Coexistence of nodular regenerative hyperplasia of the liver and pulmonary arterial hypertension in patients with connective tissue diseases: report of three cases and review of the literature

Hiroyuki Watabe; Tohru Akahoshi; Jun Okada; Shigehiro Kokubu; Yuko Kurihara; Hirobumi Kondo

doi:10.1007/s10165-006-0514-2

Coexistence of nodular regenerative hyperplasia of the liver and pulmonary arterial hypertension in patients with connective tissue diseases: report of three cases and review of the literature

Mod Rheumatol. 2006;16(6):389-94. doi: 10.1007/s10165-006-0514-2. Epub 2006 Dec 20.

Authors

Hiroyuki Watabe¹, Tohru Akahoshi, Jun Okada, Shigehiro Kokubu, Yuko Kurihara, Hirobumi Kondo

Affiliation

¹ Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, 1-15-1 Kitasato, Sagamihara, Kanagawa 228-8555, Japan. [email protected]

PMID: 17165002
DOI: 10.1007/s10165-006-0514-2

Abstract

Nodular regenerative hyperplasia of the liver (NRH) is known to be a rare condition in patients with connective tissue diseases (CTD). In this report, we document three patients with CTD who had both NRH and pulmonary hypertension (PH). All three patients developed PH during their course and thereafter developed NRH. The clinical course of these patients suggests that circulatory disturbance caused by PH might be a trigger for NRH.

Publication types

Case Reports

MeSH terms

Adolescent
Aged
Connective Tissue Diseases / complications*
Connective Tissue Diseases / pathology
Connective Tissue Diseases / physiopathology
Fatal Outcome
Female
Focal Nodular Hyperplasia / complications*
Focal Nodular Hyperplasia / pathology
Focal Nodular Hyperplasia / physiopathology
Hepatocytes / pathology
Humans
Hypertension, Pulmonary / complications*
Hypertension, Pulmonary / pathology
Hypertension, Pulmonary / physiopathology
Pulmonary Artery / pathology*
Pulmonary Artery / physiopathology
Radiography, Thoracic