Concomitant Systemic Lupus Erythematosus Research Articles

Concomitant sickle cell disease and systemic lupus erythematosus: A single-center case series.

Sickle cell disease (SCD) and systemic lupus erythematosus (SLE) are two uncommon disorders each characterized by multisystemic manifestations. Individuals with SCD exhibit abnormalities in the complement pathway, which may predispose patients to develop autoimmune disorders such as SLE. As many manifestations of SLE mimic those of SCD, diagnosis and therapeutic management of SLE in a patient with known SCD may be delayed. In this study, we describe our institutional experience of diagnosing and managing concomitant SCD and SLE. We offer insights into the complex interplay between these conditions to enhance early recognition and effective management of concurrent SCD and SLE.

7-year follow-up atherosclerotic plaque progression in patients with antiphospholipid syndrome versus diabetes mellitus and healthy controls.

Patients with antiphospholipid syndrome (APS) carry a substantial burden of cardiovascular disease and subclinical atherosclerosis. We aimed to assess a 7-year follow-up atherosclerotic plaque progression in APS patients vs diabetes mellitus (DM) and healthy controls (HC). Eighty-six patients with thrombotic APS, 86 with DM and 86 HC (all age- and sex-matched) who underwent a baseline ultrasound of carotid and femoral arteries were invited for a 7-year follow-up ultrasonography examination. We compared atherosclerosis progression among the three groups and examined determinants of plaque progression in APS patients. Sixty-four APS patients (75% females, 43.8% with primary APS), 58 patients with DM and 66 HC were included in the 7-year ultrasound re-evaluation. New plaque was detected in 51.6%, 36.2% and 25.8% of APS, DM and HC subjects, respectively. After adjusting for traditional cardiovascular risk factors (CVRFs) and baseline plaque presence, APS patients showed a 3-fold (OR = 3.07, p= 0.007) higher risk for atherosclerosis progression vs HC and 2-fold (OR = 2.25, p= 0.047) higher risk than DM patients. In multivariate analysis in the APS group, plaque progression was independently associated with systemic lupus erythematosus (SLE) co-existence (OR = 7.78, p= 0.005) and number of CVRFs (OR = 3.02, p= 0.002), after adjusting for disease-related parameters and CVRF-related medications. Sustained low-density lipoprotein target attainment reduced plaque progression risk (OR = 0.34, p= 0.021). Half of APS patients develop new atherosclerotic plaques over a 7-year follow-up, having a three-times higher risk vs HC. Concomitant SLE and number of traditional CVRFs are associated with plaque progression, supporting the need for thorough CVRF assessment and control.

Nationwide analysis of neuromyelitis optica in systemic lupus erythematosus and Sjogren's syndrome.

Neuromyelitis optica (NMO), also known as Devic's disease, is a rare inflammatory demyelinating disorder causing myelitis and optic neuritis. While there have been reports of systemic lupus erythematosus (SLE) and primary Sjogren's syndrome (SS) occurring with NMO, a formal association is not established. We aimed to investigate the occurrence of NMO in SLE and SS patients and study the clinical characteristics and outcomes of NMO and SLE/SS hospitalizations utilizing the national inpatient sample (NIS) database. The NIS database from 2016 to 2019 was used to extract data. Adult hospitalizations with the principal or secondary diagnosis of NMO were included. We classified NMO patients with and without concomitant diagnosis of SLE or Sjogren's syndrome. We evaluated and compared the clinical characteristics and outcomes of NMO hospitalizations with and without SLE or Sjogren's syndrome. STATA17 was used for data analysis. We also calculated the odds ratio of NMO in SLE and Sjogren's syndrome. There were a total of 16,360 adult hospitalizations with the principal or secondary discharge diagnosis of NMO. Among all NMO hospitalizations, 1425 (8.71%) had the primary or secondary diagnosis of SLE or SS. The odds of NMO in SLE and Sjogren's syndrome were noted to be 12.29 and 5.56, respectively. NMO with SLE/SS group had higher proportion of females (89.82% vs 79%, P value < 0.001), African Americans (56.63% vs 38.28, P value < 0.001), and Asians (5.73% vs 3.25, P value 0.04). The Charlson comorbidity index was higher for NMO-SLE/SS overlap (2.44 vs 1.28, P value < 0.001). There was no significant difference in overall mortality rates of both groups (2.11% vs 1.2%, P value 0.197). There were significantly higher reported seizures (14.73% vs 6.05, P value < 0.001) and paraplegia (21.75% vs 13.93%, P value < 0.001) in NMO-SLE/SS patients. These patients also had a longer length of stay in comparison to the reference group (7 vs 5 days, P value < 0.001) as well as higher total charges. NMO patients had a 12-fold higher risk of SLE and 5-fold higher risk of Sjogren's disease when compared to general population. Patients with overlap of NMO and SLE or Sjogren's were predominantly women and were more likely to be African-American. Co-existence of these autoimmune disorders was associated with poor prognosis in terms of higher morbidity for patients and increased health care burden. Key Points • NMO is a rare autoimmune disease seen predominantly in women in the middle age group with low overall mortality. • SLE and Sjogren's have increased odds of NMO in comparison to general population. • NMO patients have high rates of several complications such as paraplegia, quadriplegia, seizures, blindness, sepsis, and respiratory failure with even higher rates of seizures and paraplegia in those with concomitant SLE or Sjogren's.

Molecular consideration relevant to the mechanism of the comorbidity between psoriasis and systemic lupus erythematosus (Review).

Systemic lupus erythematosus (SLE), a common autoimmune disease with a global incidence and newly diagnosed population estimated at 5.14 (range, 1.4-15.13) per 100,000 person-years and 0.40 million people annually, respectively, affects multiple tissues and organs; for example, skin, blood system, heart and kidneys. Accumulating data has also demonstrated that psoriasis (PS) can be a systemic inflammatory disease, which can affect organs other than the skin and occur alongside other autoimmune diseases, such as inflammatory bowel disease, multiple sclerosis, rheumatoid arthritis and SLE. The current explanations for the possible comorbidity of PS and SLE include: i) The two diseases share susceptible gene loci; ii) they share a common IL-23/T helper 17 (Th17) axis inflammatory pathway; and iii) the immunopathogenesis of the two conditions is a consequence of the interactions between IL-17 cytokines with effector Th17 cells, T regulatory cells, as well as B cells. In addition, the therapeutic efficacy of IL-17 or TNF-α inhibitors has been demonstrated in PS, and has also become evident in SLE. However, the mechanisms have not been investigated. To the best of our knowledge, there remains a lack of substantial studies on the correlation between PS and SLE. In the present review, the literature, with regards to the epidemiology, genetic predisposition, inflammatory mechanisms and treatment of the patients with both PS and SLE, has been reviewed. Further investigations into the molecular pathogenic mechanism may provide drug targets that could benefit the patients with concomitant PS and SLE.

Suspected COVID-19 Immunization-Induced Probable Catastrophic Antiphospholipid Syndrome

In this report, we describe the case of a woman with suspected COVID-19 immunization-induced probable catastrophic antiphospholipid syndrome. The patient is a 35-year-old female with a past medical history significant for antiphospholipid syndrome, not on anticoagulation, who presented with a 5-day history of abdominal pain and distention, nausea, vomiting, and shortness of breath. She had received her first dose of the Pfizer COVID-19 vaccine one day prior to the onset of symptoms. After extensive workup at an outside hospital, she was found to be in acute heart failure exacerbated by severe mitral and tricuspid regurgitation. She was transferred to our hospital for escalation of care. EKG showed evidence of prior inferior and septal myocardial infarction. Transesophageal echocardiogram (TEE) showed reduced ejection fraction, severe mitral and tricuspid regurgitation, and a left ventricular thrombus. Cardiac MRI showed subendocardial late gadolinium enhancement indicative of ischemia. However, CTA of the coronary vessels showed no signs of obstruction. Therefore, her acute heart failure was thought to be due to small vessel thrombosis secondary to antiphospholipid syndrome. During admission, she had several absence seizure-like episodes. CT head showed several hypodensities of the deep white matter and brain MRI demonstrated multiple hyperintense T2 FLAIR signal foci with restriction diffusion and enhancement involving the cerebral hemisphere, consistent with subacute strokes attributed to being secondary to antiphospholipid syndrome or embolic from the left ventricular thrombus. She was treated with heparin for suspected catastrophic antiphospholipid syndrome and high-dose corticosteroid therapy for concomitant Systemic Lupus Erythematosus (SLE). She was discharged in a stable condition.

Serum Cytokine Levels of Systemic Lupus Erythematosus Patients in the Presence of Concomitant Cardiovascular Diseases.

Systemic lupus erythematosus (SLE) is known to be associated with an increased risk of cardiovascular diseases (CVD). SLE patients suffer from CVD 3.5 times more often than healthy people. Cytokine-mediated inflammation is actively involved in the development of cardiovascular pathology. Here, we analyzed serum levels of nine cytokines of steroids-treated SLE patients depending on the presence of concomitant CVD. The levels of interleukin (IL)-1β, IL-2, IL-4, IL-6, IL-10, IL-21, tumor necrosis factor α (TNFα), B-cell activating factor (BAFF), and a proliferation-inducing ligand (APRIL) were analyzed using multiplex immunoassay. In the total group of SLE patients (n=29), the concentrations of IL-6 and IL-10 were higher, and the APRIL level decreased compared to healthy donors (n=39, p<0.05). The same changes were observed in the group of patients without CVD (n=15); the levels of IL-6 and IL- 10 were found to be increased, and the level of APRIL was lower than in healthy individuals (p<0.05). In the group of SLE patients with CVD (n=14), the concentrations of IL-4, IL-6, IL- 10, and TNFα were found to be increased (p<0.05). Interestingly, the levels of TNFα and BAFF in SLE patients with CVD were higher than in patients without cardiovascular pathology. Thus, TNFα and BAFF levels were significantly altered in SLE with concomitant CVD compared to SLE without CVD. These findings suggest that cytokine profiles in SLE with concomitant CVD and SLE without CVD are different, which should be considered in further research with large samples.

Systemic Lupus Erythematosus and Hereditary Coproporphyria: Two Different Entities Diagnosed by WES in the Same Patient

Background Hereditary coproporphyria (HCP) is a rare autosomal dominant disorder caused by a partial deficiency of coproporphyrinogen III oxidase (CPOX), and systemic lupus erythematosus (SLE) is an autoimmune disease with a strong genetic predisposition. SLC7A7 (solute carrier family 7 member 7) may be associated with monogenic lupus disease; however, only 2 cases of concomitant HCP and SLE have been reported. Methods We report a 30-year-old woman with a six-year history of SLE presenting with abdominal pain, vomiting, dysuria, tachycardia, and hyponatremia. Whole exome sequencing (WES) and Sanger sequencing were carried out for the proband and members of her pedigree to detect the genetic background. The Gene Expression Omnibus (GEO) database was used to search the related gene expression profiles. Differentially expressed genes (DEGs) were identified using GEO2R. Result A novel heterozygous splicing mutation of CPOX (NM_000097): c.700+2 T > C (intron 2) was detected by WES in the proband, and it was considered likely pathogenic (PSV1+PM2). Sanger sequencing verified the heterozygous mutation of CPOX in the proband, although it was not detected in her father. WES also identified 62 other gene variants, especially two heterozygous variants in SLC7A7 (NM_001126106): c.250G > A (p. V84I) and c.625+1G > A (splicing). DEGs were detected from GSE51997, and the expression of CPOX was downregulated in SLE patients compared with normal controls (adj. P = 0.0071, logFC = −1.0975). Conclusion This study presents the first reported case of SLE coexisting with HCP in China; moreover, a novel splicing mutation of CPOX, i.e., c.700+2 T > C (intron 2), and two heterozygous mutations of SLC7A7 were reported. The simultaneous mutations of CPOX and SLC7A7 may explain the etiopathogenetic connections of HCP and SLE.

Prominent B-Cell Signature Differentiates Discoid from Subacute Cutaneous Lupus Erythematosus

Although B cells account for a significant proportion of the lymphocytic infiltrate in discoid lupus erythematosus (DLE), their contribution to pathogenesis is unknown. In this study, we compare the immune landscape of 17 subjects with DLE with that of 21 subjects with subacute cutaneous lupus erythematosus using transcriptomic and histologic analyses of lesional skin. A few of the subjects (3 of 17 subjects with DLE, and 5 of 21 subjects with subacute cutaneous lupus erythematosus) had concomitant systemic lupus erythematosus. Using a modified Autoimmune Profiling Panel (NanoString Technologies, Seatle, WA), we show that B-cell‒specific genes, including canonical pan‒B cell markers CD19 (P= 0.0060), MS4A1(CD20) (P= 0.0047), and CD79a (P= 0.0201), are among the most upregulated genes in DLE. Numerous other genes encoding B-cell‒associated proteins, including Igs, BAFF receptors, and FCRL family members, are similarly enriched. Relative cell type scoring reveals that among various inflammatory cell types, only B cells are more prevalent in DLE. Digital whole-image slide analysis of immunohistochemistry for B cells (CD20) and T cells (CD3) supports our gene expression findings of a disproportionately greater B-cell infiltrate in DLE lesions. Overall, this study identifies a B-cell‒predominant signature unique to DLE and highlights the importance of studying the role of cutaneous B cells in DLE pathogenesis.

Clinical Outcomes and Inpatient Mortality Among Hospitalized Patients With Concomitant Autoimmune Hepatitis and Systemic Lupus Erythematosus.

BackgroundAutoimmune hepatitis (AIH) is an inflammatory disease of the liver that is characterized by a broad disease spectrum, circulating autoantibodies, and elevated serum globulin levels. Systemic lupus erythematosus (SLE) is a chronic disease that is characterized by a high inflammatory state and is associated with multiorgan system involvement. Despite a well-known association between AIH and other autoimmune diseases, the literature is deficient on the associations between AIH-related outcomes and complications in SLE patients. This study aims to evaluate the effects of SLE on clinical outcomes and inpatient mortality in patients with AIH.MethodThe National Inpatient Sample (NIS) database was used to identify AIH-related hospitalizations from 2012 to 2014 using International Classification of Diseases Ninth Edition Revision (ICD-9) codes. Patients were divided into two groups, those with and without SLE. Primary outcomes were mortality, hospital charges, and length of stay (LOS). Secondary outcomes were complications associated with AIH: cirrhosis, gastrointestinal (GI) bleed, acute liver failure (ALF), cholangitis, pancreatitis, and sepsis. Chi-squared tests for categorical data and independent t-test for continuous data were used to compare outcomes. Multivariate analysis was performed to assess the primary outcomes after adjusting for confounding variables.ResultsThere were 17,050 AIH-related hospitalizations from 2012 to 2014 and 1,115 patients had SLE. In patients with SLE and AIH, 1,035 were female with average age of 48.6. The average LOS was 6.3 days, mortality rate was 1.35%, and total hospital charges were $48,146. SLE was associated with a statistically significant lower mortality rate compared to the control. LOS, hospital cost, and CCI (Charlson Comorbidity Index) were not found to be significantly different. For secondary outcomes, SLE was statistically significant for having higher pancreatitis rates. SLE patients had statistically significant lower cholangitis, and ALF. Differences in complications such as sepsis and GI bleed were non-significant.ConclusionSLE is known to have a high inflammatory state so it was hypothesized that there would be higher rates of complications and a higher mortality rate in those with concomitant AIH. This study showed that the mortality rate was lower in SLE patients with lower rates of complications including ALF and cholangitis. We postulate that SLE patient outcomes are likely affected by the treatment regimen involved with SLE, including corticosteroids. This would provide an immunosuppressive state, limiting the autoreactivity cascade in AIH, in effect leading to better outcomes and a mortality benefit. This study identifies a lower mortality rate and lower complication rates in patients with AIH and SLE overlap as compared to patients with AIH alone and future studies are needed to confirm these associations.

A patient with type 1 diabetes mellitus who developed exogenous insulin antibody syndrome ameliorated by immunosuppressive treatment for concomitant systemic lupus erythematosus: A case report

A patient with type 1 diabetes mellitus who developed exogenous insulin antibody syndrome ameliorated by immunosuppressive treatment for concomitant systemic lupus erythematosus: A case report

S226 Inpatient Outcomes of Clostridioides difficile Infection Among Patients With and Without Systemic Lupus Erythematosus

Introduction: Patients with systemic lupus erythematosus (SLE) are generally known to have an increased risk of infection. However, there is a lack of studies exploring how SLE affects the outcomes of Clostridioides Difficile Infection (CDI). Thus, we aim to assess the outcomes of CDI in hospitalized patients with concomitant SLE. Methods: Patients hospitalized with CDI from the National Inpatient Sample, Healthcare Cost and Utilization Project, Agency for Healthcare Research and Quality 2014 were selected. Diagnoses were identified by using ICD-9 CM codes. Patient demographics and outcomes of CDI were compared between the groups with and without SLE. The outcomes of interest were inpatient mortality, length of stay, total hospital charge, hypotension/shock, acute renal failure, ileus, megacolon, and colectomy. Chi-squared tests and independent t-tests were used to compare proportions and means respectively. Multivariate logistic regression analysis was performed to determine if SLE is an independent predictor for the outcomes, adjusting for age, sex, race, Charlson Comorbidity Index, and CDI risk factors including obesity, inflammatory bowel disease, and cirrhosis. Results: Among 29,549 patients with CDI identified in the study, 332 patients had SLE. Among patients hospitalized with CDI, those with SLE were younger (53.8 vs 66.4, P < 0.05), more likely to be female (91.0% vs 63.9%, P < 0.05), less likely to White (55.7% vs 78.4%, P < 0.05), had lower Charlson Comorbidity Index (3.8 vs 4.2, P < 0.05), and had lower inpatient mortality (0.3% vs 1.8%, P < 0.05). There were no statistically significant differences in length of stay (6.7 days vs 6.2 days, P = 0.20) and total hospital charge ($45,884 vs $41,200, P = 0.17). Patients with SLE were more likely to be obese (14.6% vs 9.7%, P < 0.05). After adjusting for age, sex, race, Charlson Comorbidity Index, and CDI risk factors, patients with SLE were less likely to have hypotension/shock (aOR 0.45, 95% CI: 0.23-0.88, P < 0.05). The odds of acute renal failure, ileus, colectomy, and inpatient mortality were not statistically significant. Megacolon was omitted due to low prevalence in both groups. Conclusion: Our study indicates that SLE is an independent protective factor against hypotension/shock in patients hospitalized with CDI while there are no significant differences in other outcomes.Table 1.: Demographics on presentation for colon cancer in patients with and without HIV

P-OT013. Paroxysmal tonic spasms and right foot drop as initial presentations of neuromyelitis optica

Introduction . Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disorder commonly presents with acute myelitis or optic neuritis. Results . We report the case of a middle age lady presented with paroxysmal tonic spasms (PTS) of bilateral lower limbs and right foot drop, without any upper motor neuron weakness or sensory level. EMG done confirmed a right peroneal neuropathy at the fibula head. MRI spine showed longitudinal extensive transverse myelitis and her serum aquaporin-4 antibody was positive, consistent with NMOSD. Incidentally, the patient also had persistent leucopenia and positive anti-nuclear antibody, secondary to concomitant systemic lupus erythematosus, which accounted for the foot drop at the onset. Conclusion . This case highlights that tonic spasms can be the initial manifestation of NMOSD, in the absence of overt myelitis or optic neuritis. Treatment with carbamazepine usually results in prompt resolution of PTS.

Systemic sclerosis overlap and non-overlap syndromes share clinical characteristics but differ in prognosis and treatments

ObjectivesTo screen for concomitant autoimmune disease in patients with systemic sclerosis (overlap SSc) and to describe their clinical characteristics and prognosis. MethodsThis was a two-center retrospective observational study. Patients diagnosed with SSc according to the 2013 ACR-EULAR scleroderma classification criteria were screened for concomitant rheumatoid arthritis (RA), Sjögren syndrome (SgS) and systemic lupus erythematosus (SLE). Patient characteristics were retrieved from the medical records and were compared to those of a non-overlap SSc cohort. ResultsAmong the 534 SSc patients studied, thirty-four (6.4%) were identified as having overlap SSc. There were 21 (3.9%) patients with RA, 14 (2.6%) with SgS and 4 (0.7%) with SLE (5 patients had 2 AISD) . The disease phenotype of overlap SSc was similar to that of non-overlap SSc in terms of cutaneous phenotype, prevalence of pulmonary arterial hypertension, interstitial lung disease, digital ulcers and mortality. Using a multivariate Cox model, age (HR = 1.04, 95% CI [1.02–1.07]), the modified Rodnan skin score (HR = 1.08 per point, 95% CI [1.05–1.11]), and the presence of concomitant SgS (HR = 3.79, 95% CI [1.38–10.40]) were significantly associated with mortality. Overlap SSc were more likely to receive corticosteroids (85.3% vs. 45%, p < 0.001), immunosuppressive drugs (82.4% vs. 49.2%, p < 0.001) and biologics (52.9% vs. 3.8%, p < ZZ0.001). ConclusionsWhile overlap and non-overlap SSc shared common characteristics, patients with SgS/SSc had a higher risk of mortality, and those with RA/SSc received more corticosteroids, methotrexate and biologics. Screening for an associated AISD should be promoted since their co-occurrence with SSc may affect prognosis and treatments.

Clinical characteristics and prognosis of concomitant systemic lupus erythematosus and primary biliary cholangitis.

This study aimed to identify the clinical characteristics of systemic lupus erythematosus-primary biliary cholangitis (SLE-PBC) patients and to compare the manifestations and prognosis with systemic lupus erythematosus (SLE) patients. Twenty-one inpatients with concomitant SLE and primary biliary cholangitis (PBC) were identified in our hospital. Baseline clinical manifestations, laboratory results, disease activity, and organ damage, as well as changes in disease manifestations and therapies, were retrospectively analyzed. Baseline clinical characteristics, survival rate, and flare-ups were compared with 254 SLE patients also from our hospital. The prevalence of concomitant PBC in SLE inpatients was 0.27%. Over half of the patients were diagnosed with SLE and PBC simultaneously. Compared with SLE patients, SLE-PBC patients started the symptom of SLE at an older age, with a longer delay before the diagnosis of SLE (P < 0.05). Hematological and muscular involvement, pulmonary arterial hypertension, and interstitial lung disease were more common in SLE-PBC patients (P < 0.05). Kaplan-Meier estimate showed a significantly lower survival rate in SLE-PBC group, with 3-year survival rate at 88.4%. Concomitant PBC might have a negative impact on the survival of SLE, with older age at SLE onset, longer delay before SLE diagnosis, and higher baseline damage. More intensive therapy and prevention of hepatic toxicity need to be considered. Key Points • Hematological and muscular involvement, PAH, and ILD were more common in SLE PBC than in SLE. • The study firstly reported the survival rate of SLE PBC patients. • More intensive therapy and prevention of hepatic toxicity are needed for SLE-PBC.

Incidence of psoriasis in patients with active systemic lupus erythematosus

Introduction: Concomitant onset of active systemic lupus erythematosus (SLE) and psoriasis is rare; however, due to the common skin involvement in both and exacerbation of psoriasis with antimalarial medications used for SLE, it is crucial to investigate the concomitant occurrence of both diseases. Objectives: The present study aims to investigate the incidence of psoriasis in people with active SLE. Patients and Methods: The present cross-sectional study was performed on 148 patients with active SLE from 2015 to 2017. Lupus activity was evaluated based on systemic lupus erythematosus disease activity index-2K (SLEDAI-2K), and then, the incidence of psoriasis in patients was investigated. Patients’ demographic information was recorded in the relevant checklist. Results: The mean score of SLEDAI-2K was 14.10±19.77. The duration of SLE was significantly associated with the SLEDAI-2K score (P=0.001, r = 0.294). Psoriasis incidence was remarkably correlated with the gender of patients with SLE (P=0.019). An insignificant association was found between the coincidences of SLE with psoriasis considering the factors, including age, SLE duration, and mean SLEDAI-2K score. Conclusion: Patients’ mean age, SLE duration, and disease activity score were not correlated with concomitant psoriasis among SLE patients; however, we found that males were more prone to concomitant SLE and psoriasis.

Systemic lupus erythematosus in the setting of HIV-1 infection: a longitudinal analysis.

Patients with concomitant HIV-1 infection and systemic lupus erythematosus (SLE) followed longitudinally at an HIV outpatient clinic from 1994 to 2019 were examined. Patients met 1982 and 1997 ACR classification criteria for SLE at the Thomas Street clinic from 1994 to 2019. Clinical and demographic comparisons were made with a non HIV-SLE patient cohort, the Lupus in Minorities Studies, Nature versus Nurture (LUMINA) study. Twenty-two patients with concomitant HIV-1 infection and SLE were identified, including 18 females, 3 males, and 1 male to female transgender. Overall, 81.8% of SLE-HIV patients were African-American compared to 55.3% of the 5856 patients seen at the HIV clinic from 2016 to 2017 (p= 0.02, OR = 3.6). There were 12 patients that developed HIV-1 in the setting of SLE and 10 patients that developed SLE in the setting HIV-1. This demographic distribution was significant when compared with the 1604 unique patients in the HIV rheumatology clinic from 1994 to 2019 (p= 0.03, OR = 5.9) and also significant when compared with the 5856 patients attending the county HIV clinic overall in 2016-2017 (p= 0.008, OR = 7.2). When comparing with the non-HIV SLE cohort, anti-dsDNA antibodies were noted less frequently in all HIV-SLE patients (p= 0.0002) including all sub-cohorts of our patients. Skin/mucosal involvement (p= 0.0003) and cytopenias (p= 0.0001) occurred less frequently in the patients that were diagnosed with HIV after their SLE diagnosis compared to non-HIV SLE patients. In a large county HIV clinic setting, the prevalence of SLE was significantly higher in African American women. Anti-dsDNA antibodies were less frequent in HIV-1 positive SLE patients.Key Points• This paper presents clinical and laboratory data on the largest cohort of SLE patients with HIV-1 infection reported to date.• The prevalence of SLE in a large outpatient HIV-1 clinic was larger than reported in HIV negative population studies.• The prevalence of SLE was particularly high in black HIV-1 infected women.• Skin/mucosal involvement and anti-ds DNA antibodies were less common in patients with HIV-1 and SLE compared to patients with SLE without SLE>.

Concomitant systemic lupus erythematosus might have a negative impact on the biochemical responses to treatment in patients with primary biliary cholangitis.

Primary biliary cholangitis (PBC) is often overlapping with other autoimmune conditions, including systemic lupus erythematosus (SLE). Since the concomitant PBC and SLE are rare, the impacts of SLE on the response and prognosis in ursodeoxycholic acid (UDCA)-treated patients with PBC remain unclear. A PBC database of 769 patients at West China hospital was used to identify 26 patients with concomitant PBC and SLE. The clinical and biochemical characteristics of these patients were collected and analyzed. Propensity score matching was used to compensate for the differences in age, total bilirubin, and alkaline phosphatase. The biochemical responses and prognoses were compared between the patients with and without concomitant SLE. The female-to-male ratio was 25:1 in the PBC patients with concomitant SLE. Compared with the group with PBC alone, the median hemoglobin and albumin values in the PBC-SLE group at the time of diagnosis of PBC were lower (both P < 0.05). After treatment, the group with PBC alone showed lower alanine aminotransferase and glutamyltransferase values and aspartate aminotransferase/platelet ratio indices at the final visit (all P < 0.05). The Kaplan-Meier estimate showed that the adverse event-free survival did not differ between the patients with and without concomitant SLE (P = 0.564). The results of this retrospective, single-center study suggested that the concomitant SLE status might have a negative impact on the biochemical responses to the treatment, while the effect of concomitant SLE on PBC progression remains to be further defined.Key Points• The prevalence of SLE in the PBC population being in a large PBC cohort was as low as 3.4%.• Compared with PBC-SLE group, the group with PBC alone showed lower alanine aminotransferase and glutamyltransferase values and aspartate aminotransferase/platelet ratio indices at the final visit, indicating that the concomitant SLE may have a negative impact on the biochemical responses to the treatment of PBC.

A case of Lemierre syndrome combined with a suspected systemic lupus erythematosus flare

Lemierre syndrome develops in healthy young patients as a result of bacteremia after oral cavity infection. It causes thrombophlebitis in the internal jugular vein. Infection can easily occur during immunosuppressive treatment in patients with systemic lupus erythematosus and become severe. We present a case of Lemierre syndrome in a patient with systemic lupus erythematosus. A 56-year-old woman presented with fever, left lower toothache, and skin symptoms from the left neck to the anterior chest. Clinical presentation and laboratory investigations revealed Lemierre syndrome. The inflammation and thrombus disappeared with antibiotic and anticoagulant therapies. However, transient hypocomplementemia and elevated antinuclear antibody levels were observed during treatment; therefore, a concomitant systemic lupus erythematosus flare was considered. In systemic lupus erythematosus patients with Lemierre syndrome, complement and antinuclear antibody levels are modified, so other indicators should be precisely evaluated, such as levels of urinary protein, sediment, serum creatinine and anti-dsDNA antibody, and systemic lupus erythematosus disease activity index.

Wilson\u2019s disease combined with systemic lupus erythematosus: a case report and literature review

BackgroundWilson’s disease (WD) is an inherited disorder in which defective biliary excretion of copper leads to its accumulation, particularly in the liver and brain. Systemic lupus erythematosus (SLE) is a multi-system disorder that can manifest in any system. Cases with concomitant WD and SLE, unrelated to treatment with penicillamine, have been rarely reported.Case presentationWe report a case of a young woman who had typical neuropsychiatric symptoms and laboratory tests results of WD. She also had concomitant massive hematuria and proteinuria, fever, multiple positive autoimmune antibodies, hypocomplementemia, abnormal lumbar puncture findings and evidence of Sjögren syndrome, which are all rare in WD. Hence, we considered the diagnosis of SLE. Tapering of steroid dosage also confirmed the diagnosis.ConclusionWilson’s disease and SLE have varied clinical manifestations. Herein, we reported a rare case in which the two conditions concomitantly existed. In clinical practice, differential diagnosis of the two diseases is necessary for patients with hepatic, neurological, and psychiatric manifestations.

Features, Treatment, and Outcomes of Macrophage Activation Syndrome in Childhood-Onset Systemic Lupus Erythematosus.

To describe the features and treatment of macrophage activation syndrome (MAS) in a single-center cohort of patients with childhood-onset systemic lupus erythematosus (SLE), and to compare childhood-onset SLE manifestations and outcomes between those with and those without MAS. We included all patients with childhood-onset SLE followed up at The Hospital for Sick Children from 2002 to 2012, and identified those also diagnosed as having MAS. Demographic, clinical, and laboratory features of MAS and SLE, medication use, hospital and pediatric intensive care unit (PICU) admissions, as well as damage indices and mortality data were extracted from the Lupus database. Student's t-tests and Fisher's exact tests were used to compare continuous and categorical variables, respectively. We calculated incidence rate ratios of hospital and PICU admissions comparing patients with and those without MAS, using Poisson models. Kaplan-Meier survival analysis was used to examine the time to disease damage accrual. Of the 403 patients with childhood-onset SLE, 38 (9%) had MAS. The majority (68%) had concomitant MAS and SLE diagnoses. Fever was the most common MAS clinical feature. The frequency of renal and central nervous system disease, hospital admissions, the average daily dose of steroids, and time to disease damage were similar between those with and those without MAS. We observed a higher mortality rate among those with MAS (5%) than those without MAS (0.2%) (P = 0.02). MAS was most likely to develop concomitantly with childhood-onset SLE diagnosis. The majority of the MAS patients were successfully treated with corticosteroids with no MAS relapses. Although the numbers were small, there was a higher risk of death associated with MAS compared to SLE without MAS.