Autoimmune Hepatitis-Primary Biliary Cirrhosis: Overlap Syndrome Concomitant with Unexpected Myasthenia and Thymoma

Abstract

The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.

Overlap Syndrome and Myasthenia Gravis: An Uncommon Association With Unusual Features

“…Our patient had definite overlap syndrome according to the recent criteria, unlike other case reports. 1,10,15–19 If steroids were given at the initial diagnosis in our case in 2012 (based on high IgG and ANA positivity in lower titer), it could have slowed disease progression.…”

“…In the only other similar report of overlap syndrome associated with MG, both antiAChR and thymoma were present. 8,15 The thymus plays an important role in the growth and differentiation of T lymphocytes and its disorder can cause a spectrum of autoimmune diseases due to disturbance in cell-mediated immunity including synthesis of anti-AChR leading to MG. In our case, possibly anti-AChR was negative because MG occurred in the absence of thymoma.…”

Prevalence of Myasthenia progression in hepatic encephalopathy syndrome

“…Figure 3 illustrates the ammonia metabolism in both the muscles, kidneys, liver and brain [22]. Myasthenia is associated with cirrhosis such as primary cholangitis, hepatitis and primary sclerosing cholangitis [23,24]. Myasthenia is muscular weakness that has reduced physical activity, especially in adults started from ocular to the bulbar and skeletal muscles [25].…”