AUTOIMMUNE CHOLESTATIC LESIONS OF BILIARY DUCTS

The review presents literature data and original findings of light and electron microscopy of pathomorphological changes in the bile ducts in primary sclerosing cholangitis (PSC), immunoglobulin G4 (IgG4)-associated autoimmune sclerosing cholangitis and overlap syndromes: PSC + chronic autoimmune hepatitis (AIH); PSC + primary biliary cirrhosis (PBC).

Primary biliary cirrhosis associated with myasthenia gravis after postpartum: a case report

Background Autoimmune diseases refers to a class of diseases involving abnormal immune response of human body and tissue damage caused by the dysregulation of autoimmune balance or destruction of immune tolerance. Recent research has revealed that the occurrence of autoimmune diseases is influenced by genetic, hormonal, immunological, and environmental factors. As sex hormone levels change obviously during pregnancy and postpartum, the morbidity and recurrence rate of autoimmune diseases increase during this period. Case presentation A 31-year-old Asian woman was admitted to our hospital for myasthenia gravis and treated with methylprednisolone and pyridostigmine bromide 3 months postpartum. Physical examination and laboratory inspection after admission suggested that the patient had primary biliary cirrhosis. Subsequently, azathioprine was added to the treatment, and the symptoms of both diseases were successfully controlled. Conclusions This case exhibits a rare condition of myasthenia gravis combined with primary biliary cirrhosis postpartum. Given the fluctuation of the immune status during the postpartum period, combined autoimmune diseases need to be taken into account when patients develop clinical symptoms of an autoimmune disease. Therefore, detailed physical and laboratory examination can help to prevent the missed diagnosis of these diseases.

Joint Analysis of Genetic Correlation, Mendelian Randomization and Colocalization Highlights the Bi-Directional Causal Association Between Hypothyroidism and Primary Biliary Cirrhosis

Background: Hypothyroidism and primary biliary cirrhosis (PBC) are often co-existed in observational epidemiological studies. However, the causal relationship between them remains unclear.Methods: Genetic correlation, Mendelian randomization (MR) and colocalization analysis were combined to assess the potential causal association between hypothyroidism and PBC by using summary statistics from large-scale genome-wide association studies. Various sensitivity analyses had been conducted to assess the robustness and the consistency of the findings.Results: The linkage disequilibrium score regression demonstrated significant evidence of shared genetic architecture between hypothyroidism and PBC, with the genetic correlation estimated to be 0.117 (p = 0.006). The OR of hypothyroidism on PBC was 1.223 (95% CI, 1.072–1.396; p = 2.76 × 10−3) in MR analysis with inverse variance weighted (IVW) method. More importantly, the results from other 7MR methods with different model assumptions, were almost identical with that of IVW, suggesting the findings were robust and convincing. On the other hand, PBC was also causally associated with hypothyroidism (OR, 1.049; 95% CI, 1.010–1.089; p = 0.012), and, again, similar results can also be obtained from other MR methods. Various sensitivity analyses regarding the outlier detection and leave-one-out analysis were also performed. Besides, colocalization analysis suggested that there existed shared causal variants between hypothyroidism and PBC, further highlighting the robustness of the results.Conclusion: Our results suggest evidence for the bi-directional causal association between hypothyroidism and PBC, which may provide insights into the etiology of hypothyroidism and PBC as well as inform prevention and intervention strategies directed toward both diseases.

Urisodeoxycholic Acid in Treatment of Liver Cirrhosis

Ursodeoxycholic acid is a dihy- droxy bile acid with a rapidly expanding spectrum of usage in acute and chronic liver diseases. The various mechanisms of action of this hydrophilic bile acid include direct cytoprotection, detergent action on dysfunctional microtubules, immunomodulation and induction of hypercholer- esis. Its efficacy in primary biliary cirrhosis and primary sclerosing cholangitis as an adjunct to medical therapy has been well established.Ursodeoxycholic acid prolongs survival in primary biliary cirrhosis and it improves biochemical parameters of cholestasis in various other cholestatic disorders including primary sclerosing cholangitis, intrahepatic cholestasis of pregnancy, cystic fibrosis and total parenteral nutritioninduced cholestasis. However, a positive effect on survival remains to be established in these diseases. Ursodeoxycholic acid is of unproven efficacy in non- cholestatic disorders such as acute rejection after liver transplantation, non-alcoholic steatohepatitis, alcoholic liver disease and chronic viral hepatitis. This review outlines the present knowledge of the Pharmacology of ursodeoxycholic acid, and presents data from clinical trials on its use in chronic liver diseases. Keywords: Liver cirrhosis, Urisodeoxycholic acid

Unilateral Trigeminal Sensory Neuropathy with Sjögren’s Syndrome with Liver and Renal Impairment

Liver and renal involvement is a rare event in Sjögren’s syndrome. Sjögren’s syndrome is characterized by the progressive immune-mediated destruction of epithelial tissues of the salivary and lacrimal glands. Sensory ganglionitis, accompanied by T-cell invasion, occurs in patients with Sjögren’s syndrome, resulting in sensory neuropathy of the face or limbs. Patients are assessed by quantitative sensory testing. A 76-year-old woman presented with numbness of her left face and was subsequently diagnosed with Sjögren’s syndrome and primary biliary cirrhosis, and was found to have renal failure. Detection of her serum anti-Ro/SSA antibody was strongly positive. Shirmer’s test or a salivary volume in the gum test also showed positive results. Her somatosensory disturbance severity was higher in the trigeminal area than in the forearm, suggesting that the trigeminal nerve is more susceptible than other parts of the nervous system in patients with Sjögren’s syndrome and primary biliary cirrhosis. A simple sensory test could be performed during regular check-ups, as sensory deficits might develop after patients are diagnosed with Sjögren’s syndrome and primary biliary cirrhosis.