title: Celiac disease associated with nodular regenerative hyperplasia, pulmonary abnormalities, and IgA anticardiolipin antibodies.
abstract: The association of nodular regenerative hyperplasia with celiac disease is not as well established as it is with hepatopulmonary syndrome and portopulmonary hypertension. IgA anticardiolipin antibodies were reported recently in celiac patients with nodular regenerative hyperplasia. The subject of this study was the description of pulmonary abnormalities and IgA anticardiolipin antibodies in celiac patients with noncirrhotic portal hypertension. Five patients with portal hypertension were investigated to diagnose its etiology. Celiac disease was diagnosed by means of autoantibody reactivity and duodenal biopsies. Liver histology revealed nodular regenerative hyperplasia in four patients and suggested its presence in 1 case. Two cyanotic patients had severe hypoxemia with a confirmed diagnosis of hepatopulmonary syndrome. Another case exhibited features of hepatopulmonary syndrome with increased levels of arterial pulmonary pressure. The remaining 2 cases had slight abnormalities of arterial oxygenation. Three patients had reactivity to IgA anticardiolipin antibodies. The concomitance of celiac disease and nodular regenerative hyperplasia, two infrequent conditions, raises suspicion of there being a nonfortuitous coincidence. Pulmonary abnormalities, and especially hepatopulmonary syndrome, are described for the first time in association with celiac disease and nodular regenerative hyperplasia.
authors.affiliation: Department of Gastroenterology, University of Sao Paulo School of Medicine, Brazil. Sao Paulo, Brazil. edulrc@uol.com.br
authors.forename: Eduardo Luiz R
authors.initials: EL
authors.lastname: Cancado
authors.forename: Denise Machado
authors.initials: DM
authors.lastname: Medeiros
authors.forename: Marta Mitiko
authors.initials: MM
authors.lastname: Deguti
authors.forename: Maira Solange Camara
authors.initials: MS
authors.lastname: Dos Santos
authors.forename: Evandro Sobroza
authors.initials: ES
authors.lastname: de Mello
authors.forename: Margarete Borges Galhardo
authors.initials: MB
authors.lastname: Vendramini
authors.forename: Flair Jose
authors.initials: FJ
authors.lastname: Carrilho
mesh_terms: D000293:Adolescent; D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000032:analysis*; D002446:Celiac Disease / Q000150:complications / Q000276:immunology*; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000150:complications / Q000276:immunology*; D020065:Hepatopulmonary Syndrome / Q000150:complications / Q000276:immunology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged
chemical_list: D017153:Antibodies, Anticardiolipin