The Autoimmune Gender Gap: A Rare Case of a Male Patient With Overlapping Autoimmune Hepatitis and Primary Biliary Cholangitis.

Autoimmune hepatitis (AIH) is a condition resulting in chronic, inflammatory changes to the liver. Primary biliary cholangitis (PBC) is an autoimmune condition that destroys intrahepatic bile ducts. Overlap syndrome with concomitant AIH and PBC comprises a rare subgroup of patients with immune-mediated liver disease, with incidence rates of male patients being exceedingly uncommon in a predominantly female patient population. Our case report investigates a rare case of a 41-year-old male patient diagnosed with overlapping AIH and PBC. He initially presented with symptoms of fatigue, pruritus, and episodes of Raynaud's phenomenon, in addition to findings of persistently elevated liver enzymes despite lifestyle modifications. He had no past medical history, no history of alcohol use disorder, and no family medical history of chronic liver disease. Imaging did not reveal evidence of cirrhosis. Further diagnostic workup was significant for elevated immunologic markers for antinuclear antibodies (ANA) with positive centromere and cytoplasmic patterns, antimitochondrial antibodies (AMA) with F-actin antibodies, anti-smooth muscle antibodies (ASMA), and cytoplasmic antinuclear cytoplasmic antibodies (ANCA C). Liver biopsy showed prominent plasma cells and rare granulomas, consistent with the diagnosis of AIH with a component of PBC, respectively. He was started on ursodeoxycholic acid (UDCA), demonstrating a near-complete clinical response with resolution of symptoms and normalization of liver enzymes. Studies investigating the low incidence of male patients with overlap syndrome are limited, as current research is overwhelmingly based on studies with predominantly female subjects. However, most studies generally recommend treatment with both UDCA and corticosteroids to reduce symptoms and biochemical markers. Our case report highlights a rare case of a male patient documenting excellent biochemical and clinical responses to monotherapy with UDCA. A possible theory is that our patient's early treatment (prior to advanced disease progression) is associated with his near-complete biochemical response and symptomatic resolution on UDCA alone. Further research is needed to fully understand the clinical course and long-term prognosis of male patients with overlap syndrome. Our patient remains in life-long follow-up to monitor if or when he requires treatment with corticosteroids in addition to current monotherapy with UDCA.​.

A Closer Look at the HEART Score.

The history, electrocardiogram, age, risk factors, and troponin (HEART) score is currently a widely used tool for acute chest pain risk stratification. Relatively soon after its inception in 2008, a number of validation studies on the HEART score showed it to be superior to Thrombolysis in Myocardial Infarction (TIMI) and Global Registry of Acute Coronary Events (GRACE) scores and at least as accurate to other existing scores for predicting short-term major adverse cardiovascular events (MACEs). However, partly due to its focus on simplicity, the HEART score has some limitations. In this article we review how the HEART score has evolved and taken on various modifications to circumvent some of its limitations. We also highlight the strength of the HEART score in comparison with other risk stratification tools and the current guidelines.

A case of ventricular standstill in a patient with acute gastrointestinal bleeding.

Ventricular stand still is an electrophysiologic phenomenon characterized by the absence of ventricular activity or contraction. It is the result of the lack of impulse formation in ventricles (absence of idioventricular automaticity) or the failure of impulse transmission to ventricles (conduction disturbance) [1]. It is an uncommon condition that can affect a wide range of age groups with life threatening consequences. There are no set guidelines on the treatment of ventricular standstill - swift and sound clinical judgment is required. The condition should be treated as a cardiac arrest, with resolution of precipitating factors. Here we present a case of a 59-year-old man with multiple comorbidities, who presented with massive gastrointestinal hemorrhage and recurrent episodes of ventricular standstill during hospitalization, with his immediate treatment and stabilization.

SVEAT Score, a Potential New and Improved Tool for Acute Chest Pain Risk Stratification.

Acute chest pain is one of the most common presenting symptoms to the emergency department. Currently available risk scores are suboptimal in identifying low-risk patients eligible for early and safe discharge. Various, initially obtained clinical data have valuable discriminating power but are not being fully utilized. We developed a new scoring system based on 5 sets of variables; characteristics of Symptoms, history of Vascular disease,  Electrocardiography,  Age, and Troponin (SVEAT score). A total of 321 subjects presenting to the emergency department or admitted to the clinical decision unit at our institution with chest pain from May 2017 to August 2018 were prospectively recruited. The subjects were followed for 30 days for any major cardiovascular events (MACE); acute myocardial infarction, confirmed coronary artery disease requiring revascularization or medical therapy or death. A 30-day MACE occurred in 19.6% of the subjects. Predictive ability of SVEAT score for a 30-day MACE was compared with HEART and TIMI risk score using receiving-operator characteristic curve. The area under the curve of SVEAT score (0.98, 95% confidence interval [CI] 0.97 to 0.99) is higher than HEART (0.92, 95% CI 0.88 to 0.96) and TIMI score (0.88, 95% CI 083 to 0.93). Using SVEAT score of 4 as a cut off, 0.8% of the subjects developed a 30-day MACE compared with 1.4% and 1.5% of those classified as low-risk based on the HEART and TIMI score respectively. SVEAT score additionally identified larger proportion of low-risk (73.8%) than the HEART (45.2%) and TIMI risk score (40.1%), (p <0.01, for both).

A Rare Case of Carotid Web Presenting with Ischemic Stroke in a Young Woman and a Brief Review of the Literature.

Carotid web is a radiological description of a shelf-like intraluminal filling defect in the carotid bulb. It is histologically defined as atypical fibromuscular dysplasia (FMD), with abnormal fibrosis and smooth muscle cell hyperplasia in the tunica intima. The spur-like intraluminal protrusion can serve as a nidus for thrombus formation, which could cause systemic embolism and ischemic strokes. We report a case of a 20-year-old female patient presenting with acute ischemic stroke in the ipsilateral middle cerebral artery (MCA) territory. We also discuss the incidence, the prevalence, the pathophysiology, the treatment, and the recurrence of carotid web based on the currently available literature.

An Interesting Case and Literature Review of a Coronary Stent Fracture in a Current Generation Platinum Chromium Everolimus-Eluting Stent.

Coronary interventions are the mainstay of treatment for stenotic coronary vascular lesions. New stent designs are constantly being evaluated to improve stent performances and clinical outcomes. Coronary stent fracture is uncommon; however, it is associated with potential major consequences including acute coronary syndrome and the need for repeated target vessel revascularization due to in-stent restenosis or stent thrombosis. We report a case of a 66-year-old man with an extensive cardiac disease history, who presented with intractable angina and was found to have a fracture of a current generation, platinum chromium everolimus-eluting stent (Synergy, Boston Scientific Inc.).

Atypical Presentation of Acute Coronary Syndrome and Importance of Wellens' Syndrome.

BACKGROUND Acute coronary syndrome (ACS) is a common and potentially life-threatening condition encountered in emergency departments. Despite its dreaded nature, nearly one-third of ACS present without chest pain and may mislead clinicians. Additionally, Wellens' syndrome is a pre-infarction stage of significant proximal left anterior descending (LAD) artery stenosis, which can lead to extensive anterior wall myocardial infarction without timely intervention.  CASE REPORT We report the case of a 74-year-old woman presenting with isolated throat pain and Wellens' pattern in the initial EKG, which prompted the proper workup and management. Subsequently, coronary angiogram revealed more than 90% occlusion of the proximal LAD artery, and a drug-eluting stent was deployed. The patient did well after the procedure and the follow-up at 2 weeks after discharge was uneventful.   CONCLUSIONS This case highlights the importance of awareness of atypical presentation of ACS and importance of Wellens' syndrome. We also discuss the incidence of craniofacial symptoms of ACS, and the epidemiology, pathophysiology, management, and prognosis of Wellens' syndrome.

A Case of Right-Sided Direct Carotid Cavernous Fistula: A Diagnostic Challenge.

BACKGROUND Carotid cavernous fistulas (CCFs) are rare potentially sight-threatening abnormal connections between carotid artery and cavernous sinus. CASE REPORT We report a case of CCF in an 83-year-old female, who presented with swollen and painful right eye. The patient was initially treated with empiric antibiotics for suspected peri-orbital cellulitis, as noted clinically and in computed tomography (CT) orbits. However, lack of clinical improvement, physical finding of orbital bruit/thrill, and enlarged superior ophthalmic vein in magnetic resonance (MR) orbits suggest alternate diagnoses. Eventually, CT angiogram (CTA) and carotid-arteriography confirmed the diagnosis of right-sided direct CCF, which was subsequently treated with endovascular embolization. Not only does this case highlight the importance of CCF, which could be a differential diagnosis of swollen red eye, it also addresses the vital importance of physical examination in modern medicine despite the seemingly promising technologies. CONCLUSIONS Internists should have a low threshold of clinical suspicion for CCF in a patient with swollen red eyes in order to provide timely and proper management.

Coronary artery ectasia presenting with acute inferior wall myocardial infarction in a young adult.

Coronary artery ectasia (CAE), a variant of coronary artery anomalies, is a rare clinical entity. Although atherosclerotic coronary artery disease (CAD) is the most common cause of acute coronary syndrome (ACS), CAE also conveys a relatively high risk for potentially life-threatening cardiac events. We report a case of a 35-year-old male with two-vessel CAE, who presented with acute inferior wall ST election myocardial infarction (STEMI). After initiating medical therapy including bivalirudin, emergent percutaneous coronary intervention (PCI) with stenting of postero-lateral branch of right coronary artery (RCA) was performed. Coronary angiography also showed diffuse ectasia of RCA and left anterior descending artery (LAD). The patient tolerated the procedure well and was discharged on appropriate medical therapy. He was followed-up at one month, with no resulting cardiac events. This case highlights the importance of CAE awareness. CAE has drawn the attention of clinicians because of its clinical implications, as well as its seemingly higher prevalence, due to the abundant use of coronary angiograms and advanced cardiac imaging in the contemporary world. Albeit there has been much progress in the understanding and management of the disease, questions still remain regarding the exact pathophysiology, management guidelines and prognosis of CAE, which are worth further study.

A Case of Cardiac Calcified Amorphous Tumor Presenting with Concomitant ST-Elevation Myocardial Infarction and Occipital Stroke and a Brief Review of the Literature.

Cardiac calcified amorphous tumor (CAT) is an extremely rare benign intracavitary tumor of the heart. It may mimic other cardiac tumors and can present with signs or symptoms of systemic embolization. There are limited data regarding CAT in the literature. We report a case of a 68-year-old woman with a cardiac CAT and mitral annular calcification (MAC), who presented with acute ST-elevation myocardial infarction (STEMI) and occipital stroke. After extensive review of the literature, we believe that this case is possibly the first description of a cardiac CAT presenting with STEMI. The CAT was surgically removed, and the diagnosis was confirmed by histology. The patient tolerated the surgery and reported no events at 6-month follow-up.

A Case of Diffuse Alveolar Hemorrhage as a Possible Complication of Bivalirudin Therapy.

BACKGROUND Diffuse alveolar hemorrhage (DAH) is a rare but potentially fatal complication of anticoagulant or antiplatelet therapy. Bivalirudin is a specific and reversible direct thrombin inhibitor (DTI). CASE REPORT We report a case of severe DAH, possibly related to bivalirudin use, in a 61-year-old patient undergoing coronary intervention. The patient had presented with an out-of-hospital cardiac arrest due to acute ST elevation myocardial infarction (STEMI). During the coronary intervention, shortly after receiving bivalirudin, the patient started having frank bleeding from the endotracheal tube and developed hemodynamic compromise. Despite aggressive intervention and intensive care, the patient died. CONCLUSIONS At this time, to our knowledge, there have been no reports of DAH associated with the use of bivalirudin.