Background SLE manifestations after ESRD may be underdiagnosed and undertreated, contributing to increased morbidity and mortality. Whether specific symptoms persist after ESRD or a shift towards new manifestations occurs has not been extensively studied, especially in the non-Caucasian patients in the United States. In addition, hydroxychloroquine (HCQ) prescribing patterns post-ESRD have not been described. The objective of this study was to assess lupus activity and HCQ prescribing before and after ESRD development. Knowledge gained from this study may aid in the identification of SLE manifestations and improve medication management post-ESRD. Methods We performed a retrospective cohort study of SLE patients with incident ESRD between 2010 and 2017. SLE-related symptoms, serologic markers of disease activity, and medication use were collected from medical records before and after ESRD development. Results Fifty-nine patients were included in the study. Twenty-five (43%) patients had at least one clinical (non-renal) SLE manifestation documented within 12 months before ESRD. Of them, 11/25 (44%) continued to experience lupus symptoms post-ESRD; 9 patients without clinical or serological activity pre-ESRD developed new symptoms of active SLE. At the last documented visit post-ESRD, 42/59 (71%) patients had one or more clinical or serological markers of lupus activity; only 17/59 (29%) patients achieved clinical and serological remission. Thirty-three of 59 (56%) patients had an active HCQ prescription at the time of ESRD. Twenty-six of the 42 (62%) patients with active SLE manifestations post-ESRD were on HCQ. Patients who continued HCQ post-ESRD were more likely to be followed by a rheumatologist (26 [87%] vs 17 [61%], p = 0.024), had a higher frequency of documented arthritis (10 [32%] vs 1 [4%], p = 0.005), CNS manifestations (6 [20%] vs 1 [4%], p = 0.055), and concurrent immunosuppressive medication use (22 [71%] vs 12 [43%], p = 0.029). Conclusions Lupus activity may persist after the development of ESRD. New onset arthritis, lupus-related rash, CNS manifestations, low complement and elevated anti-dsDNA may develop. HCQ may be underutilized in patients with evidence of active disease pre- and post ESRD. Careful clinical and serological monitoring for signs of active disease and frequent rheumatology follow up is advised in SLE patients both, pre and post-ESRD.

Background: Hydroxychloroquine or chloroquine with or without the concomitant use of azithromycin have been widely used to treat patients with SARS-CoV-2 infection, based on early in vitro studies, despite their potential to prolong the QTc interval of patients. Objective: This is a systematic review and metanalysis designed to assess the effect of hydroxychloroquine with or without the addition of azithromycin on the QTc of hospitalized patients with COVID-19. Materials and methods: PubMed, Scopus, Cochrane and MedRxiv databases were reviewed. A random effect model meta-analysis was used, and I-square was used to assess the heterogeneity. The prespecified endpoints were ΔQTc, QTc prolongation > 500 ms and ΔQTc > 60 ms. Results: A total of 18 studies and 7179 patients met the inclusion criteria and were included in this systematic review and meta-analysis. The use of hydroxychloroquine with or without the addition of azithromycin was associated with increased QTc when used as part of the management of patients with SARS-CoV-2 infection. The combination therapy with hydroxychloroquine plus azithromycin was also associated with statistically significant increases in QTc. Moreover, the use of hydroxychloroquine alone, azithromycin alone, or the combination of the two was associated with increased numbers of patients that developed QTc prolongation > 500 ms. Conclusion: This systematic review and metanalysis revealed that the use of hydroxychloroquine alone or in conjunction with azithromycin was linked to an increase in the QTc interval of hospitalized patients with SARS-CoV-2 infection that received these agents.

Background:Hydroxychloroquine (HCQ) cardiotoxicity remains an underrecognized condition. Diagnosis ultimately relies on invasive endomyocardial biopsy (EMB) and non-invasive screening methods are warranted. Strain imaging is a novel tool to detect early subclinical left ventricular (LV) dysfunction and may have a role in screening for HCQ cardiotoxicity (1). Strain measures systolic deformation indices that when decreased can predict cardiovascular outcomes more accurately than LV ejection fraction (2).Objectives:We assessed whether high HCQ cardiotoxicity risk is associated with a specific strain pattern in a group of patients with SLE and end-stage renal disease (ESRD).Methods:This was a retrospective study in a tertiary care center in New York on a group of patients with an established diagnosis of SLE, ESRD and cardiomyopathy on the index echocardiogram followed between years 2003 and 2019. The patients were stratified into two groups: high risk HCQ toxicity group was defined as either cumulative HCQ dose ≥1000g and/or an endomyocardial biopsy confirming HCQ toxicity. Low/moderate risk group was defined as a cumulative dose of HCQ <1000g. Clinical, demographic, electrocardiographic and echocardiographic strain parameters were compared between the groups.Results:A total of 16 patients were included. Two patients had EMB consistent with HCQ induced toxicity and 3 patients had cumulative HCQ doses ≥1000g. There were no significant differences in the baseline demographic characteristics between the two groups. Compared to patients with low/moderate risk, patients in the high risk group had a lower heart rate at the time of the echocardiogram (69 vs 87 beats per minute, p=0.08) and a higher frequency of LV hypertrophy (40% vs 9.1%, p=0.2). Strain analysis showed that both groups had compromised LV global longitudinal strain (GLS) and global cross-sectional strain (GCS). However, compared to the low/moderate risk group, the high risk group had a weaker LV GLS (-12.3% vs -14.9%, p=0.27).Characteristics overall and stratified by HCQ risk groupCharacteristicOverall (n=16)Low/Moderate HCQ Risk(n=11)High HCQ Risk and/or Positive EMB (n=5)P valueDemographicsAge, years47.5 (36.5,60.7)50.0 (33.9,60.5)42.5 (42.7,61.0)0.95Female, n(%)14 (87.5)9 (90)5 (83.3)0.99Clinical FeaturesSLE duration, years7.4 (4.3,17.5)5.5 (3.5,13.2)15.6 (11.6,19.3)0.15HCQ cumulative dose, g422.8 (224.2,422.8)285.4 (110.8,523.6)1140 (1006,1625.4)0.005HCQ therapy duration, years3.4 (2.5, 8.9)3.2 (1.5,5.1)7.8 (6.8,11.6)0.06HCQ daily dose, mg/d226 (200,394.9)200 (179.4,253.7)400 (389.8,400)0.007Hypertension, n (%)14 (87.5)10 (90.9)4 (80.0)0.99Diabetes, n (%)3 (18.8)2 (18.8)1 (20.0)0.99CAD, n (%)3 (18.8)2 (18.8)1 (20)0.99EchocardiogramEF, %55 (42.5,60)55 (40,60)55 (55,70)0.45LA size, cm3.8 (3.4,4.3)3.8 (3.4,4.2)4.3 (3.4,4.9)0.30LVEDD, cm4.9 (4.4, 5.5)4.8 (4.2,5.5)5.0 (4.9,5.4)0.43E/E’12.3 (8.8,16.3)12 (8.8,14.9)16.9 (4.9,21.8)0.43Moderate-severe LV hypertrophy, n(%)3 (18.7)1 (9.1)2 (40.0)0.20Strain echocardiographyGLS, %-13.9 (-16.7,-12.3)-14.9 (-16.7,-12.9)-12.3 (-15.4,-12.2)0.27Base/Apex Ratio0.8 (0.7,0.9)0.76 (0.68,0.86)0.76 (0.66,0.83)0.95GCS, %-20.2 (-21.7,-17)-19.7 (-20.5,18.0)-21.7 (-23.9,-20.9)0.16RV GLS, %-20.19 (-22.1,-17.5)-20.2 (-22.3,-17.1)-19.8 (-22.1,-17.5)0.99Conclusion:We report an association of higher HCQ cardiotoxicity risk and impaired strain in a set of SLE ESRD patients. Standard echo measures did not differentiate between high and low/moderate risk patients. Although the findings did not reach statistical significance, given the small sample size, results are still suggestive of a possible utility of strain echocardiography for detection of early HCQ toxicity.References:[1]Buss SJ, et al. J Rheumatol. 2010;37(1):79-86[2]Kalam K, et al. Heart. 2014;100(21):1673-80Disclosure of Interests: None declared

Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with Systemic Lupus Erythematosus (SLE). BSLE can present as the initial manifestation of SLE and may be a marker of severe disease. In this case report, we present a case of a 22-year-old African American woman with BSLE and impaired renal function with subsequent nephrotic range proteinuria concerning for lupus nephritis and autoimmune hemolytic anemia, refractory to systemic corticosteroids, immunoglobulin, and mycophenolate mofetil, requiring dapsone after careful desensitization due to prior history of angioedema with sulfa drugs. This case highlights the importance of the prompt recognition of BSLE as the initial manifestations of SLE and illustrates the association of BSLE with severe disease and the benefit of concomitant use of dapsone with corticosteroids and other immunosuppressant drugs, even in patients with a history of sulfa allergy.