Sterile, recurrent, and bilateral corneal perforation related to primary biliary cirrhosis complicated by secondary Sjögren syndrome and vitamin A deficiency

ABSTRACT Primary biliary cirrhosis is a rare progressive autoimmune liver disease that causes chronic cholestasis. Of patients with primary biliary cirrhosis, 75% develop secondary Sjogren syndrome and could develop vitamin A deficiency. Here, we report the case of a patient with primary biliary cir...

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Published inArquivos brasileiros de oftalmologia
Main Authors Martín, Leyre Lloreda, Rocha-de-Lossada, Carlos, Marín-Martínez, Sara, Peraza-Nieves, Jorge Ernesto
Format Journal Article
LanguagePortuguese
Published Conselho Brasileiro de Oftalmologia 2021
Subjects
OPHTHALMOLOGY
Primary biliary cirrhosis
Corneal perforation
Cirrose hepática biliar
Deficiência de vitamina A
Doença autoimune
Autoimmune disease
Sjogren syndrome
Vitamin A deficiency
Síndrome de Sjogren
Perfuração da córnea
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Summary:ABSTRACT Primary biliary cirrhosis is a rare progressive autoimmune liver disease that causes chronic cholestasis. Of patients with primary biliary cirrhosis, 75% develop secondary Sjogren syndrome and could develop vitamin A deficiency. Here, we report the case of a patient with primary biliary cirrhosis who developed a secondary Sjogren syndrome and vitamin A deficiency, which led to severe and unusual eye involvement with multiple and recurrent spontaneous corneal perforations. Corneal perforations in patients with primary biliary cirrhosis and secondary Sjogren syndrome are rare but devastating complications, in contrast to other eye clinical manifestations of the disease.
ISSN:1678-2925
DOI:10.5935/0004-2749.20210100