Systemic Lupus Erythematosus Syndrome Research Articles

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.

Myositis with prominent B-cell aggregates causing shrinking lung syndrome in systemic lupus erythematosus: a case report

BackgroundShrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE) characterized by decreased lung volumes and diaphragmatic weakness in a dyspneic patient. Chest wall dysfunction secondary to pleuritis is the most commonly proposed cause. In this case report, we highlight a new potential mechanism of SLS in SLE, namely diaphragmatic weakness associated with myositis with CD20 positive B-cell aggregates.Case presentationA 51-year-old Caucasian woman was diagnosed with SLE and secondary Sjögren’s syndrome based on a history of pleuritis, constrictive pericarditis, polyarthritis, photosensitivity, alopecia, oral ulcers, xerophthalmia and xerostomia. Serologies were significant for positive antinuclear antibodies, anti-SSA, lupus anticoagulant and anti-cardiolopin. Blood work revealed a low C3 and C4, lymphopenia and thrombocytopenia. She was treated with with low-dose prednisone and remained in remission with oral hydroxychloroquine. Seven years later, she developed mild proximal muscle weakness and exertional dyspnea. Pulmonary function testing revealed a restrictive pattern with small lung volumes. Pulmonary imaging showed elevation of the right hemidiaphragm without evidence of interstitial lung disease. Diaphragmatic ultrasound was suggestive of profound diaphragmatic weakness and dysfunction. Based on these findings, a diagnosis of SLS was made. Her proximal muscle weakness was investigated, and creatine kinase (CK) levels were normal. Electromyography revealed fibrillation potentials in the biceps, iliopsoas, cervical and thoracic paraspinal muscles, and complex repetitive discharges in cervical paraspinal muscles. Biceps muscle biopsy revealed dense endomysial lymphocytic aggregates rich in CD20 positive B cells, perimysial fragmentation with plasma cell-rich perivascular infiltrates, diffuse sarcolemmal upregulation of class I MHC, perifascicular upregulation of class II MHC, and focal sarcolemmal deposition of C5b-9. Treatment with prednisone 15 mg/day and oral mycophenolate mofetil 2 g/day was initiated. Shortness of breath and proximal muscle weakness improved significantly.ConclusionDiaphragmatic weakness was the inaugural manifestation of myositis in this patient with SLE. The spectrum of myologic manifestations of myositis with prominent CD20 positive B-cell aggregates in SLE now includes normal CK levels and diaphragmatic involvement, in association with SLS.

Systemic lupus erythematosus presenting as Guillain‒Barre syndrome

A 37-year-old male patient presented with acute symmetrical ascending areflexic flaccid quadriparesis progressive in nature for the past 6 days before admission preceded by tingling sensation in both hands and feet with the right-sided lower motor type of facial nerve palsy for 2 days. Clinically, this was consistent with Guillain–Barre (GB) syndrome and was given intravenous immunoglobulin (IVIG). Cerebrospinal fluid study and nerve conduction study also supported the clinical diagnosis of GB syndrome. He developed Type 2 respiratory failure on the 4th day of admission for which he was intubated. After successful extubation, his weakness even worsened markedly despite IVIG therapy and was subjected to plasmapheresis following which the weakness improved remarkably. On further investigation for the associated fever, arthralgia, pallor, nephritis, antinuclear antibody (3+ coarse speckled pattern), anti-smith antibody, and anti-dsDNA antibody positivity were detected with low serum complement levels, and renal biopsy revealing class III lupus nephritis. He was put on oral corticosteroids and cyclophosphamide which resulted in further clinical improvement. The initial presentation of GB syndrome of systemic lupus erythematosus is uncommon and makes this case interesting.

Neurological polymorphism of systemic lupus erythematosus

Relevance. Damage to the nervous system during the progression of systemic lupus erythematosus (SLE) is not only one of the most common phenomena, but also an unfavorable prognostic criterion for the patient. At the moment, more than 19 types of neurolupus manifestations are known in both adult patients and children. The main problem is the non-specificity and variability of neuropsychiatric symptoms, which can be associated not only with the pathogenesis of SLE, but also with other factors caused by the influence of the environment, psychosomatics, and concomitant diseases. Currently, there is no "gold standard" for diagnosing this type of pathology, and sometimes the selection of therapy does not correspond to the etiology of the lesion. Also, the urgency of this problem proves the lack of a clear classification of neurological complications of SLE, which can be used by specialists at the present time. The links of the pathogenesis of the development of both systemic lupus erythematosus itself and lesions of the nervous system in this disease are not fully understood. The purpose of this study. To study the pathogenesis and symptomatic manifestations of neurolupus, presented in the studies of Russian and foreign scientists, with the subsequent systematization of the information obtained. Material and methods. A study was made of materials from foreign and domestic literature of recent years on the issue of classification, pathogenetic and symptomatic problems of the neurological manifestation of systemic lupus erythematosus. Results. The prevalence of neurolupus among SLE patients reaches 95% of cases, expressed both in a single syndrome and in multiple simultaneous clinical manifestations. Lesions of both the central and peripheral nervous systems, as well as psychological disorders, are caused by the toxic effect of actively produced cytokines and interleukins, the deposition of immune complexes and thrombotic vasculitis, an increase in the BBB permeability and the penetration of autoantibodies tropic to the structures of the nervous tissue through it. At the moment, the classification of 2018 most fully covers all possible morphological manifestations of neurolupus, highlighting focal and diffuse lesions of the central nervous system, as well as peripheral. The most common occurrence among SLE patients is headache, which is not a specific manifestation, strokes, aseptic encephalomyelitis, cognitive disorders and depression. Conclusions. Neurolupus is a very common and poorly understood complication of systemic lupus erythematosus, which needs more detailed coverage in the medical literature. With a wide polymorphism of clinical manifestations, the neuropsychiatric syndrome of SLE is a real threat to the patient's life. The lack of a clear classification in clinical guidelines, huge gaps in knowledge about the pathogenesis of nervous pathologies, the similarity of the clinical picture of other diseases and the undeveloped diagnostic tactics of this pathology can lead to ineffectiveness of the therapy, irreversible damage to the nervous tissue and even death.