Persistent left superior vena cava is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities including atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cor triatriatum. The presence of PLSVC can render access to the right side of heart challenging via the left subclavian approach, which is a common site of access utilized when placing pacemakers and Swan-Ganz catheters. Incidental notation of a dilated coronary sinus on echocardiography should raise the suspicion of PLSVC. The diagnosis should be confirmed by saline contrast echocardiography.

BackgroundANCA-associated vasculitis [AAV] is a small-medium vessel vasculitis that presents in a multi-systemic fashion, and is associated with significant mortality. Outcomes have improved with the introduction of immunosuppressive medications [ISM], the evidence supporting the initial choice ISM in AAV is limited, and may be influenced by health disparities related to social factors.ObjectivesThe objective of this study was to compare various factors known to influence management and outcomes in AAV. Factors including, patient demographics, socioeconomics, clinical presentations, and medication choices were reviewed in relation to outcomes within the cohort.MethodsThis was a retrospective, single centre, hospital-affiliated cohort study. A list of all patients seen by the Rheumatology service between 2011 and 2016 with a diagnosis of AAV was generated. A review of the 3000 charts confirmed 77 patients that met the 1990 American College of Rheumatology criteria for AAV. General demographic data including age, gender, zip code, and median household income as well as disease related data including serology, disease manifestation, and treatment were obtained through a chart review and recorded in the database. Supplemental socioeconomic information for each patient zip code was obtained from the United States Government Census website.ResultsIn our cohort the anti-proteinase 3 [PR3] antibody was the most common positive antibody. There was a relationship between PR3 antibody positivity rate and body mass index [table 1]. Given the low number of non-Caucasians we were unable to comment on the relationship between antibody and race.Medication choice and mortality were independent of mean household income. Rituximab was the most commonly prescribed ISM [44%]. The median age of rituximab exposure patients was 65 years and 60 years for unexposed patents. There was no significant relationship between age and medication choice. Rituximab was prescribed 60% of the time in renal AAV [p<0.01] and 53% of the time in pulmonary AAV [p<0.01]. Other commonly prescribed medications in the cohort included Mycophenolate [20%], Methotrexate [24%] and Cyclophosphamide [28%].The overall mortality rate was 17% [13/75] [figure 1]. There was no significant difference in the mortality rate of patients 65 years and older [23%, 9/39] compared to patients 64 and younger [11%, 4/36; p=0.17]. The mortality rate was also independent of median household income.Abstract AB0645 – Table 1PR3 antibodyMPO antibody NegativePositiveNegativePositive BMI<29.969%31%71%29%BMI 30–39.935%65%75%25%BMI>4020%80%100%N/Ap-value<0.010.38BMI: body mass index, PR3: proteinase 3, MPO: MyeloperoxidaseAbstract AB0645 – Figure 1ConclusionsThere was no relationship between age, sex, income, immunosuppressive therapy, and mortality in our AAV cohort. The mortality rate was higher in more severe disease and rituximab was more commonly used in such cases as well as in older patients. The limitations of the study include this being a hospital based cohort, where social demogr...

Humoral immune responses can produce autoantibodies against self-cellular proteins and nucleic acids without the presence of autoimmune diseases. Numerous kinds of autoantibodies are detected in liver diseases such as viral hepatitis, alcoholic liver disease (ALD), and nonalcoholic fatty liver disease (NAFLD), where their production could be secondary to hepatocellular inflammation and necrosis. Hence, the presence of an autoantibody does not necessarily indicate the presence of autoimmune disease; nor does it predict its severity and potential response to therapy. In literature, the spectrum and methods of diagnosis of liver disease in lupus are well described. However, chronic liver disease can manifest with signs similar to those in lupus, and it is important to recognize that autoantibodies in patients with chronic liver disease can be seen without the presence of autoimmune rheumatic disease. In this report, we discuss a very interesting case of a middle-aged female with a history of ALD presenting with calciphylaxis, thrombocytopenia, hypocomplementemia, and positive serologies, but without any clinical evidence of autoimmune rheumatic disease.