Nodular regenerative hyperplasia of the liver: survival and associated features in a UK case series.

AIMS: Nodular regenerative hyperplasia (NRH) is a rarely identified liver disorder. It is characterized histologically by nodular hepatocyte regeneration without significant fibrosis, and clinically by portal hypertension and abnormal liver function tests (LFTs). Survival data in an unselected cohort after diagnosis of NRH have not been previously described. This study aims to identify a regional cohort with NRH, to determine survival after diagnosis and to assess the relative frequency of associated conditions.
METHODS: Patients were identified retrospectively from liver biopsy reports within pathology databases, over a 13-year period from Glasgow, Scotland, UK. Case notes were retrieved, clinical information extracted and survival was determined.
RESULTS: Forty-two patients were identified (19 males). Common presenting features were abnormal LFTs (predominantly cholestatic) (76%) and portal hypertension (9.5%). None had severe liver dysfunction (Child-Pugh score A: 81%, B: 19%, C: 0%). Varices were detected in 26%, and portal hypertension was detected in 31%. There were five (12%) variceal bleeds, one fatal. The patients were subdivided into four groups according to associated clinical conditions: malignancy (29%), prothrombotic (21%), rheumatological (24%) and idiopathic/other (26%). Mean survival was 8.1 years, although survival was highly variable, and was associated with age and associated disease, but not with portal hypertension or varices. No patients in the rheumatological subgroup died.
CONCLUSION: NRH is usually associated with malignant, prothrombotic or rheumatological conditions. Survival is highly variable and related to age and the underlying disease process, but not to portal hypertension overall. Liver function remains well preserved.