A Case of Macrophage Activation Syndrome Manifesting as the Initial Presentation of Systemic Lupus Erythematosus.

Macrophage activation syndrome (MAS) is a potentially fatal complication of an autoimmune rheumatologic disease characterized by overwhelming inflammation, multiorgan failure, and high mortality if untreated. We report a rare case of a 56-year-old man who presented with fever for three weeks and had a constellation of clinical features and laboratory findings, meeting the diagnostic criteria for systemic lupus erythematosus (SLE) and SLE-associated MAS. He was treated with high dose intravenous corticosteroid and hydroxychloroquine, resulting in resolution of fever and dramatic clinical improvement.

Hepatic Infarction in a Patient With Sickle Cell Trait Presenting With HELLP Syndrome.

Hepatic infarction is uncommon due to the dual blood supply from the hepatic artery and portal vein. The majority of the cases are caused following liver transplant or hepatobiliary surgery, hepatic artery occlusion, or shock. Hepatic infarction is a rare complication of hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome. HELLP is an obstetrical emergency requiring prompt delivery. The presence of elevated liver enzymes, mainly alanine aminotransferase and aspartate aminotransferase in pre-eclampsia, should warrant diagnosis and treatment in the line of HELLP syndrome. Our patient with underlying sickle cell trait presented with features of HELLP syndrome in her third trimester of pregnancy. She underwent cesarean delivery on the same day of the presentation. The liver enzymes continued to rise following delivery and peaked on postoperative day two. Contrast computed tomography scan showed multifocal hepatic infarctions. Pre-eclampsia by itself is a state of impaired oxygenation and can lead to hepatic hypoperfusion, and appeared to be a clear contributor to the hepatic infarction in this case. However, this case also raises the question of whether the underlying sickle cell trait might have potentiated the hepatic infarction. Although sickle cell disease is well known to cause hepatic infarctions, it is unknown whether the sickle cell trait affects the liver to a similar extent as sickle cell disease. In addition, there have been case reports of sickle cell trait causing splenic infarcts and renal papillary necrosis, but it remains unclear if it can be directly associated with hepatic infarction.

Pseudohyperkalemia Associated With Leukemia.

Elevated potassium levels can be a life-threatening emergency. We describe a case of falsely elevated serum potassium level in a patient with leukemia, which was suspected to be falsely elevated because the patient was asymptomatic with a normal electrocardiogram (EKG). Common reasons behind such a discrepancy in leukemia patients are the use of a tourniquet before collection, use of vacuum/pneumatic tubes for transportation, prolonged periods of incubation, use of heparin for sample collection, and processing of samples via centrifugation. Since the process is related to the method of collection and processing, we recommend using rapid point of care testing in such cases to differentiate between false and true potassium elevation, as it is a well-validated tool. Moreover, there is a good correlation between potassium measured with the blood gas, point of care, and central laboratory analyzers when the concentration of potassium is above 3 mEq/L.

Lambl's Excrescences Associated with Cardioembolic Stroke.

BACKGROUND Lambl's excrescences (LE) are threadlike fronds that occur along valve closure lines where minor endothelial damage occurs, resulting in thrombus formation. It is often asymptomatic but can result in cerebral embolism and coronary artery obstruction. The criterion standard for diagnosis is transesophageal echocardiography. CASE REPORT We report an interesting case of a 73-year-old right-handed man presenting with a visual disturbance. An MRI head demonstrated an acute-to-subacute infarct in the right posterior cerebral artery territory involving the posterior right temporal, right occipital lobes, and right thalamus, which was in keeping with embolic stroke. Investigation with transthoracic and transesophageal echocardiography showed echo density on the ventricle surface of the left coronary cusp, which was concerning for Lambl's excrescences, with no significant arrhythmia on his implantable loop recorder at 3-month follow-up. Therefore, we believe that his stroke was due to embolization from Lambl's excrescences. Since this was his first episode of stroke, monotherapy with aspirin was continued. CONCLUSIONS Although rare, Lambl's excrescences should be considered in the differential diagnosis of embolic stroke. However, there is no established guideline for its management. Patients with the first stroke episode can be treated conservatively with antiplatelet therapy. In patients with recurrent ischemic events, anticoagulation should be offered, along with discussion about surgical excision.

Rapidly Progressive Granulomatous Amoebic Encephalitis in a Diabetic Individual.

We present a case of rapidly progressive granulomatous amoebic encephalitis caused by Balamuthia mandrillaris in an individual with diabetes mellitus. Our patient presented with occipital headache, blurry vision, confusion, and gait imbalance of one week's duration. Brain imaging revealed numerous peripheral ring-enhancing lesions concerning malignancy. Brain biopsy was consistent with Balamuthia mandrillaris infection. He passed away 45 days after presentation despite being treated with a five-drug regimen. This case highlights the importance of considering amoebic brain infections, especially with ring-enhancing lesions on imaging. There are opportunities to design modalities for rapid diagnosis and better treatment.

Trends in hospitalizations for vertebral compression fracture in ankylosing spondylitis: data from the National Inpatient Sample 2000-2014.

Ankylosing spondylitis (AS) patients are at increased risk of vertebral compression fractures (VCF). Our objective was to examine the yearly trend of VCF hospitalizations in AS patients as compared to rheumatoid arthritis (RA) and the general population. National Inpatient Sample (NIS) database (2000-2014) was used to identify adult (≥ 18 years) hospitalizations, based on validated ICD-9 diagnosis codes. The rate of VCF hospitalizations, as a primary diagnosis, was assessed in three mutually exclusive groups: AS, RA, and the general population. The prevalence of VCF hospitalization was highest in AS (2.70%), compared to 0.77% in RA and 0.35% in the general population. Over the 15-year period, VCF hospitalization in AS was noted to have an increasing trend (Annual Percent Change (APC) = 4.73, p < 0.05) in contrast to the stable trend in the general population (APC = 0.34, p = NS) and a declining trend in RA (APC -3.61, p < 0.05). VCF related to AS was also associated with a longer hospital stay as compared to the general population (8.1 days vs. 5.1 days, p < 0.05) and higher inpatient mortality (3.4% vs. 1.0%, p < 0.05). A higher rate of VCF hospitalization along with an increasing trend was noted in AS as compared to RA and compared to the general population. Better screening approaches and treatment strategies for AS patients with VCF risk are urgently needed to reduce hospitalizations and related complications. Key Points • An increasing trend of VCF hospitalization was noted in AS, in contrast to a declining trend in RA and a stable trend in the general population. • VCF in AS was associated with longer hospital stay and higher inpatient mortality than in RA and the general population.

Necrotising myopathy and concurrent thyroiditis in a patient with COVID-19 infection.

A 76-year-old man with hypogammaglobulinemia on monthly intravenous immunoglobulin infusions presented to the hospital with fever, cough, and shortness of breath and was diagnosed with COVID-19 pneumonia requiring intensive care unit admission but not intubation. He was treated with convalescent plasma, remdesivir and corticosteroids. Sixteen days into his hospitalisation he began to report weakness without sensory symptoms and was found on biopsy to have a necrotising myopathy.

Pharmacotherapies in Heart Failure With Preserved Ejection Fraction: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: Heart failure (HF) with preserved ejection fraction (HFpEF) causes significant cardiovascular morbidity and mortality. It is a growing problem in the developed world, especially, in the aging population. There is a paucity of data on the treatment of patients with HFpEF. We aimed to identify pharmacotherapies that improve peak oxygen consumption (peak VO2), cardiovascular mortality, and HF hospitalizations in patients with HFpEF. METHODS: We conducted a systematic literature search for English studies in PubMed, EMBASE, Cochrane Central Register of Controlled Trials, Web of Science, Scopus, and Google scholar. We searched databases using terms relating to or describing HFpEF, stage C HFpEF, and diastolic HF and included only randomized controlled trials (RCTs). RevMan 5.4 (The Cochrane Collaboration, 2020, London, UK) was used for data analysis, and two independent investigators performed literature retrieval and data-extraction. We used PRISMA guidelines to report the outcomes. We included 14 articles in our systematic review and six studies in meta-analysis. RESULTS: We calculated the pooled mean difference (MD) of peak VO2 between placebo and pharmacotherapies. Our meta-analysis showed that the peak VO2 was comparable between pharmacotherapies and placebo in HFpEF (MD = 0.09, 95% CI: -0.11, 0.30, I2 =28%). Our systematic review highlights that statins and spironolactone use should be further studied in larger RCTs due to their potential beneficial effect on all-cause mortality and hospitalizations, respectively. CONCLUSION: Compared to placebo, none of the pharmacotherapies significantly improved peak VO2 in HFpEF except ivabradine. In our meta-analysis, the pooled improvement in peak VO2 is non-significant. This needs validation with larger studies. We are lacking larger studies on pharmacotherapies that improve peak VO2 in HFpEF. Statin and spironolactone should be further studied in patients with HFpEF as few trials have shown improvement in all-cause mortality and reduction in HF hospitalizations in selected patients, respectively.

Bell's Palsy as a Late Neurologic Manifestation of COVID-19 Infection.

Bell's palsy is acute peripheral facial nerve palsy; its cause is often unknown but it can be triggered by acute viral infection. Coronavirus disease 2019 (COVID-19) infection commonly presents with respiratory symptoms, but neurologic complications have been reported. A few studies have reported the occurrence of facial nerve palsy during the COVID-19 pandemic. We present a case of Bell's palsy in a 36-year-old man with COVID-19 infection and a past medical history of nephrolithiasis. He presented to the emergency room with a day history of sudden right facial weakness and difficulty closing his right eye four weeks following a diagnosis of COVID-19 infection. Physical examination revealed right lower motor neuron facial nerve palsy (House-Brackmann grade IV). Serologic screen for Lyme disease, human immunodeficiency virus (HIV), and herpes simplex virus (HSV) 1 and 2 were negative for acute infection; however, neuroimaging with MRI confirmed Bell's palsy. He made remarkable improvement following treatment with a course of valacyclovir and methylprednisolone. This case adds to the growing body of literature on neurological complications that should be considered when managing patients with COVID-19 infection.

Sustained Ventricular Tachycardia as a Harbinger of Cardiac Amyloidosis.

BACKGROUND Cardiac amyloidosis is an infiltrative cardiomyopathy caused by the extracellular deposition of insoluble precursor protein amyloid fibrils. These depositions of protein amyloid fibrils are found on the atria and ventricles and can cause a wide array of arrhythmias; however, sustained ventricular arrhythmias are quite uncommon. CASE REPORT A 71-year-old man with a history of hypertension developed a sudden onset of shortness of breath, profuse diaphoresis, lightheadedness, and presyncope. Upon emergency medical services' arrival, an initial electrocardiogram revealed wide complex tachycardia with a heart rate of 220 to 230 beats per min. He was subsequently given, in succession, magnesium, adenosine, and amiodarone with no change in heart rate or rhythm. Due to ongoing symptoms of diaphoresis and the development of dyspnea, he underwent direct current cardioversion and was converted from ventricular tachycardia to atrial fibrillation at controlled rates. A transthoracic echocardiogram and cardiac magnetic resonance imaging showed features suspicious for cardiac amyloidosis. A subsequent 99m technetium pyrophosphate single-photon emission computerized tomography scan revealed a grade 3 visual uptake and a heart-to-contralateral lung ratio of 1.92, consistent with transthyretin amyloidosis. The patient was treated with tafamidis and an implantable cardioverter-defibrillator for secondary prevention of ventricular arrhythmia. CONCLUSIONS This case highlights the need to consider cardiac amyloidosis in the differential diagnoses of patients with persistent ventricular arrhythmia and no prior history of heart disease.

Sweet's syndrome in a granulocytopenic patient with acute myeloid leukemia on FLT3 inhibitor.

INTRODUCTION: Gilteritinib is a FLT3 kinase inhibitor approved for FLT3-mutated acute myeloid leukemia (AML). We present a case of febrile neutropenia and neutrophilic dermatosis consistent with Sweet's syndrome (SS). CASE HISTORY: A 55-year-old woman presented with fever and skin lesions after 4 weeks of initiation of Gilteritinib for AML. She was febrile, pancytopenic and neutropenic with absolute neutrophil count (ANC) of 0.1x10E3/UI. Examination revealed reddish and violaceous rashes on her extremities. Pathology showed superficial dermal edema, widespread epidermal spongiosis and multiple neutrophils in the dermal infiltrate. Rash improved with prednisone 60 mg daily and started to flare with taper. She was still on Gilteritinib all this time. Gilteritinib was finally stopped due to non-response and possible contribution in flaring her SS. Shortly after, the patient succumbed to progressive disease and complications of sepsis. DISCUSSION: There have been reports of SS in neutropenic patients although SS is typically a neutrophilic dermatosis. The pathogenesis of SS in neutropenia remains uncertain. Our study represents an additional medication-associated cutaneous complication of AML therapy. Clinicians need to be aware of potential neutrophilic dermatoses with FLT-3 inhibition, even with peripheral neutropenia.

Herbaspirillum Infection in Humans: A Case Report and Review of Literature.

Introduction. Herbaspirillum seropedicae are Gram-negative oxidase-positive nonfermenting rods of Betaproteobacteria class, commonly found in rhizosphere. More recently, some Herbaspirillium species have transitioned from environment to human hosts, mostly as opportunistic (pathogenic) bacteria. We present a 58-year-old female with non-small-cell lung cancer (NSCLC) who presented with pneumonia and was found to have Herbaspirillum seropedicae bacteremia. Case History. A 58-year-old woman with NSCLC on Pralsetinib presented with fevers and rigors for 2 days. Coarse breath sounds were auscultated on the right upper lung field. Labs revealed leukopenia and mild neutropenia. CT chest revealed right upper lobe pneumonia. She was admitted for sepsis secondary to pneumonia and placed on broad spectrum antibiotics with intravenous piperacillin-tazobactam and vancomycin. The patient continued to have fever 2 days after admission (max: 102.8°F). Preliminary blood cultures grew Gram-negative rods. The patient continued to have temperature spikes on the 3rd day of antibiotics (T max 101.5°F). Blood cultures revealed oxidase-positive nonfermenting rods. The patient's antibiotic was changed to IV meropenem on the 4th day of hospitalization. Ultimately, on the seventh day of hospitalization, the blood culture was confirmed from outside lab as Herbaspirillum seropedicae. The patient started feeling better and defervesced after about 24 hours. Discussion. More recently, Herbaspirillum spp. have been recovered from humans. Our patient had Herbaspirillum bacteremia, and reported regularly cleaning her pond and weeding her garden with possible exposure to this environmental proteobacterium. Herbaspirillum may be more prevalent than earlier thought owing to misidentification. With the institution of appropriate antimicrobial therapy, the outcomes seem mostly favorable.

Diffuse exanthematous drug eruption associated with intravenous immunoglobulin.

Diffuse exanthematous drug eruption due to intravenous immunoglobulin (IVIg) is a rare adverse event reported only in case reports. We present a case of a 71-year-old woman with a diffuse maculopapular rash 5 days after receiving an IVIg infusion for treatment of chronic inflammatory demyelinating polyneuropathy. She was managed conservatively with antihistamines; she was already receiving prednisone 25 mg daily as part of treatment for the neuropathy. The rash resolved over 2 weeks.