Sandrine Barge1, Véronique Grando1, Jean-Charles Nault1,2, Chloé Broudin3, Michel Beaugrand1, Nathalie Ganne-Carrié1,2, Dominique Roulot4, Marianne Ziol2,3. 1. Service d'Hépato-Gastro-entérologie, Groupe hospitalier Paris-Seine-Saint Denis, Hôpital Jean Verdier, France et Université Paris 13, Bobigny, France. 2. Faculté de Médecine, INSERM UMR-1162, Génomique fonctionnelle des Tumeurs solides, IUH, France Université Paris Descartes, Paris, France. 3. Service d'Anatomie pathologique et Centre de Ressources biologiques, Groupe hospitalier Paris-Seine-Saint Denis, Hôpital Jean Verdier, France et Université Paris 13, Bobigny, France. 4. UF Hépatologie, Groupe hospitalier Paris-Seine-Saint Denis, Hôpital Avicenne, AP-HP, France et Université Paris 13, Bobigny, France.
Abstract
BACKGROUND: Nodular regenerative hyperplasia (NRH) is a rare histological disorder associated with a wide variety of systemic diseases. AIMS: We aimed (i) to report the prevalence of NRH in a database of liver biopsies (LBs) and the frequency of portal hypertension (PHT) at diagnosis, and (ii) to investigate whether associated diseases and/or specific histological lesions, including abnormalities of the microvasculature, were related to PHT. METHODS: Patients with a histological diagnosis of NRH, referred by seven clinical departments, were retrospectively selected. Clinical, biological, radiological, haemodynamic and endoscopic data at diagnosis were recorded. LBs were reassessed for microvascular abnormalities. RESULTS: NRH was diagnosed in 4.4% of LBs (n = 159, male: 52%, mean age: 54). Among patients referred for unexplained liver enzyme abnormalities, 15% had NRH. PHT was present at diagnosis in 45 patients (38%), including 13 with portal thrombosis; 65% of patients had an associated disorder. Obliteration of portal vein branches, observed in the LBs of 17 patients (11%), was significantly associated with PHT (P = 0.02). Periportal angiomatosis, observed in 101 patients (63%), was associated with the absence of PHT (P < 10(-4) ). CONCLUSION: We suggest that NRH is a frequent histological lesion in the setting of unexplained liver enzyme abnormalities. PHT is present at the time of diagnosis in 1/3 of patients regardless of the presence of associated disease. The frequency of periportal angiomatosis in NRH without obliteration of portal vein branches, and its association with the absence of PHT suggest that obstructive portal venopathy would not represent the most frequent mechanism involved in NRH.
BACKGROUND:Nodular regenerative hyperplasia (NRH) is a rare histological disorder associated with a wide variety of systemic diseases. AIMS: We aimed (i) to report the prevalence of NRH in a database of liver biopsies (LBs) and the frequency of portal hypertension (PHT) at diagnosis, and (ii) to investigate whether associated diseases and/or specific histological lesions, including abnormalities of the microvasculature, were related to PHT. METHODS:Patients with a histological diagnosis of NRH, referred by seven clinical departments, were retrospectively selected. Clinical, biological, radiological, haemodynamic and endoscopic data at diagnosis were recorded. LBs were reassessed for microvascular abnormalities. RESULTS: NRH was diagnosed in 4.4% of LBs (n = 159, male: 52%, mean age: 54). Among patients referred for unexplained liver enzyme abnormalities, 15% had NRH. PHT was present at diagnosis in 45 patients (38%), including 13 with portal thrombosis; 65% of patients had an associated disorder. Obliteration of portal vein branches, observed in the LBs of 17 patients (11%), was significantly associated with PHT (P = 0.02). Periportal angiomatosis, observed in 101 patients (63%), was associated with the absence of PHT (P < 10(-4) ). CONCLUSION: We suggest that NRH is a frequent histological lesion in the setting of unexplained liver enzyme abnormalities. PHT is present at the time of diagnosis in 1/3 of patients regardless of the presence of associated disease. The frequency of periportal angiomatosis in NRH without obliteration of portal vein branches, and its association with the absence of PHT suggest that obstructive portal venopathy would not represent the most frequent mechanism involved in NRH.
Authors: Berrie Meijer; Melek Simsek; Hans Blokzijl; Robert A de Man; Minneke J Coenraad; Gerard Dijkstra; Carin Mj van Nieuwkerk; Chris Jj Mulder; Nanne Kh de Boer Journal: United European Gastroenterol J Date: 2016-11-16 Impact factor: 4.623