Abstract
Although autoimmune diseases often coexist, concomitant cases of systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC) are uncommon. In this review paper, 34 cases of SLE with concomitant PBC found in English and Japanese scientific literature and Japanese proceedings were reviewed and summarized, including cases with liver dysfunction complicated by SLE. Of the 34 reported concomitant cases of SLE and PBC, 97.1% (33/34) were females, and PBC was diagnosed initially in 69.0% (20/29), except for five cases in which both SLE and PBC were simultaneously diagnosed. Sjögren's syndrome was the most common autoimmune disease complicating concomitant SLE and PBC (23.5%, 8/34). Five deaths have been reported: two elderly patients died of liver failure because of the worsening of PBC, and another two patients died from pulmonary infection associated with SLE pharmacotherapy. It is uncertain whether concomitant cases occur by chance or share a common immunological or genetic basis.
Highlights
Autoimmune diseases exhibit an increased immune response to self-antigens and predominantly occur in females [1]
The case report by Tunccan et al [35], which is a case of leishmaniasis with clinical and laboratory features mimicking Systemic lupus erythematosus (SLE) and autoimmune liver disease, was excluded from this analysis, because autoimmune reactions induced by leishmaniasis are common, and autoimmune reactions were thought to be induced by leishmaniasis in this particular case [35]
A single-nucleotide polymorphism (SNP) at nt position 9,250 (C–T) in exon 7 in OPN was highly associated with SLE, Kikuchi et al [77] reported that symptoms and pathologic stages of primary biliary cirrhosis (PBC) did not correlate with the variation of this SNP, suggesting no associations between this polymorphism and susceptibility to PBC in Japan
Summary
Autoimmune diseases exhibit an increased immune response to self-antigens and predominantly occur in females [1]. Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that results from a combination of genetic, environmental, and hormonal factors [3,4,5]. It is characterized by the presence of pathogenic autoantibodies such as anti-double stranded DNA or anti-histones and immune complexes in the serum and target tissues, inducing serious inflammatory conditions by the activation of the complement system [3, 4]. A literature search and a review of cases of SLE with concomitant PBC, including autoimmune liver diseases, were conducted to clarify the clinical features of concomitant SLE and PBC cases
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