SLE strikes the heart! A rare presentation of SLE myocarditis presenting as cardiogenic shock.

BACKGROUND: Although systemic lupus erythematosus (SLE) can affect the cardiovascular system in many ways with diverse presentations, a severe cardiogenic shock secondary to SLE myocarditis is infrequently described in the medical literature. Variable presenting features of SLE myocarditis can also make the diagnosis challenging. This case report will allow learners to consider SLE myocarditis in the differential and appreciate the diagnostic uncertainty. CASE PRESENTATION: A 20-year-old Filipino male presented with acute dyspnea, pleuritic chest pain, fevers, and diffuse rash after being diagnosed with SLE six months ago and treated with hydroxychloroquine. Labs were notable for leukopenia, non-nephrotic range proteinuria, elevated cardiac biomarkers, inflammatory markers, low complements, and serologies suggestive of active SLE. Broad-spectrum IV antibiotics and corticosteroids were initiated for sepsis and SLE activity. Blood cultures were positive for MSSA with likely skin source. An electrocardiogram showed diffuse ST-segment elevations without ischemic changes. CT chest demonstrated bilateral pleural and pericardial effusions with dense consolidations. Transthoracic and transesophageal echocardiogram demonstrated reduced left ventricular ejection fraction (LVEF) 45% with no valvular pathology suggestive of endocarditis. Although MSSA bacteremia resolved, the patient rapidly developed cardiopulmonary decline with a repeat echocardiogram demonstrating LVEF < 10%. A Cardiac MRI was a nondiagnostic study to elucidate an etiology of decompensation given inability to perform late gadolinium enhancement. Later, cardiac catheterization revealed normal cardiac output with non-obstructive coronary artery disease. As there was no clear etiology explaining his dramatic heart failure, endomyocardial biopsy was obtained demonstrating diffuse myofiber degeneration and inflammation. These pathological findings, in addition to skin biopsy demonstrating lichenoid dermatitis with a granular "full house" pattern was most consistent with SLE myocarditis. Furthermore, aggressive SLE-directed therapy demonstrated near full recovery of his heart failure. CONCLUSION: Although myocarditis during SLE flare is a well-described cardiac manifestation, progression to cardiogenic shock is infrequent and fatal. As such, SLE myocarditis should be promptly considered. Given the heterogenous presentation of SLE, combination of serologic evaluation, advanced imaging, and myocardial biopsies can be helpful when diagnostic uncertainty exists. Our case highlights diagnostic methods and clinical course of a de novo presentation of cardiogenic shock from SLE myocarditis, then rapid improvement.

Real world utilization of the myositis autoantibody panel.

OBJECTIVE: Myositis autoantibody panel results can offer diagnostic and prognostic information in patients with concern for idiopathic inflammatory myopathy (IIM). However, there has been widespread utilization of myositis autoantibody testing clinically, often in situations where concern for an IIM is unclear. We sought to determine ordering practices and factors predicting positive results on ordered myositis antibody panels. METHODS: We included all patients in the Duke University Health System who had a "myositis antibody panel" ordered from October 2014 through December 2016. Retrospective chart review was performed evaluating antibody positivity, provider specialty, ordering location, demographics, medical history, review of systems (ROS), physical examination (PE), and laboratory values. Fisher's exact and t test tests and backward multivariable regression analysis were performed for statistical analysis. RESULTS: There were 642 unique tests obtained with 114 positive autoantibodies (17.7%) over the 26-month period. Myositis-specific autoantibodies (MSAs) were the most common and anti-Mi-2 was the most frequent (40% of MSAs). Pulmonology providers ordered the majority of tests (383; 59.6%). Adult Rheumatology had the highest antibody positivity rate (34.3%, p=0.0001) among specialties with at least 10 panels ordered. In backward multivariable regression analysis, factors independently associated with a positive myositis antibody panel were chronic corticosteroid use (OR: 2.10, 95% CI: 1.30-3.38) and sclerodermoid skin changes (OR: 6.89; 95% CI: 2.02-23.47). CONCLUSION: The positivity rate of myositis antibody panel testing in this real-world clinical setting was 18%. Anti-Mi-2 antibody was the most frequent autoantibody present. Specific factors associated with positive results can be utilized to identify patients at higher risk for IIM. KEY POINTS: • Only eighteen percent of all myositis antibody panel tests ordered returned positive. • Anti-Mi-2 antibody was the most frequent autoantibody in our cohort. • Specific factors associated with positive results can help identify patients at higher risk for IIM, particularly for non-rheumatologists.

How Well Do Rheumatology Fellows Manage Acute Infusion Reactions? A Pilot Curricular Intervention.

OBJECTIVE: Infusible disease-modifying antirheumatic drugs (DMARDs) are commonly prescribed in rheumatology and other fields. There are no published formal educational curricula that rheumatology fellowship programs can use to teach infusion reaction management skills to fellows. We aimed to better understand this educational gap and implement and assess the effectiveness of an experiential curriculum on acute infusion reaction management. METHODS: We included current rheumatology fellows and recent graduates from 5 fellowship programs. Using a novel behavioral checklist, we assessed fellows' performance managing an infusion reaction in a simulation, followed by a didactic session focused on infusion reactions. Pre- and postsurveys assessed experiences to determine relevance, as well as attitudes and knowledge. RESULTS: Despite ubiquitous prescribing of infusible biologic DMARDs, >50% of fellows were uncomfortable managing infusion reactions. Only 11% of fellows reported infusion reaction training during fellowship, but 56% reported managing actual patient infusion reactions. In the simulated infusion reaction, fellows managed grade-1 reactions appropriately, but grade-4 reactions poorly, meeting <50% of objectives. All fellows discontinued the infusion in the setting of anaphylaxis, but only 56% administered epinephrine. There was no difference in performance or written knowledge by training year. All fellows felt more prepared to manage infusion reactions postcurriculum and were satisfied with the experience. CONCLUSION: We confirmed an education gap in rheumatology fellowship training regarding infusion reactions, both in knowledge and performance. We developed and implemented a brief experiential curriculum including simulation of a high-risk patient-care scenario. This curriculum was well received and is easily exportable to other programs.