG - 103--106 VI - 45 IP - 1 4099 - http://www.jrheum.org/content/45/1/103.short 4100 - http://www.jrheum.org/content/45/1/103.full SO - J Rheumatol2018 Jan 01; 45 AB - 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.