Celiac disease associated with nodular regenerative hyperplasia, pulmonary abnormalities, and IgA anticardiolipin antibodies.
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.
Cancado
Eduardo Luiz R
EL
Department of Gastroenterology, University of Sao Paulo School of Medicine, Brazil. Sao Paulo, Brazil. edulrc@uol.com.br
Medeiros
Denise Machado
DM
Deguti
Marta Mitiko
MM
Dos Santos
Maira Solange Camara
MS
de Mello
Evandro Sobroza
ES
Vendramini
Margarete Borges Galhardo
MB
Carrilho
Flair Jose
FJ
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
D017153:Antibodies, Anticardiolipin