Liver damage in primary biliary cirrhosis and accompanied by primary Sjögren’s syndrome: a retrospective pilot study

Abstract

IntroductionPrimary biliary cirrhosis (PBC) and primary Sjögren's syndrome (pSS) have been referred to as “generalized autoimmune epithelitis”. Indeed, the pathogenic mechanisms, clinical features, and optimal therapeutic approaches for them are not yet fully defined.Material and methodsA retrospective analysis was carried out on clinical data obtained from 302 inpatients newly diagnosed with PBC, pSS, or the coexistence of PBC and SS between May 2011 and December 2014. Forty-two patients with abnormal hepatic function were divided into the PBC group (n = 17), the coexistent group (PBC accompanied by SS, n = 13), and the pSS group (n = 12). Their clinical symptoms, laboratory data, and pathological features were collected and analyzed when they were first diagnosed. The clinical and laboratory data were collected at 0, 1, and 3 months after treatment.ResultsOf the 42 patients with abnormal liver function, 4 were male and 38 were female patients. Compared with the patients in the PBC group, the patients in the other 2 groups were more likely to have an elevated erythrocyte sedimentation rate (ESR) and serum immunoglobulin G (IgG) levels. Abnormal serum immunoglobulin M levels (IgM) were more frequent in the PBC group. Corticosteroids were effective in normalizing elevated liver enzyme levels in patients with SS and in those with coexistent conditions.ConclusionsThis pilot study suggests that patients with PBC, pSS, and PBC/SS coexistence and having liver function abnormality share similar symptoms, but have different pathogenesis and prognosis.

Laboratory risk factors for coexistent primary biliary cholangitis in patients with Sjögren’s syndrome: a retrospective study

“…It has been reported that 59% of patients with SS display cytoplasmic pattern positivity, and patients with autoimmune disease and cytoplasmic pattern positivity exhibit hepatic involvement [32]. Our study found that 85.4% of patients with SS ± PBC had a cytoplasmic pattern, which was similar to the 62.2-85.7% of patients with PBC reported in the literature [31,33] but higher than the 57.14% (4/13) of patients with SS ± PBC in the literature [33]. This may be due to the low case numbers in the literature, and the characteristic pattern of AMA (cytoplasmic granular type AC-21) was not separated from other cytoplasmic patterns in our study, which might have led to an overestimation of the results.…”

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

“…Of patients with PBC, 25% are asymptomatic at the time of diagnosis, but as the disease progresses, complications such as portal hypertension, osteoporosis, and liposoluble vitamin deficiency (vitamins A, D, E, and K) can occur (1,2) . PBC is associated with many other immune manifestations such as SS (75%) with eye involvement (2,3) , with AMA being positive in >90% of patients (3,5) . Our patient showed high AMA levels and, consequently to her disease, developed a severe secondary SS with multiple ocular perforations.…”

“…PBC is associated with many other immune manifestations such as and antimitochondrial antibodies (AMA), which occur in >90% of patients. Of patients, 75% develop secondary Sjogren syndrome (SS) with eye involvement (3) .…”

Type I renal tubular acidosis caused by Sjögren’s syndrome with hypokalemia as the first symptom: a case report

“…Indeed, 70% to 80% of patients complain of dry mouth; approximately 50% of patients present with intermittent alternating parotid swelling and pain involving one or both sides, and approximately 20% have liver damage, especially patients with autoimmune hepatitis or primary biliary cirrhosis. 9 SS occurs more often in women, and the age of onset is 40 to 50 years. SS can also occur in children.…”