Primary biliary cholangitis associated with SARS-CoV-2 infection

Abstract

To the Editor:We would like to present the case of a female patient who developed primary biliary cholangitis (PBC) concomitant with Guillain-Barré syndrome (GBS) during severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. 1 A 44-year-old obese and hypertensive woman already suffering from Hashimoto's thyroiditis, was admitted to hospital with fever, cough and dyspnoea on March 18, 2020. Chest X-rays showed bilateral interstitial pneumonia and she had a positive SARS-CoV-2 nasopharyngeal swab. Because of clinical deterioration, the patient was intubated and treated in the intensive care unit with mechanical ventilation and the following drugs in chronological order: tocilizumab, ceftriaxone, azithromycin, darunavir/cobicistat, anakinra, remdesivir and fluconazole. After 2 weeks of mechanical ventilation, the patient's condition began to improve. She was extubated but, because of respiratory worsening, non-invasive ventilation was initiated. Then, she experienced a rapid cognitive deterioration (Glasgow coma scale 3) with good oxygen saturation and was reintubated. In the following days the state of consciousness improved with complete recovery, but she displayed a proximal hyposthenia at the 4 limbs without altered sensitivity; osteo-tendon reflexes were absent at the upper limbs, weakly evocable the patellars, present at the heels. The electromyography showed signs of radiculo-neuropathy with prevalent interesting of the proximal section of the four limbs and diffuse signs of active neurogenic sufferance with modest re-innervating activity. An acute motor axonal neuropathy (AMAN) subtype of GBS was diagnosed and the patient was treated with intravenous immunoglobulins with a progressive clinical improvement enabling transfer to a rehabilitation ward.During the hospitalization in intensive care, an important rise in serum gamma-glutamyltransferase (GGT) was observed with initially normal levels of alkaline phosphatase (AP) that progressively increased later (Fig. 1A). Serum bilirubin was normal or slightly augmented while alanine aminotransferase (ALT) tended to fluctuate between normal and slightly increased levels (28-120 IU/L, normal range 1-31). The patient had no history of previous increases in cholestastic indexes (last blood exams in 2015, including GGT and AP, were normal) or symptoms before COVID-19 infection. Family history was negative for autoimmune and hepato-biliary diseases; clinical examination was unremarkable. An abdominal ultrasound showed a slightly enlarged liver with moderate steatosis and a mildly enlarged spleen. Liver stiffness measured with Fibroscan was 9.1 kPa. Serology for hepatotropic viruses was negative, immunoglobulins were normal, while total cholesterol was slightly augmented (251 mg/dl, normally <200) as was low-density lipoprotein (LDL) cholesterol (174 mg/dl, normal <110). The patient tested positive for anti-nuclear (titre 1:160, cytoplasmic pattern) and anti-mitochondrial (titre 1:640) autoantibodies, but negative for anti-smooth muscle, anti-liver kidne...

Cholangiopathy as part of post-COVID syndrome

“…Autoimmune mediated conditions such as primary biliary cholangitis have been associated with post–COVID cholangiopathy [ 7 ]. Patients presented mild hyperbilirubinemia, lymphocyte infiltration in a liver biopsy as well as positivity for antinuclear (ANAs), and anti-mitochondrial antibodies (AMA) [ 7 ]. In our case, the liver biopsy did not show periportal lymphocyte infiltrates, and the panel of autoantibodies was negative.…”

Antimitochondrial Antibodies: from Bench to Bedside

“…A liver biopsy was performed and showed florid ductal lesions, therefore leading to the definite diagnosis of PBC. This was the first reported case of PBC induced by SARS-CoV-2, which is a RNA virus capable of inducing a profound activation of the immune system and also by the previous finding that infection by a human RNA beta-retrovirus, related to the mouse mammary tumor virus, was suggested as a possible trigger for PBC development [ 100 ].…”

“…Since early 2020, SARS-CoV-2 infection has dramatically changed our perspectives. Of note, a case of PBC developing during or soon after COVID-19 has been reported [ 100 ]. Remarkably, the patient was diagnosed after being admitted for severe COVID-19 with the need of mechanical ventilation and treatment with tocilizumab, ceftriaxone, azithromycin, darunavir/cobicistat, anakinra, remdesivir, and fluconazole.…”

Systematic review and meta-analysis of clinical outcomes of COVID-19 patients undergoing gastrointestinal endoscopy

“…4 COVID-19 is not only able to cause respiratory illness, but may also lead to gastrointestinal (GI) diseases, for example, enteritis, 5 , 6 pancreatitis, 7 and cholangitis. 8 The potential mechanism might be that SARS-CoV2 binds to the angiotensin-converting enzyme-2 (ACE2) receptors, which are widely expressed in lung, intestine, and liver. 9 , 10 Hence, COVID-19 may have significant impact on the clinical outcomes of GI endoscopic procedures, including disease transmission, rate of fatality, complications, and procedural success.…”