Abstract Title: Unveiling the Cholestatic Phenotype: A Case of Autoimmune Hepatitis with Severe Hyperbilirubinemia 

Background: Autoimmune hepatitis (AIH) is defined by clinical, laboratory, and histologic findings as a hepatitic syndrome rather than a cholestatic syndrome. Cholestasis in AIH is typically due to associated primary biliary cirrhosis or primary sclerosing cholangitis. However, 8% cases of AIH have unexplained cholestasis (the Cholestatic-phenotype). In this case report, we described a patient who presented with cholestasis but was later diagnosed with AIH, responding well to steroid treatment. 

Case Presentation: A 52-year-old woman presented to the emergency department with a 15-day history of yellowing of the eyes and urine, accompanied by fatigue and pale-stools, following a brief febrile illness. Initial treatment with empirical antibiotics and supportive care at a local health center showed no improvement. Her medical history was unremarkable except for a similar episode of jaundice 4 years prior. Physical examination was normal, but laboratory tests revealed normocytic normochromic anemia, markedly elevated bilirubin (total 27 mg/dl, direct 15 mg/dl, indirect 12 mg/dl), and liver enzymes (AST-239 IU/L, ALT - 93 IU/L). Further evaluation showed a positive antinuclear antibody (ANA) profile with antibodies against SS-A, Scl-70 and Jo-1, but negative for anti-LKM, anti SMA and AMA. Liver ultrasound revealed altered liver echotexture without strictures or other abnormalities. Liver biopsy demonstrated features of auto-immune hepatitis(Figure 1), with interface hepatitis, cholestasis, chronic inflammatory infiltrates and portal fibrosis. Based on the Revised original scoring system for AIH, the patient had a pretreatment score of 15 (Simplifed scoring system score of 8) ,confirming the diagnosis. Treatment with azathioprine and prednisolone was initiated, with subsequent follow-up showing a positive response. Bilirubin levels normalized within two months. 

Discussion: For patients with cholestasis, especially with elevated bilirubin and positive ANA profile, AIH should be one of the differential diagnosis. Even if the classic serological markers like anti LKM and anti-SMA are negative, biopsy is the key to confirm the diagnosis in patients with such atypical presentation. The standard treatment for AIH is immunosuppressive therapy using steroids and steroid sparing agent such as azathioprine. Steroids such as prednisolone reduces the inflammation acutely and azathioprine is used for long-term chronic therapy to maintain the remission without the fear of steroid dependence.

Unveiling the Cholestatic Phenotype: A Case of Autoimmune Hepatitis with Severe Hyperbilirubinemia

Background
Alpha-1 Antitrypsin Deficiency (AATD) is an underrecognized genetic condition affecting an estimated 1 in 2,000 to 1 in 5,000 individuals worldwide, with approximately 70,000 to 100,000 cases reported in the United States. Alpha-1 Antitrypsin (AAT) plays a crucial role in protecting the lungs by inhibiting the activity of neutrophil elastase (NE), but in cases of AAT deficiency, uncontrolled NE activity can lead to lung damage through quantum proteolysis. Recurrent spontaneous pneumothorax is a significant complication observed in patients with underlying lung diseases like AATD and chronic obstructive pulmonary disease (COPD). The association between AATD and spontaneous pneumothorax remains relatively unexplored, presenting unique challenges in managing recurrent pneumothorax in these patients. This case report aims to provide valuable insights into the clinical features, diagnostic workup, and therapeutic interventions and associated challenges in managing a patient with recurrent spontaneous
pneumothorax in the context of AATD and COPD, with the objective of offering diagnostic and therapeutic considerations for similar cases.

Case Presentation
61 yo M with A1AT deficiency with severe bullous disease, no known cirrhosis, OSA intolerant of CPAP, hx of recurrent Lt pneumothorax and associated COPD presented with dyspnea. Examination revealed absent lung sounds on the left side. Oxygen support was initiated, and a chest x-ray confirmed left-sided pneumothorax. The patient underwent a successful left-sided pigtail catheter placement procedure to relieve the pneumothorax. This was his fourth episode of Secondary Spontaneous Pneumothorax. Two episodes occurred when he was younger, and the most recent two episodes required the implantation of a left chest tube. The very last episode
appeared on 5/2021. The patient's history of heavy smoking, substance abuse, and occupational asbestos exposure contributed to the severity of respiratory issues. Although his condition improved with pulmonary rehabilitation and medical therapy, the limitations of available interventions underscore the need for individualized approaches in complex cases. Lung volume reduction surgery and endobronchial valves were not viable due to extensive lung involvement and airway hyperdynamic collapse. The recommendation for lung transplantation aimed to enhance the quality of life and life expectancy. Pleurodesis emerged as a potential option if transplantation was not feasible. Further investigations were conducted for the transplant workup, leading to approval by the Medical Review Board. Following the successful placement of the Heimlich valve, the patient was discharged with instructions for outpatient follow-up.

Discussion
This case emphasizes the association between AATD and Recurrent secondary spontaneous pneumothorax, bringing attention to the difficulties encountered in the management of this condition. Further research is needed to optimize treatment approaches, explore the efficacy of alternative interventions, and understand long-term outcomes in this complex patient population.

Alpha-1 Antitrypsin Deficiency Unleashed:
A Case Report On Tackling Recurrent Secondary Spontaneous Pneumothorax

Gastroenterology / Hepatology (Clinical Vignettes)

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### Grand Prize Sponsor

AMA would like to thank Laurel Road for sponsoring a $10,000 grand prize for the winner of the AMA Research Challenge. Laurel Road is not involved in the judging or scoring process to select the winner. The winner will be announced on February 4, 2024 during the AMA Research Challenge finals. Payment of the grand prize will be directly from Laurel Road. See the AMA Research Challenge Official Rules for terms and conditions.

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Unveiling the Cholestatic Phenotype: A Case of Autoimmune Hepatitis with Severe Hyperbilirubinemia

Alpha-1 Antitrypsin Deficiency Unleashed: A Case Report On Tackling Recurrent Secondary Spontaneous Pneumothorax

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