Late–onset systemic lupus erythematosus-associated primary biliary cirrhosis

Abstract

BackgroundThe development of Primary Biliary Cirrhosis (PBC) during the course of Systemic Lupus Erythematosus (SLE) is extremely rare. We report the case of a geriatric woman who was diagnosed with SLE at 69 years of age then with primary biliary cirrhosis one year later.Case presentationA 70-years-old woman, who had been diagnosed with SLE at 69 years, was admitted for further examination of liver dysfunction. PBC was confirmed based on elevated serum levels of transaminase, high levels of antimitochondrial antibodies and following a liver biopsy. The oral administration of ursodeoxycholic acid stabilized the liver dysfunction.ConclusionWe described an original case report of elderly patient with coexisting PBC and SLE. To date, according to the best of our knowledge, there have been few case reports of SLE/PBC co-occurrence. The aetiology of this complex remains unknown, autoimmune mechanisms, environmental and genetic factors are considered important in the susceptibility to both diseases. Osteopontin might play an important role.

Clinical picture of late-onset systemic lupus erythematosus in a group of Polish patients

“…11, 12 Older patients frequently show positivity for rheumatoid factor, anti-Ro, and antiLa antibodies, while hypocomplementemia and the presence of anti-ds-DNA antibodies are uncommon in this group. 6,10,11 Cytopenias are more common in late-onset SLE.…”

An Atypical Syndrome in an Elderly Patient With Systemic Lupus Erythematosus, Sjögren Syndrome, and Primary Biliary Cirrhosis

“…10 Osteopontin, recognized as one of the spontaneous autoimmune models of SLE, may also be related. 11 Although the majority of patients have active disease and are on immunosuppressive therapy prior to developing PRES, they still require an increase or continuation of their immunosuppressive therapy to control disease activity after the onset of PRES.…”

Coexistence of Systemic Lupus Erythematosus and Primary Biliary Cirrhosis

“…In addition, the frequency of concomitant PBC in patients with SLE who have abnormal liver enzyme values and liver dysfunction is reportedly 0%-7.5% [23,27,29,30,32]. In addition, no obvious correlations between SLE activity and the incidence of PBC have been reported in patients with SLE [9,11,12]. In patients with concomitant SLE and PBC, SLE flare-up is not a usual sequel [11,12].…”

“…In addition, no obvious correlations between SLE activity and the incidence of PBC have been reported in patients with SLE [9,11,12]. In patients with concomitant SLE and PBC, SLE flare-up is not a usual sequel [11,12]. In fact, no reported cases experienced a flare-up of SLE after developing PBC among five SLE cases followed for the development of PBC [9,11,12,17,18].…”

“…Systemic lupus erythematosus (SLE) is an autoimmune disease that often coexists with other collagen disorders, such as rheumatoid arthritis (RA) and Sjögren's syndrome (SjS) [1,2]; however, cases of concomitant SLE and primary biliary cirrhosis (PBC) are rare [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. SLE is a multisystem autoimmune disease that results from a combination of genetic, environmental, and hormonal factors.…”