Onset Of Systemic Lupus Erythematosus Research Articles

Peripheral nervous system involvement in systemic lupus erythematosus: a retrospective study on prevalence, associated factors and outcome

Despite its potentially significant impact on disease outcome, peripheral nervous system involvement in systemic lupus erythematosus has received little attention. The objective of this study was to assess the prevalence and clinical features of peripheral nervous system involvement in a large cohort of systemic lupus erythematosus patients. The records of systemic lupus erythematosus patients examined at two tertiary referral centres over a period of 14 years (from 2000 to 2014) were analyzed. Peripheral nervous system events were ascertained according to the 1999 American College of Rheumatology case definitions and by using an attribution algorithm for neuropsychiatric events. Prevalence of peripheral nervous system in systemic lupus erythematosus and demographic, clinical and laboratory features were assessed. Patients with peripheral nervous system events were compared with a control group of systemic lupus erythematosus patients without peripheral nervous system involvement. In a retrospective cohort of 1224 patients, the overall prevalence of peripheral nervous system involvement was 6.9% (85 patients, 95% confidence interval 0.06-0.08), with 68% of peripheral nervous system events attributable to systemic lupus erythematosus. Polyneuropathy was the most common manifestation observed (38 events, 39.2%), followed by cranial neuropathy in 30 cases (30.9%) and 12 cases of single (12.4%) or multiple (eight events, 8.2%) mononeuritis. The average age of systemic lupus erythematosus onset was significantly higher in patients with peripheral nervous system events than in controls (mean ± standard deviation: 45.9 ± 14.8 vs. 37.1 ± 14.0) and they were more likely to have higher SLEDAI-2K and SLICC/ACR Damage Index scores, as well as hypertension and livedo reticularis. A subgroup analysis of events deemed to be systemic lupus erythematosus-related provided similar results. Peripheral nervous system manifestations are a potential complication of systemic lupus erythematosus. Careful neurological assessment should therefore be included in the diagnostic workup of patients with systemic lupus erythematosus, especially in those with later onset and greater damage and disease activity.

Psychosis in Systemic Lupus Erythematosus: Results From an International Inception Cohort Study.

ObjectiveTo determine, in a large, multiethnic/multiracial, prospective inception cohort of patients with systemic lupus erythematosus (SLE), the frequency, attribution, clinical, and autoantibody associations with lupus psychosis and the short‐ and long‐term outcomes as assessed by physicians and patients.MethodsPatients were evaluated annually for 19 neuropsychiatric (NP) events including psychosis. Scores on the Systemic Lupus Erythematosus Disease Activity Index 2000, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and the Short Form 36 (SF‐36) were recorded. Time to event and linear regressions were used as appropriate.ResultsOf 1,826 SLE patients, 88.8% were female and 48.8% were Caucasian. The mean ± SD age was 35.1 ± 13.3 years, the mean ± SD disease duration was 5.6 ± 4.2 months, and the mean ± SD follow‐up period was 7.4 ± 4.5 years. There were 31 psychotic events in 28 of 1,826 patients (1.53%), and most patients had a single event (26 of 28 [93%]). In the majority of patients (20 of 25 [80%]) and events (28 of 31 [90%]), psychosis was attributed to SLE, usually either in the year prior to or within 3 years of SLE diagnosis. Positive associations (hazard ratios [HRs] and 95% confidence intervals [95% CIs]) with lupus psychosis were previous SLE NP events (HR 3.59 [95% CI 1.16–11.14]), male sex (HR 3.0 [95% CI 1.20–7.50]), younger age at SLE diagnosis (per 10 years) (HR 1.45 [95% CI 1.01–2.07]), and African ancestry (HR 4.59 [95% CI 1.79–11.76]). By physician assessment, most psychotic events resolved by the second annual visit following onset, in parallel with an improvement in patient‐reported SF‐36 summary and subscale scores.ConclusionPsychosis is an infrequent manifestation of NPSLE. Generally, it occurs early after SLE onset and has a significant negative impact on health status. As determined by patient and physician report, the short‐ and long‐term outlooks are good for most patients, although careful follow‐up is required.

Sex and Pubertal Differences in the Type 1 Interferon Pathway Associate With Both X Chromosome Number and Serum Sex Hormone Concentration.

Type 1 interferons (IFN) are an antiviral cytokine family, important in juvenile onset systemic lupus erythematosus (jSLE) which is more common in females, around puberty. We report that plasmacytoid dendritic cells (pDC) from healthy females produced more type 1 IFN after toll like receptor (TLR) 7 signaling than males, even before puberty, but that puberty itself associated with increased production of type 1 IFN. A unique human model allows us to show that this was related to X chromosome number, and serum testosterone concentration, in a manner which differed depending on the number of X chromosomes present. In addition, we have showed that pDC were more activated in females overall, and immune cell TLR7 gene expression was higher in females after puberty. Therefore, sex hormones and X chromosome number were associated individually and interactively with the type 1 IFN response, which contributes to our understanding of why females are more likely to develop an IFN mediated disease like jSLE after puberty.

Gwałtowny początek tocznia rumieniowatego układowego z rzadko obserwowanymi zmianami skórnymi

Gwałtowny początek tocznia rumieniowatego układowego z rzadko obserwowanymi zmianami skórnymi

Assessment of Health Related Quality of Life (HRQL) in Egyptian Children with Rheumatic Diseases; Its Relation to Disease Activity and Functional Disability

Background Many pediatric rheumatic diseases persist into adulthood, with negative squeal from the disease or its treatment. We aimed to assess health related quality of life in a group of children with rheumatic diseases and its relation to disease activity and functional disability. Materials and Methods Fifty one patients were divided into three groups: Group 1 includes 27 patients diagnosed as juvenile idiopathic arthritis (JIA), Group 2 includes 15 patients diagnosed as juvenile onset systemic lupus erythematosus (SLE) and Group 3 includes nine patients; three diagnosed as Juvenile Dermatomyositis, three diagnosed as Familial Mediterranean Fever and three female’s patients diagnosed as Juvenile Scleroderma. Childhood Health Assessment Questionnaire (CHAQ), Pediatric Quality of Life generic core scale version 4.0, Visual analogue scale for pain and Visual analogue scale (VAS) for global assessment were recorded. The activity index was assessed in each patient according to the nature of the disease. Results In JIA patients, the mean PedsQL score was 73.6+ 15.4, the mean CHAQ score was 0.7+ 0.7, the mean DAS28 was 3.5+ 0.9, with a significant negative correlation between PedsQL and CHAQ (p=0.48), VAS pain (p=0.001), and DAS 28 activity index (p=0.017). In SLE patients, the mean PedsQL was 66.4 + 20.3, mean CHAQ score was 0.7 + 0.67 and mean SLEDAI-2K was 24.2 + 14.6 with no significant correlation between functional disability and SLEDAI-2K (p=0.539). PedsQL showed a significant negative correlation with SLEDAI-2k (p=0.001), and positive correlation between CHAQ (p=0.022). Conclusion Health related quality of life in patients with juvenile rheumatic diseases is correlated with disease activity and functional disability and should be assessed in regular basis.

Clinicopathological characteristics and renal outcomes of childhood-onset lupus nephritis with acute kidney injury: A multicenter study

Objectives: Acute kidney injury (AKI) at onset of adult systemic lupus erythematosus (SLE) is a risk factor for end stage kidney disease (ESKD). However, data on childhood-onset lupus nephritis (LN) with AKI are scarce.Methods: We retrospectively reviewed the complete files of pediatric SLE patients from 1995 to 2010. All patients underwent kidney biopsy promptly after diagnosis.Results: Thirty-six patients (10 males and 26 females) were enrolled. Mean age at diagnosis and observation period were 11.6 ± 2.4 and 8.1 ± 4.4 years, respectively. Seven patients had AKI at onset of SLE. Compared with those without AKI, patients with AKI had significantly higher proportions of pathologically proliferative LN. Only one patient with AKI progressed to ESKD without complete recovery of renal function. Overall and renal survival rates were 100and 97.2%, respectively. There was no significant difference in estimated glomerular filtration rate at the final visit (85ml/min/1.73 m2 in the AKI group vs. 103.2 ml/min/1.73 m2 in the non-AKI group; p = .11).Conclusion: Our study demonstrated favorable renal outcomes in childhood-onset LN with AKI in the near to midterm period. Inducing complete remission may be important for preserving renal function.

The Transcription Factor Ets1 Suppresses T Follicular Helper Type 2 Cell Differentiation to Halt the Onset of Systemic Lupus Erythematosus

Single-nucleotide polymorphisms in ETS1 are associated with systemic lupus erythematosus (SLE). Ets1-/- mice develop SLE-like symptoms, suggesting that dysregulation of this transcription factor is important to the onset or progression of SLE. We used conditional deletion approaches to examine the impact of Ets1 expression in different immune cell types. Ets1 deletion on CD4+ Tcells, but not Bcells or dendritic cells, resulted in the SLE autoimmunity, and this was associated with the spontaneous expansion of T follicular helper type 2 (Tfh2) cells. Ets1-/- Tfh2 cells exhibited increased expression of GATA-3 and interleukin-4 (IL-4), which induced IgE isotype switching in B cells. Neutralization of IL-4 reduced Tfh2 cell frequencies and ameliorated disease parameters. Mechanistically, Ets1 suppressed signature Tfh and Th2 cell genes, including Cxcr5, Bcl6, and Il4ra, thus curbing the terminal Tfh2 cell differentiation process. Tfh2 cell frequencies in SLE patients correlated with disease parameters, providing evidence for the relevance of these findings to human disease.

Immunogenicity, safety and tolerability of anti-pneumococcal vaccination in systemic lupus erythematosus patients: An evidence-informed and PRISMA compliant systematic review and meta-analysis.

The immunological perturbations associated with systemic lupus erythematosus (SLE) put many patients at a higher risk of infections, including pneumococcal pneumonia. However, the uptake and utility of anti-pneumococcal vaccines in SLE patient is both controversial and not completely agreed upon. Indeed, several epidemiological studies of anti-pneumococcal vaccine safety and efficacy in SLE have reported short-term immunogenicity with elevated anti-pneumococcal antibody titres but inconsistent long-term findings, with some studies finding poor responses, mainly for long-term immune protection. Moreover, the safety and efficacy of the pneumococcal vaccine in SLE patients remains controversial due to the different types of anti-pneumococcal vaccines, and the heterogeneity of SLE patients. Several reviews addressing anti-pneumococcal vaccination in SLE patients exist, however, to the best of our knowledge, the present is the first systematic review and meta-analysis. To better understand the efficacy and safety of pneumococcal vaccination in SLE, a comprehensive literature search was performed identifying 18 studies, which have been included in the present systematic review and meta-analysis. All studies were designed as longitudinal investigations, 2, in particular, were of high quality, being randomized, double-blind trials (RCTs). Four studies had control groups. Total sample size included 601 participants. Vaccine immunogenicity in terms of subjects with protective antibody titers ranged from 36% to 97.6%. According to our systematic review and metanalysis, high erythrocyte sedimentation rate (ESR), older age, earlier SLE onset, high disease activity, and immunosuppressive therapy were predictors of poor immunogenicity, although belimumab was found to have no significant impact. With regard to safety, no serious adverse events were found, with up to one third of cases reporting mild/low-grade complaints. In conclusion, due to the high risk of pneumococcal infection in SLE patients and given the safety and, at least partial, effectiveness, according to our systematic review and meta-analysis, in such patients, preventive strategies mainly by immunization, are required in all age groups and, in those needing immunosuppressive therapy, immunization should be given prior the initiation of the treatment. PROSPERO registration code CRD42018103605.

New structural insights into the role of TROVE2 complexes in the on-set and pathogenesis of systemic lupus erythematosus determined by a combination of QCM-D and DPI.

The mechanism of self-recognition of the autoantigen TROVE2, a common biomarker in autoimmune diseases, has been studied with a quartz crystal microbalance with dissipation monitoring (QCM-D) and dual polarization interferometry (DPI). The complementarity and remarkable analytical features of both techniques has allowed new insights into the onset of systemic lupus erythematosus (SLE) to be achieved at the molecular level. The in vitro study for SLE patients and healthy subjects suggests that anti-TROVE2 autoantibodies may undergo an antibody bipolar bridging. An epitope-paratope-specific binding initially occurs to activate a hidden Fc receptor in the TROVE2 tertiary structure. This bipolar mechanism may contribute to the pathogenic accumulation of anti-TROVE2 autoantibody immune complex in autoimmune disease. Furthermore, the specific calcium-dependent protein-protein bridges point out at how the TRIM21/TROVE2 association might occur, suggesting that the TROVE2 protein could stimulate the intracellular immune signaling via the TRIM21 PRY-SPRY domain. These findings may help to better understand the origins of the specificity and affinity of TROVE2 interactions, which might play a key role in the SLE pathogenesis. This manuscript gives one of the first practical applications of two novel functions (-df/dD and Δh/molec) for the analysis of the data provided by QCM-D and DPI. In addition, it is the first time that QCM-D has been used for mapping hidden Fc receptors as well as linear epitopes in a protein tertiary structure. Graphical abstract ᅟ.

Improvement of survival rates in the last decade in Thai childhood-onset systemic lupus erythematosus

BackgroundMorbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more severe than adult onset SLE. Long-term follow up is needed to determine the prognosis. The objectives of this study are to describe the mortality of childhood SLE in a single tertiary care centre over three decades, compare trends in survival over time, and determine predictors for survival.MethodsWe retrospectively reviewed the medical records of children aged < 18 years who were diagnosed with SLE at the Department of Pediatrics, Songklanagarind Hospital, Thailand, from 1985 to 2016.ResultsThere were 331 children (272 girls, 59 boys) with a mean age at presentation of 11.5 ± 2.6 years. The mean follow-up duration was 7.0 ± 5.0 (range 1–28) years, 77 children (23.3%) died, 28.6% within the first year after diagnosis.The overall mortality rate was 3.3 per 100 person-years. Survival rates at 1, 5 and 10 years were 93.4%, 83.1% and 72.6%, respectively. Ten-year survival rates for the children diagnosed in the decades 1985–1996, 1997–2006 and 2007–2016 were 67.4%, 63.4% and 82.8%, respectively (p < 0.001).Boys had worse survival than girls (hazard ratio = 2.3, 95% CI: 1.4–3.7) even after adjusting for decade of diagnosis. Lupus nephritis (LN) class IV had similar survival compared to LN classes II/III/V combined (hazard ratio = 1.0, 95% CI: 0.6–1.7).ConclusionIn our setting, the survival rate of childhood onset SLE has improved during the past 10 years, but mortality is still high compared to developed countries, particularly in boys.

Podocytes contribute, and respond, to the inflammatory environment in lupus nephritis.

Lupus nephritis (LN) affects up to 80% of juvenile onset systemic lupus erythematosus patients, leading to end stage renal failure requiring dialysis or transplantation in 10–15%. Podocytes are specialized epithelial cells of the glomerulus known to be a key site of damage in glomerular diseases. However, their roles in LN have yet to be fully identified. This project aims to identify structural and functional roles of podocytes in an in vitro model of LN. Conditionally immortalized podocytes were treated with proinflammatory cytokines (IL-1β, TNF-α, IFN-α, and IFN-γ) alone and in combination in an in vitro model of LN and were assessed for their structural and functional characteristics. Podocytes produce TNF-α, IL-6, IL-8, VEGF, granulocyte-monocyte colony stimulating factor (GM-CSF), and macrophage colony stimulating factor (M-CSF) at relatively low levels under basal conditions; stimulation with IL-1β led to increased secretion of IL-6 (P = 0.011), IL-8 (P = 0.05), VEGF (P = 0.02), and M-CSF (P = 0.03). Stimulation with TNF-α led to increased secretion of M-CSF (P = 0.049) and stimulation with IFN-γ led to novel production of IL-10 (P = 0.036) and interferon-γ-inducible protein-10 (P = 0.036). Podocytes demonstrate a reduction in the area covered by filamentous-actin in response to IL-1β treatment within 1 h (P = 0.011), which is restored by 24 h, associated with an increase in the level of intracellular calcium but not with increased cell death. Podocytes contribute to the inflammatory milieu in LN through cytokine/chemokine secretion and respond to the inflammatory milieu via rearrangement of the actin cytoskeleton leading to effacement, a well-known method of protection against apoptosis in these cells. This demonstrates that podocytes are involved in the pathogenesis of LN.

Use of Intravenous Immunoglobulin Therapy at Unconventional Doses in Refractory Fulminant Systemic Lupus Erythematosus.

The use of human intravenous immunoglobulins (IVIg) in systemic lupus erythematosus (SLE) currently relies on evidence from small case series and is mainly regarded as an off-label strategy in cases that are refractory to conventional therapies or poorly controlled with high doses of corticosteroids. Standard dosage regimens typically entail the administration of a total amount of 2 g/kg of IVIg divided into five consecutive days in order to minimize the risk of severe adverse events. We herein describe the case of a 28-year-old woman with a known history of antiphospholipid syndrome (APS) who was admitted to our hospital following fulminant onset of SLE in spite of ongoing immunosuppressive therapy. Acute renal insufficiency with nephrotic-range proteinuria, central nervous system involvement, severe thrombocytopenia, malar rash, pancreatic injury and moderate-severe aortic valve steno-insufficiency were the most prominent clinical manifestations, along with high titres of anti-dsDNA antibodies. Pulses of methyl-prednisolone followed by high-dose corticosteroids proved ineffective. Strikingly, IVIg therapy delivered at unconventional doses (1.2 g/kg) due to the presence of multiple risk factors for adverse events resulted in a significant, comprehensive clinical improvement. Although large-scale randomized double-blind studies are needed, the use of IVIg might constitute a valuable therapeutic modality as a last-resort strategy in cases of fulminant SLE. The total dose of immunoglobulins should be dictated by the clinical response as well as the presence of pre-existing risk factors for adverse events.LEARNING POINTSThe use of immunoglobulins in the treatment of systemic lupus erythematosus is mainly based on small prospective studies and case series.Their use as a rescue strategy in cases of systemic lupus erythematosus that are refractory to conventional immunosuppressive therapy may be a valid therapeutic alternative in selected patients.The short-term clinical response and the presence of risk factors for adverse effects should dictate the overall dose of immunoglobulins administered to the patient.

Active NET formation in Libman–Sacks endocarditis without antiphospholipid antibodies: A dramatic onset of systemic lupus erythematosus

Although neutrophil extracellular traps (NETs) have been highlighted in several systemic inflammatory diseases, their clinical correlates and potential pathological role remain obscure. Herein, we describe a dramatic onset of systemic lupus erythematosus (SLE) with clear-cut pathogenic implications for neutrophils and NET formation in a young woman with cardiac (Libman–Sacks endocarditis) and central nervous system (psychosis and seizures) involvement. Despite extensive search, circulating antiphospholipid autoantibodies, a hallmark of Libman–Sacks endocarditis, could not be detected. Instead, we observed active NET formation in the tissue of the mitral valve, as well as in the circulation. Levels of NET remnants were significantly higher in serially obtained sera from the patient compared with sex-matched blood donors (p = .0011), and showed a non-significant but substantial correlation with blood neutrophil counts (r = 0.65, p = .16). The specific neutrophil elastase activity measured in serum seemed to be modulated by the provided immunosuppressive treatment. In addition, we found anti-Ro60/SSA antibodies in the cerebrospinal fluid of the patient but not NET remnants or increased elastase activity. This case illustrates that different disease mechanisms mediated via autoantibodies can occur simultaneously in SLE. NET formation with release of cytotoxic NET remnants is a candidate player in the pathogenesis of this non-canonical form of Libman–Sacks endocarditis occurring in the absence of traditional antiphospholipid autoantibodies. The case description includes longitudinal results with clinical follow-up data and a discussion of the potential roles of NETs in SLE.

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

Systemic lupus erythematosus (SLE) is a common rheumatologic condition with known GI involvement. Acute pancreatitis (AP) is a rare GI complication of SLE and is typically associated with increased disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multi-organ dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare complicated by MAS in an adult patient successfully treated with anakinra.

Outcomes in juvenile onset lupus: single center cohort from a developing country.

Introduction About 10-20% of systemic lupus erythematosus (SLE) patients have onset in childhood and have more severe organ involvement. Survival of juvenile SLE patients is improving worldwide. Long-term data of childhood onset SLE from developing countries is scarce. Methods Clinical and laboratory data at initial presentation and follow-up visits were retrieved from clinic files, hospital information system and personal interviews. Treatment received, complications, flares, outcomes and death were recorded. Survival was calculated using Kaplan-Meier survival curves and regression analysis was done for predictors of mortality. Results Children with SLE ( n = 273, 250 girls) had a median age at onset of 14 years and duration of illness prior to diagnosis at our hospital of 1 year. Fever and arthritis were the most common presenting manifestations. Renal disease was seen in 60.5% and central nervous system (CNS) disease in 29%. The median follow-up period in 248 patients was 3.5 years. Fourteen children died, and 10 of these had active disease at the time of death. The mean actuarial survival was 24.5 years and survival rates at 1, 5 and 10 years were 97.9%, 95% and 89% respectively. Fever, CNS disease, anti-dsDNA levels and serious infections predicted death on univariate and multivariate analysis. Infections were seen in 72 children (26.3%), and 38 of these infections were serious. One-third of the patients had damage on the last follow-up. Flares were seen in 120 children, the majority being major flares. Conclusion Outcomes of pediatric SLE in North Indian children are similar to those seen in developed countries. Infections pose a major challenge in these patients.

Late Onset Systemic Lupus Erythematosus in an Afro-Caribbean Patient: A Diagnostic Challenge

Systemic Lupus Erythematosus may be missed in the elderly population as the clinical features may resemble more common conditions. Treatment remains a challenge in this group given the co-existing chronic illnesses and drug-related toxicities. Herein, we report a case of a 72-year-old Afro-Caribbean woman diagnosed with systemic lupus erythematosus after having pulmonary and renal manifestations.

Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center.

The aim was to describe the macrophage activation syndrome (MAS), a life-threatening syndrome characterized by excessive immune activation that can be triggered by conditions affecting immune homeostasis, in a cohort of adult Italian patients with systemic lupus erythematosus (SLE). This was a monocentric retrospective evaluation. The utility of the H-score, developed to estimate the individual risk of having reactive MAS in adult patients, was assessed. Among 511 patients with SLE, 7 cases (1.4%) of MAS (all females) were identified and their medical records reviewed. In all cases, MAS was simultaneous to the onset of SLE. All patients had fever, lymphadenopathy, hematological involvement, and high titer of anti-dsDNA antibodies. Workup for infections and malignancies was negative. In all cases, the H-score was higher than the cut-off suggested for the classification of reactive MAS. All cases required hospital admission, and 2 patients were admitted to the intensive care unit. Most patients were treated successfully with high doses of corticosteroids and with immunosuppressive drugs, whereas the full therapeutic regimen developed for primary hemophagocytic lymphohistiocytosis HLH was used only in one case. No death from MAS was observed. MAS is a rare and severe disorder that complicated the onset of SLE in our cohort. The H-score may be useful in the classification of these patients.

Genetic variants in systemic lupus erythematosus susceptibility loci, XKR6 and GLT1D1 are associated with childhood-onset SLE in a Korean cohort

Impact of genetic variants on the age of systemic lupus erythematosus (SLE) onset was not fully understood. We investigated a cumulative effect of SLE-risk variants on the age of SLE onset and scanned genome-wide SNPs to search for new risk loci of childhood-onset SLE (cSLE). We analyzed 781 Korean single-center SLE subjects who previously genotyped by both Immunochip and genome-wide SNP arrays. Individual genetic risk scores (GRS) from well-validated SLE susceptibility loci were calculated and tested for their association with cSLE (<16 years at onset). Single-variant association tests were performed using a multivariable logistic regression adjusting for population stratification. GRS from SLE susceptibility loci was significantly higher in cSLE than aSLE (p = 1.23 × 10−3). Two SNPs, rs7460469 in XKR6 (p = 1.26 × 10−8, OR = 5.58) and rs7300146 in GLT1D1 p = 1.49 × 10−8, OR = 2.85), showed the most significant associations with cSLE. The model consisting of GRS of SLE and two newly identified loci showed an area under curve (AUC) of 0.71 in a receiver operating characteristics (ROC) curve for prediction of cSLE. In conclusion, cSLE is associated with a high cumulative SLE-risk effect and two novel SNPs rs7460469 and rs7300146, providing the first predictive model for cSLE in Koreans.

Pregnancy-triggered triple autoimmunity (Hashimoto’s thyroiditis, antiphospholipid syndrome and systemic lupus erythematosus)

Introduction: We describe a case of 22 year old female with her first pregnancy triggered Hashimoto’s thyroiditis (HT), Antiphospholipid Syndrome (APS) and Systemic Lupus Erythematosus (SLE). Case Report: A 22 year old female was diagnosed with HT on levothyroxine during the early first trimester. During 21 weeks of gestation patient has intrauterine fetal demise and underwent medical abortion. SLE work up including antinuclear antibody and anti-double stranded DNA were positive. She underwent kidney biopsy, which revealed membranous and mesangial proliferative lupus nephritis. Diagnosis of SLE and APS was made. Treatment with anticoagulation therapy was started. SLE therapy was initiated with prednisone, mycophenolate mofetil and hydroxychloroquine with complete resolution of symptoms.We report a 23-year-old gravida in her first pregnancy, suffering from MGN and severe nephrotic syndrome, complicated by APLA syndrome. The patient was treated with enoxaparin, aspirin azathioprine, and Prednisone for a short time, in addition to furosemide and albumin intravenously. She was delivered at 30 weeks due to deteriorating maternal and foetal conditions. Discussion: APS is a prothrombotic disorder with various manifestations, most commonly venous and arterial thromboembolism and recurrent pregnancy loss. Pregnancy may trigger an underlying APS, which may well be the causative for the miscarriage. New onset SLE during pregnancy is rare. However, in our case, the anemia, thrombocytopenia, and proteinuria led us to the correct diagnosis of SLE. HT is associated with higher rates of infertility and early miscarriages, due to the associated hormonal changes and instability. However, the association of APS and HT is not well recognized in pregnant women. Conclusion: We present here a challenging case of new-onset triple autoimmune disorders trigged by pregnancy. Clinicians should be aware of this association and initiate early autoimmune work up for SLE and APS in patients with new onset of HT during pregnancy. A successful neonatal and maternal outcome was achieved in this case. The patient's history revealed thrombocytopenia and APLA syndrome and continues to be treated chronically with enoxaparin. Kidney biopsy performed after delivery showed membranous MGN stage II-III. Herein, we present a case of successful pregnancy and foetal outcome in a young woman with APLA syndrome and MN. Keywords: Pregnancy triggered Hashimoto’s thyroiditis, Antiphospholipid Syndrome and Systemic Lupus Erythematosus

Pregnancy-triggered triple autoimmunity (Hashimoto’s thyroiditis, antiphospholipid syndrome and systemic lupus erythematosus)

Introduction: We describe a case of 22 year old female with her first pregnancy triggered Hashimoto’s thyroiditis (HT), Antiphospholipid Syndrome (APS) and Systemic Lupus Erythematosus (SLE). Case Report: A 22 year old female was diagnosed with HT on levothyroxine during the early first trimester. During 21 weeks of gestation patient has intrauterine fetal demise and underwent medical abortion. SLE work up including antinuclear antibody and anti-double stranded DNA were positive. She underwent kidney biopsy, which revealed membranous and mesangial proliferative lupus nephritis. Diagnosis of SLE and APS was made. Treatment with anticoagulation therapy was started. SLE therapy was initiated with prednisone, mycophenolate mofetil and hydroxychloroquine with complete resolution of symptoms. We report a 23-year-old gravida in her first pregnancy, suffering from MGN and severe nephrotic syndrome, complicated by APLA syndrome. The patient was treated with enoxaparin, aspirin azathioprine, and Prednisone for a short time, in addition to furosemide and albumin intravenously. She was delivered at 30 weeks due to deteriorating maternal and foetal conditions. Discussion: APS is a prothrombotic disorder with various manifestations, most commonly venous and arterial thromboembolism and recurrent pregnancy loss. Pregnancy may trigger an underlying APS, which may well be the causative for the miscarriage. New onset SLE during pregnancy is rare. However, in our case, the anemia, thrombocytopenia, and proteinuria led us to the correct diagnosis of SLE. HT is associated with higher rates of infertility and early miscarriages, due to the associated hormonal changes and instability. However, the association of APS and HT is not well recognized in pregnant women. Conclusion: We present here a challenging case of new-onset triple autoimmune disorders trigged by pregnancy. Clinicians should be aware of this association and initiate early autoimmune work up for SLE and APS in patients with new onset of HT during pregnancy. A successful neonatal and maternal outcome was achieved in this case. The patient's history revealed thrombocytopenia and APLA syndrome and continues to be treated chronically with enoxaparin. Kidney biopsy performed after delivery showed membranous MGN stage II-III. Herein, we present a case of successful pregnancy and foetal outcome in a young woman with APLA syndrome and MN. Keywords: Pregnancy triggered Hashimoto’s thyroiditis, Antiphospholipid Syndrome and Systemic Lupus Erythematosus