A case of primary biliary cirrhosis associated with pernicious anemia: a case report

Abstract

Primary biliary cirrhosis is often associated with autoimmune diseases. However, its association with pernicious anemia has rarely been reported.We report a case of a 68-year-old woman who presented jaundice and pruritus. Mildly elevated serum levels of alkaline phosphatase and γ-glutamyl transpeptidase were detected. The titer of anti-mitochondrial M2 anti-body was elevated. Histology of liver biopsy showed features of primary biliary cirrhosis. In addition, aregenerative macrocytic anemia was found in the full blood count. The diagnosis of pernicious anemia was established by megaloblastosis in bone marrow, atrophic gastritis without Helicobacter pylori, low level of vitamin B12 and good response to treatment regimen of vitamin B12. The association of primary biliary cirrhosis and pernicious anemia is unlikely to be casual and may be explained by autoimmune mechanism commonly shared by the diseases.

Pernicious Anemia in Patients with Primary Biliary Cirrhosis, Autoimmune Hepatitis, and Chronic Viral Hepatitis

“…However, age, gender, and clinical features in two cases were not mentioned. The cases of concomitant PBC and PA are rarely reported; to the best of our knowledge, only five cases have been reported in the English and Japanese scientific literature [3,[15][16][17]23], excluding the two cases reported by Culp et al [22]. All five of these patients were females.…”

“…PA is a disease of complex autoimmune origin and is caused by the impaired absorption of vitamin B 12 due to the absence of IF, thus inducing megaloblastic anemia [1][2][3][4][5]. PA is relatively common in humans older than 60 years and is the most common cause of vitamin B 12 deficiency and megaloblastic anemia [2][3][4][5]. The autoimmune origin of PA is supported by the presence of autoantibodies for gastric parietal cells and/or IF [4].…”

“…However, PCA can be detected in other autoimmune diseases, such as autoimmune thyroid diseases [i.e., Hashimoto's thyroiditis (HT)], Addison's disease, and type I Diabetes Mellitus (DM), all of which are well documented as PA-associated autoimmune diseases [5,16,17]. Moreover, the incidence of PCA decreases due to the progression of autoimmune gastritis or at a later stage of PA [3,5]. Therefore, the detection of PCA may not be specific for PA [5,16].…”

“…All five of these patients were females. PBC was first diagnosed in two cases [15,23], and both diseases, PBC and PA, were diagnosed almost simultaneously in the remaining three cases [3,15,16]. The age of the patients at the time of diagnosis of both diseases ranged from 46 to 72 years, and the interval between the diagnoses of the primary and concomitant diseases ranged from 0 to 19 years.…”

“…Autoimmune Liver Diseases (ALDs) include Auto Immune Hepatitis (AIH), Primary Biliary Cirrhosis (PBC), and Primary Sclerosing Cholangitis (PSC). Pernicious Anemia (PA) involves an autoimmune process in which atrophy of the gastric mucosa reduces the production of Intrinsic Factor (IF) via parietal cells, inducing vitamin B 12 deficiency and megaloblastic anemia [1][2][3][4][5]. Although autoimmune diseases often coexist in patients, cases of concomitant PA and ALD are rarely reported.…”