Systemic Lupus Erythematosus Syndrome Research Articles

ANA Profile in Population of West Bengal

Antinuclear antibodies (ANA) are significant biomarkers that are used to detect and classify autoimmune connective tissue disorders more efficiently. This paper provides a thorough overview of all the ANA profiling procedures, their testing advancements, clinical significance, and future directions. This study aimed to analyze the ANA profiles along with the prevalence of specific antibodies in patients presumed to have been diagnosed with autoimmune disorders in an Eastern Indian tertiary care hospital. The results were correlated with demographic data to conclude. ANA profiles of 48 patients from Peerless Hospitex Hospital, Kolkata were evaluated between January 24 to June 15, 2024. Out of them, 13 patients (27%) had positive ANA profiles. The most prevalent autoantibodies detected were SS-A and SS-B, which occurred in 7 patients (53.85%), followed by PM-Scl in 4 patients (30.77%). The study demonstrates how the ANA profile varies among different age groups, with the highest prevalence of 6 patients (46.15%) seen in the 60–75 age range. The clinical symptoms of diseases like autoimmune hepatitis, polymyositis, dermatomyositis, PSS (progressive systemic sclerosis), SLE (Systemic Lupus Erythematosus), and Sjogren's syndrome align with the ANA profile findings. This study offers insights into the distribution of autoantibodies targeting antigens present in ANA profiles, using clinical samples collected from a 550-bed tertiary care hospital in Eastern India. The findings underscore the need for comprehensive diagnostic approaches to autoimmune disorders.

Restless leg syndrome in systemic lupus erythematosus: Associations with disease activity, quality of life and depression

ObjectivesThe aim of this study was to determine the prevalence of restless leg syndrome (RLS) among patients with SLE, describe their clinical characteristics, examine its impact on health-related quality of life (HRQoL), and evaluate its association with depression. MethodsA total of 124 SLE patients were recruited, with data on demographics, and clinical features collected. RLS diagnosis was based on the international RLS study group criteria, while depression was assessed by the patient health questionnaire. HRQoL was assessed by a disease-specific validated questionnaire, the LupusQoL, pain intensity was examined through the pain visual analogue scale, and disease activity was evaluated via the patient global assessment. These variables were compared between SLE patients with RLS and without RLS using t-tests or Wilcoxon and the chi-square test of independence for categorical variables. A p-value ≤0.05 was considered statistically significant. ResultsAmong the SLE patients (mean age 48, 87.1 % women), 32 % had RLS. The SLE patients with RLS were found to have a longer delay in diagnosis (1 vs 0.5 years; p = 0.019) and were less likely to be employed (65 % vs 45 %, p = 0.040) compared to non-RLS patients. In addition, RLS patients were more likely to have coexisting Major Depressive Disorder (MDD) (p = 0.019), higher levels of pain (p = 0.006) and disease activity based on patient global assessment (p = 0.014). Further, most of the domains of LupusQoL were significantly lower in the RLS patients group suggesting a worse HRQoL. ConclusionRLS was present in one-third of the SLE cohort, significantly impairing HRQoL and correlating with depression, higher pain, and increased disease activity. These findings underscore the importance of early RLS detection and management in SLE patients.

SLE: a cognitive step forward-a synthesis of rethinking theories, causality, and ignored DNA structures.

Systemic lupus erythematosus (SLE) is classified by instinctual classification criteria. A valid proclamation is that these formally accepted SLE classification criteria legitimate the syndrome as being difficult to explain and therefore enigmatic. SLE involves scientific problems linked to etiological factors and criteria. Our insufficient understanding of the clinical condition uniformly denoted SLE depends on the still open question of whether SLE is, according to classification criteria, a well-defined one disease entity or represents a variety of overlapping indistinct syndromes. Without rational hypotheses, these problems harm clear definition(s) of the syndrome. Why SLE is not anchored in logic, consequent, downstream interdependent and interactive inflammatory networks may rely on ignored predictive causality principles. Authoritative classification criteria do not reflect consequent causality criteria and do not unify characterization principles such as diagnostic criteria. We need now to reconcile legendary scientific achievements to concretize the delimitation of what SLE really is. Not all classified SLE syndromes are "genuine SLE"; many are theoretically "SLE-like non-SLE" syndromes. In this study, progressive theories imply imperative challenges to reconsider the fundamental impact of "the causality principle". This may offer us logic classification and diagnostic criteria aimed at identifying concise SLE syndromes as research objects. Can a systems science approach solve this problem?

CARDIOVASCULAR INVOLVEMENT IN SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract Background Cardiovascular involvement is frequent in systemic lupus erythematosus (SLE). Aortic insufficiency is a common valvular abnormality seen in patients with systemic lupus erythematosus (SLE), it may be caused by a primary valve pathology or by an aortitis One of the cardiac manifestations associated with SLE and antiphospholipid syndrome (APLS) is Libman–Sacks endocarditis, also known as non–bacterial thrombotic endocarditis Although rare, there are reported cases of lupus aortitis which has also been associated with dissection, aneurysm and thrombus. Case Report We present a case of a 40 –years–old lady with lupus glomerulonephritis requiring immunosuppression and dialysis, who presented to the emergency department with fever and dyspnea. She was first treated with dialysis. Blood cultures drawn from central venous catheter peripheral were positive for staphylococcus epidermidis, the cultures from peripheral blood was negative instead.Transthoracic and transesophageal Echocardiography showed severe aortic regurgitation (not present three month earlier) with doubt valvular vegetations and with a focal enlargement of the aortic root (figure 1, 2).The patient met one major criterion (blood culture) and two minor criteria (high risk and fever), implying possible infective endocarditis. The patient was then started on intravenous antibiotics (vancomycin) and immunosuppression was reduced. Given the hemodynamic instability due to the severe aortic insufficiency, an Heart CT scan was performed to planned the surgical intervention. The CT scan confirmed the presence of small, multiple vegetation attached to the aortic leaflet and an aortic dissection on the right coronary cusp (figure 3). She proceeded to urgent surgery with aortic valve and aortic root replacement, the histology revealed evidence of Libman–Sacks endocarditis and aortitis. Discussion Our patient showed two different cardiovascular complications of SLE: the more frequent Libman –Sack endocarditis and the rare lupus aortitis associated to aortic dissection.The patient had risk factors for infective endocarditis and she did fulfil the modified Duke’s criteria for infective endocarditis and was treated with antibiotics and with a reduction of immunosuppression therapy. In fact, it can be difficult to distinguish between infective endocarditis and Libman–Sacks endocarditis especially in the setting of risk factors for both. Antibiotics and immunosuppressants should be used both.

Regulatory B Cells Evaluation in Systemic Lupus Erythematosus Patients with Subclinical Atherosclerosis and Secondary Antiphospholipid Syndrome.

The current knowledge of human studies that address B cells in Systemic Lupus Erythematosus (SLE) patients with subclinical atherosclerosis remains insufficient. We aimed to evaluate the contribution of Breg cells in SLE and secondary antiphospholipid syndrome (APS) patients taking into consideration its relation to subclinical atherosclerosis and the disease activity. Thirty SLE patients and 23 controls were included. Systemic Lupus Erythematosus Disease Activity Index-2000 was estimated. Evaluation of Breg cells percentage using flow cytometry was done. All participants underwent carotid doppler ultrasound examination for measurements of the intima-media thickness of the common carotid artery (cIMT). The coronary artery calcium scoring was calculated using the Agatston method. The mean± SD of age was 32.60±8.34 years, while of the age of onset was 28.27±7.60 years. Twenty-three patients (76.7%) had subclinical atherosclerosis. There was a highly significant difference in Breg cells between SLE and APS patients with subclinical atherosclerosis and controls (P= 0.001, 0.005). SLE and APS patients had significantly higher mean cIMT than control (P=0.01, 0.050). Breg cells had 70% sensitivity and 87% specificity for diagnosing of SLE (P=0.01). Multivariate regression analysis indicated that low Breg cells were predictive for the disease activity (OR=1.76, 95% CI=1.21- 2.85; P= 0.01). SLE patients had a high frequency of subclinical atherosclerosis, those and patients with secondary APS had a high risk of plaque formation. We found a contribution of Breg cells in SLE patients with subclinical atherosclerosis. Breg cells are considered a good predictor of diagnosis of SLE.

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.

S2741 Cyclic Vomiting Syndrome in Systemic Lupus Erythematosus: A Diagnostic Conundrum and Therapeutic Success with Mycophenolate Mofetil

S2741 Cyclic Vomiting Syndrome in Systemic Lupus Erythematosus: A Diagnostic Conundrum and Therapeutic Success with Mycophenolate Mofetil

Macrophage activation syndrome in systemic lupus erythematosus - a case report

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition that results from the immune system hyperactivation. The primary form of HLH appears mainly in children and is caused by a genetically determined failure of NK and T cells. Secondary (acquired) HLH usually occurs due to insufficiency of these cells caused by other disease entities or drugs. Potential factors that may be responsible for the appearance of this rare condition include: malignancies (especially lymphomas), infections (EBV, CMV, HIV), autoimmune diseases (SLE, Still’s disease). The clinical appearance of hemophagocytic lymphohistiocytosis is dominated by persistent fever, hepatomegaly, splenomegaly, pallor of the skin, signs of hemorrhagic diathesis, lymph node enlargement. Characteristic abnormalities in laboratory tests include: hypertriglyceridemia, cytopenia, hyperferritinemia, hyperfibrinogenemia and hypertransaminasemia. One subtype of the secondary form of hemophagocytic lymphohistiocytosis, which is associated with rheumatic diseases, is called the macrophage activation syndrome (MAS). The following describes a case of a 26-year-old woman with exacerbated systemic lupus erythematosus, who was admitted to a Rheumatology Unit in severe general condition. Clinical presentation of the patient included primarily: long-lasting fever, hepatomegaly and splenomegaly. Various test were performed during her hospitalization, including laboratory test and a bone marrow biopsy. Finally, the woman was diagnosed with the macrophage activation syndrome. She was successfully treated with ciclosporin and methylprednisolone.

The Prevalence of Dry Eye Syndrome in Systemic Lupus Erythematosus Patients in Saudi Arabia

INTRODUCTION: Systemic Lupus Erythematosus (SLE) is a chronic multi-systemic autoimmune connective tissue disorder that is known to have variable manifestations with a remitting/relapsing course depending on the affected system. SLE can affect all the major organs in the human body including the heart, brain, and kidneys. Although the eye is not a primary target of autoimmune insult in SLE patients, one third of patients can present with significant ocular manifestations as it can affect any part of the ocular system. Furthermore, ocular involvement could be the first presenting symptom of SLE and may mirror the systemic disease activity. The worldwide prevalence of SLE varies, however, based on a study conducted in 2002 in central Saudi Arabia, the prevalence of SLE was estimated to be 19.28 per 100,000.&#x0D; &#x0D; STUDY AIM: To identify the prevalence of dry eye syndrome in Systemic Lupus Erythematosus diagnosed patients in Riyadh.&#x0D; &#x0D; METHODS: A cross sectional study that involved a total of 135 patients from Rheumatology outpatient clinic setting at King Khalid University Hospital in Riyadh, Kingdom of Saudi Arabia. Data was collected via McMonnies questionnaire, which is used as a screening tool for dry eye syndrome. Data analysis included descriptive statistics computed for continuous variables, including means, standard deviation (SD), minimum and maximum values, as well as 95% confidence interval (CI). Percentages and 95% CI were used for categorical variables.&#x0D; &#x0D; RESULTS: Out of 135 SLE patients, females were 87.4% &amp;plusmn;2.9 (95%CI: 80.6&amp;ndash;92.1.3), while males were 12.6% &amp;plusmn;2.9 (95%CI: 7.9&amp;ndash;19.4), Among the 118 female patients, 56% (n=66) lied between the age of 25-45 years. Most common symptom in 45.9% (95%CI: 37.6&amp;ndash;54.5) of our patients was Dryness of Eyes. The predominant age group of our cohort was that of 25&amp;ndash;45 years (57%) Among our cohort, 50.4% (95%CI: 41.9&amp;ndash;58.8) patients suffered from Dry eye syndrome, with 12.6% males and 87.4% females.&#x0D; &#x0D; CONCLUSION: A high prevalence of dry eye syndrome was found in our study with a percentage of 50.4%, also a female predominance was found in patients having dry eye syndrome.

The pathogenic role of lupus-specific autoantibodies and Interleukin-6 on demyelination of the brainstem and spinal cord in systemic lupus erythematosus.

Demyelinating syndromes that result in brainstem and/or spinal cord lesions similar to those observed in neuromyelitis optica spectrum disorder (NMOSD) as neuropsychiatric syndromes in systemic lupus erythematosus (NPSLE) occasionally develop in patients with SLE. Cerebrospinal fluid (CSF) interleukin (IL)-6 is a known biomarker for NMOSD; however, its application in patients with SLE with brainstem and/or spinal cord lesions is unknown. Additionally, the breakdown of blood-brain barrier (BBB) integrity by autoantibodies is another mechanism of NMOSD; however, it is not elucidated in SLE. Therefore, this study was designed to clarify the use of CSF IL-6 and investigate whether autoantibodies contribute to BBB breaches and the development of brainstem and/or spinal cord lesions. Data from patients with NPSLE who had NMOSD-like demyelinating lesions in the central nervous system (CNS), including brainstem and/or spinal cord lesions, were retrospectively analyzed. We retrospectively investigated the interval changes in CSF IL-6 and clinical and serological factors related to BBB permeability using CSF/serum albumin ratio (QAlb). Twelve patients with NPSLE who had demyelinating lesions in the brainstem and/or spinal cord were recruited. Before treatment, CSF IL-6 levels were 29.1pg/mL and significantly decreased to 3.8pg/mL by treatment (p = 0.008). Before treatment, CSF IL-6 was significantly correlated with the anti-dsDNA antibody titer (p = 0.027). Furthermore, before treatment, QAlb was significantly correlated with the serum anti-Smith antibody titer. In patients with atypical NMOSD who had specific lesions defined in the NMOSD diagnostic criteria but were negative for antiaquaporin four antibody, a significant correlation was observed between the serum anti-Smith antibody titer and CSF IL-6 (p = 0.025) and QAlb (p = 0.033) values before treatment. CSF IL-6 could be a surrogating marker for disease activity, and serum anti-Smith antibody permeabilizes the BBB in patients with NPSLE, supporting the development of NMOSD-like CNS lesions.

Differences and similarities in cytokine profiles of macrophage activation syndrome in systemic lupus erythematosus and adult-onset Still's disease.

To clarify the differences and similarities in the cytokine profiles of macrophage activating syndrome (MAS) between systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD). The study participants included 9 patients with MAS-SLE, 22 with non-MAS-SLE, 9 with MAS-AOSD, and 13 with non-MAS-AOSD. Serum cytokine levels were measured using a multiplex bead assay. Cytokine levels were compared between patients with SLE and AOSD with/without MAS. Moreover, cytokine patterns were examined using principal component analysis (PCA) and cluster analysis. IL-6, IL-8, IL-18, and TNF-α levels were elevated in patients with SLE and AOSD. IFN-α levels were elevated in SLE, whereas IL-1β and IL-18 levels were elevated in AOSD. In SLE, IFN-α and IL-10 levels were higher in MAS than in non-MAS and controls. PCA revealed distinctive cytokine patterns in SLE and AOSD, SLE with IFN-α and IP-10, AOSD with IL-1β, IL-6, and IL-18, and enhanced cytokine production in MAS. PCA and cluster analysis showed no differences in cytokine patterns between the MAS and non-MAS groups. However, serum ferritin levels were correlated with IFN-α levelsin SLE. Cytokine profiles differed between SLE and AOSD but not between MAS and non-MAS. MAS is induced by the enhancement of underlying cytokine abnormalities rather than by MAS-specific cytokine profiles. Type I IFN may be involved in MAS development in patients with SLE.

Challenges in Family Planning and Pregnancy for Women with Rheumatic Diseases

Systemic rheumatic diseases (RDs) often affect women in their reproductive years and may complicate family planning and pregnancy. For women with RD who are not pursuing pregnancy, especially those at the highest risk of maternal morbidity or on teratogenic medications, effective contraception is important. For women with active systemic lupus erythematosus (SLE) and/or antiphospholipid antibodies, oestrogen-containing contraceptive methods are generally not recommended. Emergency contraception and induced abortion are safe for women with RD and should be discussed when clinically appropriate. Women with RD commonly have questions and concerns about the impact of their disease on fertility and conception. RD-associated factors may contribute to difficulties in conceiving and decreased family size. Assisted reproductive technology (ART) is often a safe option for women with RD. However, precautions and specific treatment modifications may be needed, particularly in women with SLE and/or antiphospholipid antibody positivity. SLE and antiphospholipid syndrome (APS) are the RDs associated with the greatest risk of maternal and foetal pregnancy complications. Regardless of the specific RD, having an active disease is a significant risk factor for poor pregnancy outcomes. With appropriate preconception planning and multidisciplinary management during and after pregnancy, most women with RD can have successful pregnancies.

Editorial introductions.

Editorial introductions.

Overlap syndrome in a 12-year-old girl with systemic lupus erythematosus and anti-oj antibody-positive polymyositis: a case report

BackgroundThe peculiar presentation of overlap syndrome in children makes precise diagnosis difficult. Children with overlap syndrome may or may not have specific antibodies. We present the case of a 12-year-old girl diagnosed with overlap syndrome of systemic lupus erythematosus (SLE) and juvenile polymyositis (JPM) who tested positive for anti-OJ antibodies.Case presentationWe describe the case of a 12-year-old girl diagnosed with SLE at the age of 7 and presented with fever with malar rash, periungual erythema, generalized weakness, and multiple joint pain at admission. The patient had persistent joint pain and weakness after intravenous methylprednisolone administration and complained of an inability to walk with a positive test for Gower's sign one week after admission, accompanied by elevated alanine aminotransferase (ALT) and creatine-phospho-kinase (CPK) levels. The results of nerve conduction velocity test were normal. Electromyography revealed abundant spontaneous activity and myopathic motor unit action potentials in the right deltoid, biceps, and iliopsoas, in addition to fibrillation and mild myopathic motor unit action potentials in the right rectus femoris muscle. Magnetic resonance imaging revealed diffusely increased signal intensities in the myofascial planes of the bilateral iliopsoas, gluteus, obturator, pectineus, and hamstring muscles. Anti-nuclear antibody, anti-RNP, and rheumatoid factor IgG tests were positive, and inflammatory myopathy autoantibodies revealed anti-OJ antibody positivity, which strongly indicated autoimmune myositis. High-resolution computed tomography of the lung revealed mild pericardial effusion without any evidence of interstitial lung disease. We initiated intravenous pulses of methylprednisolone treatment, followed by cyclosporine, mycophenolate mofetil, and oral steroids. Clinical improvement with a delayed, slowly reduced CPK level after the above treatment and she was discharged after the 18th day of hospitalization.ConclusionOverlap syndrome with inflammatory myositis can occur years later in pediatric SLE cases. We should be alert when patients with SLE develop a new presentation characterized by decreased SLE-specific autoantibody titers, positive anti-RNP antibodies, and elevated CPK. Treatment of the overlap syndrome of SLE and JPM is individualized, and the course differs between pediatric and adult patients.

SHRINKING LUNG SYNDROME IN SYSTEMIC LUPUS ERYTHEMATOSUS: DIAGNOSIS OF RARE ENTITY

SHRINKING LUNG SYNDROME IN SYSTEMIC LUPUS ERYTHEMATOSUS: DIAGNOSIS OF RARE ENTITY

SHRINKING LUNG SYNDROME: A RARE COMPLICATION OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

SHRINKING LUNG SYNDROME: A RARE COMPLICATION OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

Secondary antiphospholipid syndrome in systemic lupus erythematosus – screening, diagnosis and treatment methods

Systemic Lupus Erythematosus is the hallmark of autoimmune diseases, being characterized by multiple organ involvements and immune abnormalities, amongst which the presence of antiphospholipid antibodies with or without specific clinical manifestations (vascular thromboses and pregnancy morbidities) has a significant impact on the disease course, both short and long term, causing the accumulation of irreversible damage. This study evaluates the aforementioned impact, highlighting the importance of very early screening for these antibodies.

The Intersection of Cellular and Systemic Metabolism: Metabolic Syndrome in Systemic Lupus Erythematosus.

A high prevalence of metabolic syndrome (MetS) has been reported in multiple cohorts of systemic lupus erythematosus (SLE) patients, most likely as one of the consequences of autoimmune pathogenesis. Although MetS has been associated with inflammation, its consequences on the lupus immune system and on disease manifestations are largely unknown. The metabolism of immune cells is altered and overactivated in mouse models as well as in patients with SLE, and several metabolic inhibitors have shown therapeutic benefits. Here we review recent studies reporting these findings, as well as the effect of dietary interventions in clinical and preclinical studies of SLE. We also explore potential causal links between systemic and immunometabolism in the context of lupus, and the knowledge gap that needs to be addressed.

Surgical treatment of acute aortic dissection in a patient with SLE and prior antiphospholipid syndrome on therapy for over 30 years: a case report

BackgroundIn patients with systemic lupus erythematosus (SLE), lengthy treatment and long-term steroid use are the main risk factors for developing aortic aneurysms or aortic dissections. In patients with cardiac tamponade, hemodynamic collapse may lead to acute renal and hepatic failure.Case presentationWe report the successful treatment of a 55-year-old woman with SLE since the age of 21. She suddenly felt chest pain approximately 2 weeks before developing fever and vomiting and was admitted to our hospital. Initially, she had severe liver dysfunction and was admitted to the hepatology department, where treatment for fulminant hepatitis was initiated. However, computed tomography (CT) showed an acute aortic dissection (DeBakey type II) and severe bloody pericardial effusion. Therefore, we performed emergency pericardial drainage. Plasma exchange therapy was initiated as emergency aortic surgery was deemed impossible due to impaired liver function tests and coagulation. Ten days later, the patient developed peritonitis due to small bowel perforation, and laparotomy was performed for abscess drainage and perforation closure. She had received steroid pulse therapy at the age of 21. At 40 years of age, she developed deep vein thrombosis due to antiphospholipid antibodies and was prescribed prednisolone. She was ambulatory at 3 months after the onset of acute aortic dissection, and CT revealed a rapidly enlarging true aneurysm in the distal arch. We performed elective aortic surgery. Although there were no antiphospholipid antibodies, surgery could have led to a devastating antiphospholipid syndrome. Therefore, we decided to treat the patient with triple therapy. Methylprednisolone was intravenously administered intraoperatively and at 1 day postoperatively. The patient was discharged without complications after returning to her usual oral prednisolone regimen.ConclusionsThe patient described herein had a systemic circulatory failure due to cardiac tamponade, accompanied by liver failure. This condition is a significant cause of death in patients with aortic dissection-associated SLE and is extremely dangerous. However, multi-specialty intervention helped the patient recover, and she has been attending the outpatient clinic. Aortic surgery requiring hypothermia in SLE patients with antiphospholipid syndrome and a history of thrombocytopenia or thrombosis requires a multi-disciplinary treatment team, including cardiac surgeons and medical experts.

Demyelinating Syndromes in Systemic Lupus Erythematosus: Data From the "Attikon" Lupus Cohort.

BackgroundThe demyelinating syndromes of the central nervous system (CNS) that occur in the context of systemic lupus erythematosus (SLE) may represent a manifestation of neuropsychiatric lupus (NPSLE) or an overlap of SLE and multiple sclerosis (MS). The differential diagnosis between the two entities has important clinical implications because the therapeutic management differs.ObjectivesTo characterize CNS demyelinating syndromes in a large SLE cohort as neuropsychiatric SLE (NPSLE) or SLE-MS overlap using a multidisciplinary approach and existing diagnostic (for MS) and classification criteria (for SLE).MethodsPatients from the “Attikon” lupus cohort (n = 707) were evaluated for demyelinating syndromes. Clinical, laboratory, and neuroimaging data were recorded for each patient. Following multidisciplinary evaluation and application of criteria, the demyelinating syndrome was attributed to either SLE or MS. Patients with transverse myelitis were not included in this study.ResultsWe identified 26 patients with demyelinating syndromes (3.7%). Of them, 12 were diagnosed as primary SLE-demyelination (46.2%) and 14 as overlap SLE-MS (53.8%). The two groups did not differ with respect to rheumatologic and neurologic manifestations or autoantibodies. SLE patients with demyelination manifested mild extra-CNS disease mainly involving joints and skin, while severe non-CNS manifestations were rare. However, these patients were less likely to have elevated IgG index (OR 0.055 95% CI: 0.008–0.40) and positive oligoclonal bands (OR 0.09 95% CI: 0.014–0.56), as well as brain lesions in the spinal cord, infratentorial, periventricular, and juxtacortical regions. A single brain region was affected in 9 patients with SLE-demyelination (75%), while all patients with MS-SLE had multiple affected brain regions. MS-SLE overlap was associated with an increased likelihood of neurologic relapses (OR 18.2, 95% CI: 1.76–188), while SLE-demyelination patients were less likely to exhibit neurological deficits (EDSS >0) at the last follow-up visit (50 vs. 78.6% in SLE-MS, respectively).ConclusionsDemyelination in the context of SLE follows a more benign course compared to a frank SLE-MS overlap. Extension of follow-up will ascertain whether patients with SLE-demyelination evolve to MS, or this is a bona fide NPSLE syndrome.