A Case of Primary Biliary Cholangitis in a Patient With Multiple Sclerosis.

Multiple sclerosis (MS) is a chronic inflammatory disease that causes demyelination in the brain and spinal cord, leading to significant neurological disability in young adults. Patients with MS are predisposed to other autoimmune disorders, though the co-occurrence of MS and primary biliary cholangitis (PBC) is rare. PBC is an autoimmune liver disease that affects bile ducts, leading to cholestasis and liver cirrhosis, predominantly in women aged over 40 years. We report the case of an 81-year-old woman with a history of MS and hypertension, bedridden for 10 years, who was admitted with a severe sacral ulcer and bacteremia. During hospitalization, she developed persistent itching, and elevated liver enzymes were detected. Imaging ruled out cholecystitis but revealed a large gallstone and hepatomegaly. Elevated M2 antimitochondrial antibodies confirmed PBC. The patient was treated with ursodeoxycholic acid, leading to symptom improvement. This case highlights the necessity for a thorough evaluation of autoimmune comorbidities in patients with MS and suggests a potential genetic and environmental link between MS and PBC. Further research is needed to explore this association and improve treatment strategies.

Evaluating the Usage of Janus Kinase Inhibitors in Rheumatology and Its Impact on Cardiovascular Risk.

Background/purpose Janus kinase (JAK) inhibitors have been widely used in treating rheumatological conditions like rheumatoid arthritis (RA) and psoriatic arthritis (PsA). Despite their efficacy, there are concerns regarding major adverse cardiovascular events (MACE) and venous thromboembolism (VTE) associated with JAK inhibitors. This study aimed to evaluate the risk of MACE, VTE, and the impact on lipid profiles in patients being treated with JAK inhibitors. Methods We retrospectively reviewed electronic medical records of patients aged 45-65 years old treated with Tofacitinib, Baricitinib, or Upadacitinib in a rheumatology clinic. We collected data on demographics, comorbidities, medication use, laboratory results, and cardiac complications potentially related to JAK inhibitors. Results Among 100 patients prescribed JAK inhibitors, 71 were included in the study (with an average treatment duration of 2.5 years). The majority of patients were white (72%), followed by Hispanic (6%), Indian (11%), African American (10%), and Asian (1%). Patients were being treated primarily for RA (57%), followed by PsA (17%), colitis (20%), and alopecia areata (6%). There were no significant cases of VTE reported, with one patient developing a pulmonary embolism (PE) during treatment while also having COVID-19, making it difficult to attribute it solely to the medication. Similarly, only one case of atrial fibrillation occurred. However, 43% (31 patients) experienced worsening of their lipid profile, with increased cholesterol (18%), LDL (12.5%), both LDL and cholesterol (11%) or triglycerides (1.5%). In relation to diabetes mellitus (DM), 24 patients who experienced worsening of their lipid panel did not have a history of DM. Conclusion The study findings suggest that patients on Tofacitinib, Baricitinib, and Upadacitinib did not exhibit a high risk for MACE or DVT. However, there was a notable incidence of lipid panel worsening among patients, where 24 patients out of 31 did not have diabetes. Further research and monitoring may be needed to better understand the long-term effects of JAK inhibitors on cardiovascular health and lipid profiles in these patient populations. This real-world data reflects the current evidence that JAK inhibitors do not significantly raise the risk of MACE in patients with RA but do increase cholesterol levels in these patients that should be monitored closely.

Upper Gastrointestinal (GI) Manifestations of Inflammatory Myositis: A Tale of Two Patients.

Myositis is a group of rare autoimmune disorders characterized by chronic inflammation of skeletal muscles that leads to a hallmark triad of muscle weakness, fatigue, and myalgia. Extra-muscular manifestations are sometimes seen and involve various organ systems, including the gastrointestinal (GI) tract. In this case series, two patients with polymyositis (PM) and dermatomyositis (DM), both of whom developed dysphagia as a complication of myositis, are discussed. Case 1 was a female with a known history of biopsy-proven dermatomyositis who presented with progressive peripheral edema and weakness affecting all extremities. Concurrently, she displayed symptoms of pneumonia and dysphagia associated with frequent spontaneous or self-induced vomiting to alleviate retrosternal discomfort. Esophagogastroduodenoscopy (EGD) revealed esophageal dilatation and an absence of a contractile response, consistent with myositis. Treatment comprised intravenous immunoglobulin (IVIG), mycophenolate, and lifestyle modifications, including dietary adjustments and maintaining an upright position postprandial. The second case was a female with muscle weakness and dysphagia. Video-fluoroscopic swallow assessment was significant for pharyngeal dysfunction without a sensory response to penetrated material, and the patient was at high risk of aspiration with any oral intake. The presence of pharyngeal dysfunction and dysphagia prompted treatment with IVIG, mycophenolate, and percutaneous endoscopic gastrostomy (PEG) tube placement. These cases have highlighted the upper GI complications observed in patients with myositis, accentuating the necessity for a personalized treatment approach. Timely intervention has shown promising results in symptomatic relief and improving patient outcomes. This emphasizes the importance of a multidisciplinary approach when addressing myositis-related upper GI manifestations.

Unmasking Carcinoid Syndrome in a Chronic Obstructive Pulmonary Disease (COPD) Patient: A Rare Presentation with Wheezing and Angioedema.

Carcinoid syndrome is a rare condition resulting from neuroendocrine tumors (NETs) that secrete vasoactive substances like serotonin. This report describes the case of a 61-year-old man with a history of chronic obstructive pulmonary disease (COPD) and hypertension who presented with new-onset angioedema, loss of consciousness, and a fall. He had been treated for COPD exacerbations during ER visits without improvement and was unaware of a prior mesenteric carcinoid tumor diagnosis from 2012. The next emergency evaluation revealed significant airway and facial edema necessitating intubation. Imaging and biopsy identified a well-differentiated grade 1 NET with extensive liver metastases. Laboratory tests showed elevated levels of serum serotonin, chromogranin A, and 24-hour urine 5-hydroxyindoleacetic acid (5-HIAA). Post-discharge, a PET scan confirmed metastatic lesions primarily in the liver and small bowel, with an unresectable mesenteric mass. The patient was treated with lanreotide and became symptom-free. This case underscores the need to consider carcinoid syndrome in patients with COPD presenting with unexplained respiratory symptoms, as timely diagnosis and treatment can significantly enhance patient outcomes.