Woman Diagnosed Postpartum With MG, Autoimmune Liver Disease
A 31-year-old woman was diagnosed with two autoimmune conditions — myasthenia gravis (MG) and a liver disease called primary biliary cirrhosis — after giving birth, a recent case study reported.
“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,” its scientists wrote.
The case study, “Primary biliary cirrhosis associated with myasthenia gravis after postpartum: a case report,” was published in the Journal of Medical Case Reports.
MG is an autoimmune disease marked by the immune system wrongly launching an attack against specific proteins involved in nerve-muscle communication, causing weakness and fatigue. Early symptoms may include eyelid droopiness and swallowing difficulties.
Studies suggest that both the risk of developing MG and its severity are higher in women after giving birth.
Primary biliary cirrhosis (PBC) is caused by the immune system wrongly attacking the small bile ducts of the liver, leading bile and other compounds to build to toxic levels, and eventually to liver scarring and cirrhosis.
Symptoms include fatigue and itchy skin, and are most commonly seen in middle-aged and older women. Previous studies also suggested that this disease’s risk and symptom severity are linked to changes in female sex hormones.
Investigators at the First Affiliated Hospital of Soochow University in China reported the rare case of a woman diagnosed with both autoimmune disorders, MG and PBC, after giving birth.
“This report firstly described the occurrence of MG and PBC in a patient in the postpartum period,” the team wrote.
Three months after giving birth to her second child via cesarean section, the woman, a 31-year-old Asian, developed slurred speech, drooping eyelids and, eventually, limb weakness. These symptoms worsened during periods of activity, and eased after rest.
In a physical exam, doctors saw a yellow discoloration to the skin of her face and in the whites of her eyes. Neurological examination confirmed she had mild speech problems, mild eyelid droopiness, and generalized muscle weakness after exercise, particularly in her lower limbs.
A test using repetitive nerve stimulation showed the transmission of electrical signals in the right axillary nerve — a nerve traveling from the armpit to the arm — was significantly impaired, as it was in the left facial nerve.
Her MG diagnosis was confirmed after blood tests showed very high levels of antibodies targeting the acetylcholine receptor — the most common type of self-reactive antibodies found in people with MG.
Further CT scans found a lower-than-usual density of the liver parenchyma — the functional tissue of the liver made up of specialized cells called hepatocytes. Blood tests also revealed high levels of liver enzymes, a sign of liver damage, and the presence of anti-mitochondrial antibody M2, consistent with PBC. As a result, she was also diagnosed with PBC.
Based on the MG diagnosis, the woman was treated with pyridostigmine bromide (brand name, Mestinon; generics available), as well as the anti-inflammatory methylprednisolone, which was gradually increased for three days, then decreased for maintenance treatment.
Clinicians treated PBC with ursodeoxycholic acid to improve her liver function, and with the immunosuppressant azathioprine.
Her condition steadily improved over the course of the month she remained hospitalized. After discharge, she continued on the same combined medications for both MG and PBC.
In the two years following her discharge, methylprednisolone’s dose was gradually reduced then discontinued within one year. She received daily ursodeoxycholic treatment for six months after discharge until she she eventually stopped this medication on her own. Her liver enzyme levels remained in the normal range, even after stopping with ursodeoxycholic acid.
She remained on long-term maintenance treatment with pyridostigmine bromide and azathioprine, with no signs of discomfort, except for weight gain.
“This case exhibits a rare condition of myasthenia gravis combined with primary biliary cirrhosis postpartum,” the scientists wrote, adding that “detailed physical and laboratory examination can help to prevent the missed diagnosis of these diseases.”