Discussion: The patient in our case had recurrent abdominal pain caused by pancreatic duct stones resulting in pancreatic outflow obstruction. Both attempts using ERCP with balloon sweeping were unsuccessful in complete stone extraction leading to the use of POP Spyglass with laser lithotripsy for the management of the multiple remaining stones. Direct visualization of the PD with technology like Spyglass, has shown to reduce the risk of duct injury, allows the visualization of stones that may have been missed previously, and permits confirmation of clearance of the PD (Figure). As seen in this case, POP allowed for visualization of PD stones that could not be accessed through ERCP management and used in conjunction with laser lithotripsy, the PD stones were successfully and safely fragmented. POP-LL has been shown to successfully fragment difficult to manage PD stones, as evidenced in this case and other studies, and should be considered as a useful alternative in the management of numerous PD stones and/or multiple unsuccessful ERCP attempts.[1732] Figure 1. Laser lithotripsy with holmium laser being applied through the catheter (red arrow) to the PD stone (White arrow).

Introduction: Anti-mitochondrial antibody-positive primary biliary cholangitis (AMA-pos PBC) is an autoimmune disorder in which monoclonal antibodies are produced against epitopes with in the mitochondrial membranes of biliary epithelial cells, resulting in progressive non-suppurative biliary cholangitis. Up to 5 % of PBC patients lack these auto antibodies, termed as anti-mitochondrial antibody negative (AMA-neg) PBC. This is a somewhat new entity and a variant of AMA-pos PBC but not an overlap syndrome. There have not been good studies describing this phenomenon or associated terminology in the literature. Case Description/Methods: An 87-year-old woman was referred to our clinic after her medical oncologist found elevated isolated levels of serum alkaline phosphatase (714 units/L). She reported fatigue but denied any other symptoms. The physical examination was benign, except for bilateral lower extremity swelling secondary to lymphedema. Her serum alkaline phosphatase level decreased to 413 units/L after an initial dose of prednisone 40 mg daily, and she was maintained on 10 mg daily. Her antinuclear antibody titer was greater than 1:2560 in a centromere pattern. Anti-mitochondrial antibody was not detected. Total IgG level was 871 mg/dL (normal, , 1600 mg/dL), serum anti-smooth muscle antibody was negative, and the hepatitis panel was normal. Computed tomography of the abdomen and pelvis without contrast showed normal liver parenchyma and no acute intra-abdominal pathology. Histopathological examination indicated florid duct lesions. Background parenchyma showed no significant steatosis, and the inflammatory changes were limited primarily to the portal areas. Periodic acid-Schiff staining highlighted the intact hepatic parenchyma and architecture. The patient was diagnosed with AMA-neg PBC and responded well to Urosdeoxycholic acid therapy. (Figure) (Table). Discussion: This case highlights the importance of recognizing AMA-neg PBC as a variant of AMA-pos PBC and being able to differentiate them. Autoimmune cholangitis is a vague and imprecise term that cannot be used in this context. All AMA-negative PBC patients should be tested for other PBC-specific autoantibodies. Although prognosis and bile duct damage and loss are worse in AMA-neg PBC for unknown reasons, treatment remains the same for both.

Antimitochondrial antibody-positive primary biliary cholangitis (AMA-pos PBC) is an autoimmune disorder in which monoclonal antibodies are produced against epitopes in the mitochondrial membranes of biliary epithelial cells, resulting in progressive nonsuppurative biliary cholangitis. Up to 5% of patients lack these autoantibodies, termed antimitochondrial antibody-negative (AMA-neg) PBC. Although a somewhat new variant of AMA-pos PBC, it is not an overlapping syndrome. Few studies to date have described this phenomenon. An 87-year-old woman was referred to our clinic with elevated serum alkaline phosphatase (714 U/L). She reported fatigue but no other symptoms. A physical examination revealed a benign lesion and bilateral lower extremity swelling secondary to lymphedema. The serological profile was significant for a high antinuclear antibody titer (>1:2,560) with a centromere pattern and negative for antimitochondrial antibody (AMA). The hepatitis panel was negative for viruses A, B, and C. Her serum immunoglobulin G level was 871 mg/dL (normal, <1,600 mg/dL). The rest of the serological tests, including anti-smooth muscle antibodies (ASMA) and anti-liver/kidney microsomal antibodies, were negative. Computed tomography of the abdomen and pelvis without contrast showed normal liver parenchyma and no acute intra-abdominal pathology. Histopathology indicated florid duct lesions. The background parenchyma showed no significant steatosis, and inflammatory changes were limited to the portal areas. Periodic acid-Schiff staining revealed intact hepatic parenchyma and architecture. The patient was diagnosed with AMA-neg PBC and responded well to ursodeoxycholic acid therapy. This case highlights the importance of recognizing AMA-neg PBC as a variant of AMA-pos PBC and differentiating between them. Autoimmune cholangitis is a vague and imprecise condition. All patients with AMA-negative PBC should be tested for other PBC-specific autoantibodies. Although the prognosis and bile duct damage and loss are worse in AMA-neg PBC for unknown reasons, treatment remains the same for both.