# Primary Biliary Cholangitis With Pulmonary Manifestations and Concurrent Enterococcus Pneumonia: A Diagnostic Challenge Resembling Sarcoidosis or Silicosis

## TL;DR

A rare case of primary biliary cholangitis with lung issues and enterococcus pneumonia is described, highlighting diagnostic challenges.

## Contribution

This case report highlights a rare presentation of PBC with concurrent pulmonary manifestations and enterococcus pneumonia.

## Key findings

- PBC was diagnosed based on AMA presence and absence of biliary obstruction.
- Enterococcus pneumonia was identified via bronchoalveolar lavage and antibiotic response.
- The case was managed without invasive tests like MRCP or ERCP.

## Abstract

Primary biliary cholangitis (PBC) is common in females during middle age, presenting with fatigue and itching. In our case, an African-American male patient presented with abdominal pain, vomiting, fatigue, and lung manifestations such as interstitial lung disease, granulomatous lung disease, and pulmonary hypertension. In our case, the patient reported abdominal pain and fatigue with abnormal chest X-ray findings (bilateral pulmonic nodular lesion with calcifications), which mimicked silicosis/sarcoidosis lung findings such as bronchiectasis and parenchymal nodules. We diagnosed PBC as there was an absence of extrahepatic biliary obstruction and the presence of antimitochondrial antibodies (AMA) at a titer of 1:40 or higher. Bronchoalveolar lavage was performed due to the suspicion of interstitial lung disease and sarcoidosis, which was inconclusive but revealed enterococcus faecalis organisms. Initial antibiotic response heightens suspicion of infection, not colonization, leading to the diagnosis of enterococcal pneumonia. In our case, the diagnosis was made using clinical and laboratory criteria, and treatment with Ursodeoxycholic acid was opted for without resorting to more expensive and invasive tests like magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP). In summary, this case report presented the unique diagnostic challenges that will aid clinicians in considering a broad range of differential diagnoses and management plans.