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 Focal Segmental Glomerulosclerosis in a Patient With Lupus Nephritis.

Systemic lupus erythematosus (SLE) is a persistent autoimmune disease where the immune system produces autoantibodies against nuclear and cytoplasmic antigens, leading to multi-organ involvement. Typically, nephrotic-range proteinuria is indicative of proliferative or membranous lupus nephritis. However, on rare occasions, patients with SLE and nephrotic syndrome may exhibit focal segmental glomerulosclerosis (FSGS) lesions upon kidney biopsy. We describe a 28-year-old Middle Eastern male with SLE and biopsy-proven lupus nephritis who experienced a significant increase in proteinuria and creatinine levels despite treatment with mycophenolate mofetil. Further investigation revealed FSGS without active lupus nephritis. The patient's treatment regimen was adjusted to rituximab in response to these findings. This case underscores the importance of renal biopsies in SLE management to accurately diagnose and tailor treatment. It highlights the need to consider alternative renal complications, such as FSGS, which can coexist with lupus nephritis, necessitating a collaborative approach between rheumatologists and nephrologists to achieve optimal patient outcomes.

The Rare Adverse Effect of Cefepime-Induced Neutropenia.

Cefepime is widely considered a safe and effective antibiotic, but it can have rare and serious side effects. We present a case of a 33-year-old female patient who developed severe and potentially life-threatening neutropenia after being on cefepime for 25 days. Despite extensive investigations, no other causes of neutropenia could be identified. Discontinuing the medication and administering a single dose of filgrastim produced a rapid and dramatic response. This case highlights the rare but serious risk of cefepime-induced neutropenia and underscores the need for clinicians to remain vigilant for this potential adverse effect. It is important to note that discontinuing the medication can rapidly reverse the effects, making timely recognition and intervention crucial for patient outcomes.

Miller Fisher Syndrome With Positive Anti-GQ1b/GQ1d Antibodies Associated With COVID-19 Infection: A Case Report.

The association between Guillain-Barré Syndrome (GBS) and its variants including Miller Fisher syndrome (MFS) has been reported and debated in the literature. Herein, we are reporting a 59-year-old male patient who had flu-like symptoms for 10 days prior to presentation with rapidly progressive weakness, dysphagia, and dysarthria. He tested positive for COVID-19 and further workup showed positive anti-GQ1b and GQ1d antibodies. The diagnosis of MFS was presumed and prompted the commencement of intravenous immunoglobulin (IVIG). Respiratory deterioration prompted intubation and failure of extubation necessitated plasmapheresis. This treatment culminated in successful extubation and discharge to a long-term care facility. This case adds to the currently limited body of cases that report the association of a rare GBS variant with COVID-19 infection. Only a few of the reported cases of COVID-19-related MFS cases had positive anti-GQ1b antibodies. This may well be the first reported case of COVID-19-related MFS with positive anti-GQ1b and anti-GQ1d antibodies.

Cortical Ribbon and Crossed Cerebellar Diaschisis in Subclinical Status Epilepticus: A Case Report.

Cortical ribbon is an uncommon finding that is characteristic of Creutzfeldt-Jakob disease but has a broad differential diagnosis. On the other hand, crossed cerebellar diaschisis is also an uncommon finding in brain magnetic resonance imaging (MRI). Herein, we are describing an 88-year-old male patient with dementia, ambulatory dysfunction, and frequent falls who presented with acute on chronic right-sided subdural hemorrhage that was discovered after an episode of seizure. Although the subdural hemorrhage was associated with mild midline shift and lateral ventricle compression, no surgical drainage was attempted, and only middle meningeal artery embolization was pursued. Lack of further evidence of seizure and clinical stability prompted discharge. However, he was soon re-admitted for left-sided focal seizure that failed multiple antiepileptic medications and evolved into status epilepticus. MRI brain showed evidence of both cortical ribbon as well as crossed cerebellar diaschisis. No evidence of infection or autoimmune inflammation was found with continuous mental status deterioration. Code status was changed by his family, and comfort care was pursued. This case is not only interesting because of the rarity of both cortical ribbon and crossed cerebellar diaschisis, but this case helps to remind clinicians of the relationship between these findings and seizure/status epilepticus.

Direct/Conjugated Hyperbilirubinemia as an Uncommon Presentation of Acute Alcoholic Hepatitis: A Case Report.

Alcoholic liver disease (ALD) is a common pathology in clinical practice and is clinically diverse. Acute alcoholic hepatitis is an acute inflammation of the liver with or without underlying cholestasis and steatosis. In this case, we are presenting a 36-year-old male with a past medical history of alcohol use disorder who presented with two weeks of right upper quadrant abdominal pain and jaundice. However, direct/conjugated hyperbilirubinemia with relatively low aminotransferases in labs prompted investigation for obstructive and autoimmune hepatic pathologies. Unrevealing investigations prompted consideration of acute alcoholic hepatitis with cholestasis and a course of oral corticosteroids that gradually improved the patient's clinical symptoms and liver function test. This case helps to remind clinicians that although ALD is usually associated with indirect/unconjugated hyperbilirubinemia and elevated aminotransferases, presentation of ALD with mainly direct/conjugated hyperbilirubinemia with relatively low aminotransferases is a possibility. Although imaging tests should be pursued to rule out obstructive etiologies, invasive tests and liver biopsies are not indicated in typical clinical settings.

Anti-Glutamic Acid Decarboxylase (GAD) 65 Encephalitis Mistaken for Herpes Encephalitis and Hashimoto's Encephalitis (HE): A Case Report.

Autoimmune encephalitis is increasingly recognized in clinical practice. We are presenting a 72-year-old female patient who initially presented with a new onset seizure with temporal lobe abnormality on imaging. This was initially attributed to herpes encephalitis although herpes polymerase chain reaction (PCR) was negative. The patient was treated with acyclovir and antiepileptic medication (AEM) with some clinical improvement. She presented again with refractory seizure evolving to status epilepticus. Escalation of AEMs was pursued and positive anti-thyroid peroxidase (TPO) antibody prompted consideration of Hashimoto's encephalitis (HE) and treatment with high-dose corticosteroids and intravenous immunoglobulin (IVIG). However, poor response to steroid argued against HE, and extended autoimmune encephalitis panel revealed positive anti-glutamic acid decarboxylase (GAD) antibody. This case raises the clinical pearl that anti-thyroid antibodies, e.g anti-TPO antibody, can be seen in those with autoimmune encephalopathies other than HE and HE remains a diagnosis of exclusion. It also helps to remind clinicians that a new onset refractory seizure even with temporal lobe changes is not pathognomonic for herpes encephalitis, and although negative serology does not rule out the diagnosis, it is recommended to rule out autoimmune encephalitis as it shares similar clinical and radiological picture.

The Clinical Challenge of Refractory Octreotide-Induced Thrombocytopenia in Active Gastrointestinal Bleeding: A Case Report.

The association between octreotide and thrombocytopenia has been documented in the literature but it remains a rare finding. We are reporting a 59-year-old female patient with alcoholic liver cirrhosis who presented with the gastrointestinal tract (GIT) bleeding secondary to esophageal varices. Initial management involved fluid and blood products resuscitation and initiation of both octreotide and pantoprazole infusion. However, the abrupt onset of severe thrombocytopenia was evident within a few hours of admission. Platelet transfusion and discontinuation of pantoprazole infusion failed to correct the abnormality prompting the holding off of octreotide. However, this also failed to control the decline in platelet count and prompted intravenous immunoglobulin (IVIG). This case helps to remind clinicians to closely monitor platelet count once octreotide is initiated. This allows early detection of the rare entity of octreotide-induced thrombocytopenia, which can be life-threatening with extremely low platelet count nadir.

A Spontaneous Spinal Epidural Hematoma Secondary to Long-Term Low-Dose Aspirin and Clopidogrel Use: A Case Report.

The association between antiplatelet agents such as aspirin, clopidogrel, and ticlopidine and spontaneous spinal epidural hematoma is based on multiple case reports in the literature. Here, we present the case of a 76-year-old male patient who presented with acute low back pain associated with sudden-onset paralysis of the lower extremities. His past medical history was remarkable for coronary artery disease with a stent placement history on dual antiplatelet therapy including low-dose aspirin and clopidogrel. An extensive posterior thoracolumbar epidural hematoma was seen on the imaging test, and rapid clinical improvement was evident early during his presentation. This prompted a conservative approach that led to complete neurological recovery. This case is in line with limited English-language literature evidence that suggests a possible association between spontaneous spinal epidural hematoma and antiplatelet agents. We aim to enhance clinicians' awareness of this clinical entity, association, presentation, and management.

Important Clues for the Diagnosis of Anti-LGI1-Antibody Autoimmune Encephalitis: A Case Report.

Anti-leucine-rich-glioma-inactivated 1 (LGI1) antibody autoimmune encephalitis is a rare autoimmune encephalitis. We present a 68-year-old female patient who initially presented with episodic confusion, hallucinations, behavioral changes, and unexplained hyponatremia. History was also remarkable for intermittent abnormal movement affecting the left upper extremity and face. She was initially thought to be suffering from dementia and was discharged home. However, progressive symptoms led to her second admission, where evidence of autonomic dysfunction with episodic bradycardia and persistent symptomatic orthostatic hypotension were evident. Generalized cortical hyperexcitability and subclinical seizures were seen. Diagnosis of LGI1 encephalitis was confirmed with a positive Anti-LGI1 antibody in the cerebrospinal fluid, and treatment with intravenous immunoglobulin and steroids improved her cognitive function. This case helps to highlight important features that should raise early clinical suspicion of LGI1 encephalitis, including unexplained progressive hyponatremia, autonomic dysfunction, and frequent refractory seizures. This can lead to earlier recognition of this condition, where earlier implementation of immunosuppressive therapy is linked to better clinical outcomes and brain structural preservation.

A Possible Case of Autoimmune Encephalitis After mRNA COVID-19 Booster Vaccine: A Case Report.

As the use of COVID-19 vaccines gains more prevalence, rare and uncommon side effects are reported in the medical literature. This is a case report of a 75-year-old male patient who presented on the second day after receiving the Moderna Bivalent mRNA COVID-19 booster vaccine with abrupt onset behavioral changes and global aphasia with no focal deficits. Stroke and infectious meningitis/encephalitis were ruled out. Signs of aseptic inflammation were seen on cerebrospinal fluid (CSF) analysis. Workup for autoimmune and paraneoplastic encephalitis was unyielding. The observation of rapid clinical improvement prompted watchful waiting that concluded in the resolution of clinical manifestations within less than a week of onset. This case is reported to support the currently limited knowledge of rare neurological sequelae of mRNA vaccine and is in line with recently published few cases that suggest vaccine-related encephalitis.

Lymphoplasmacytic Lymphoma/Waldenstrom Macroglobulinemia Masquerading as IgM Warm Antibody Autoimmune Hemolytic Anemia in Association With Mycoplasma pneumoniae Infection: A Case Report.

Warm antibody autoimmune hemolytic anemia (AIHA) is mostly of IgG subtype. IgM subtype is extremely rare and has not been reported in association with lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM). We are reporting the case of a 75-year-old female patient who presented with severe hemolytic anemia and Mycoplasma pneumoniae pneumonia (MPP). Cold agglutinin and serum protein electrophoresis (SPEP) were negative but immunofixation was positive for IgM. Ultimately, hemolytic anemia was labeled warm antibody AIHA in association with MPP. She presented again one year later with more severe hemolytic anemia. Persistently elevated IgM was seen in immunofixation and triggered bone marrow biopsy that confirmed LPL/WM. This case highlights the clinical pearl that warm antibody AIHA in association with MPP is a rare entity and more intensive investigation to rule out other etiologies is mandated. Also, this case is rare as it is of IgM subtype warm AIHA and observed in the context of LPL/WM.

Acute Ischaemic Stroke by a Different Mechanism.

Central venous lines are often inserted but their removal requires some precautions. We describe a 51-year-old female patient who was due for discharge but had an unfortunate event arising from removal of her central venous line even though the appropriate measures had been taken. She experienced an acute ischaemic stroke secondary to air embolism. The deficits persisted even though extensive efforts were made to reverse them. This case reminds clinicians that bedside procedures can result in devastating complications even if all precautions have been followed. We review the mechanisms of air embolism, provide suggestions to limit its incidence following central line removal, and recommend therapeutic measures. LEARNING POINTS: Precautions should be taken when removing a central venous line to reduce the risk of air embolism.Air embolism can manifest with acute stroke.Acute stroke in this setting requires hyperbaric oxygen therapy.