Findings In Systemic Lupus Erythematosus Research Articles

Multimodal imaging in systemic lupus erythematosus patients with diffuse neuropsychiatric involvement

The objective of this paper is to investigate conventional and nonconventional brain magnetic resonance imaging (MRI) findings in systemic lupus erythematosus (SLE) patients with diffuse neuropsychiatric involvement (dNPSLE) compared to healthy controls (HCs). Twenty-six (26) SLE patients with one or more diffuse NP syndromes related to the central nervous system (CNS) (dNPSLE) and 36 age- and sex-matched HCs were scanned on a 3T MRI using a multimodal imaging approach. Univariate and multivariate analyses were used to determine MRI-specific measure differences between dNPSLE and HCs for lesion burden, tissue-specific atrophy, magnetization transfer ratio (MTR) and diffusion-tensor imaging (DTI) outcomes. In univariate analyses, dNPSLE patients showed significantly increased T1 lesion number (p = .001) and T1-lesion volume (LV, p = .008) compared to HCs. dNPSLE patients showed decreased whole brain volume (p < .0001), gray matter volume (p < .0001), cortical volume (p < .0001) and increased lateral ventricle volume (p = .004) compared to HCs. dNPSLE patients had increased axial diffusivity (AD) of NAWM (p = .008) and NA brain tissue (p = .017) compared to HCs. In the multivariate regression analysis, decreased cortical volume was associated with SLE (R (2) = 0.59, p < .0001). This study shows that cortical and central atrophy are associated with SLE patients with diffuse CNS syndromes. Microscopic tissue injury in the NAWM on AD DTI measures in SLE patients indicates a predominant reduction of axonal density.

An Evaluation of the Association of Leukopenia and Severe Infection in Patients With Systemic Lupus Erythematosus

Leukopenia is a common finding in systemic lupus erythematosus (SLE) and may contribute to severe infections. The objectives of this study were to determine the prevalence of leukopenia in SLE patients and examine the association between these conditions and severe infections noting the risk factor of severe infections. This study was a prospective inception lupus cohort of newly diagnosed SLE patients seen between May 2007 and June 2011. Only cases that had been observed for a minimum of 1 year or died during the study were included. There were 89 SLE patients (92% females), with their mean (SD) age and disease duration at the study entry of 31.7 (12.2) years and 2.4 (2.9) months. Leukopenia was found at the diagnosis in 51.6% of the cases. The cumulative prevalence of leukopenia, lymphopenia, and neutropenia was observed in 57.3%, 96.6%, and 60.7%, respectively. Persistent lymphopenia, noted continuously for more than or equal to 75% of the observation period, was found in 41.6%, but there was no persistent neutropenia. The incidence rate of severe infection was 12.4 per 100 patient-years. There was no difference of severe infection-free survival rate between patients who ever and never had leukopenia. In the multivariate analysis, using cyclophosphamide was the independent predictor for severe infection in SLE (hazard ratio, 2.73; 95% confidence interval, 1.10-6.77). Leukopenia was common in SLE but usually not persistent. In this study, the presence of leukopenia at any time was not the risk factor for severe infection in SLE. Cyclophosphamide was the important predictor for severe infection in SLE.

Libman–Sacks endocarditis, and other echocardiographic findings in systemic lupus erythematosus: Case report

Case report of a 19 year-old female patient with systemic lupus erythematosus (SLE) who was presented to Ain Shams University Hospital complaining of dyspnea on moderate exertion. Echocardiography showed the presence of sterile vegetation on the mitral valve, Libman–Sacks endocarditis (LSE).

Are Cognitive and Olfactory Dysfunctions in Neuropsychiatric Lupus Erythematosus Dependent on Anxiety or Depression?

Depressed mood and cognitive impairments are common findings in systemic lupus erythematosus (SLE) and frequently coexist. We assessed the neuropsychological functioning of patients with SLE and investigated its association with psychopathological symptoms. A total of 85 patients with SLE (28 with neuropsychiatric syndromes: NPSLE) and 85 healthy control subjects with similar demographic characteristics were asked to perform a series of neuropsychological tests. A self-report questionnaire (the Hospital Anxiety and Depression Scale) was used to screen for psychopathology symptoms. Patients with SLE underwent a neurological examination. Patients with NPSLE were more depressed and were more frequently impaired in cognitive and olfactory functions than controls or non-NPSLE patients. The NPSLE group remained statistically different from the other 2 groups on a series of neuropsychological measures (the Auditory Verbal Learning Test, Trail Making Test - Part A, Nine-Hole Peg Test, and Brief Smell Identification Test) even after control for elevated anxiety and depressed mood. Non-NPSLE and control groups were not significantly different regarding either psychopathological symptoms or neuropsychological functioning. Verbal memory, psychomotor speed, and olfaction are particularly vulnerable to dysfunction in NPSLE; impairment in these neuropsychological domains is not completely explained by psychopathology symptoms.

Retroperitoneal Fibrosis and Aortitis as the Initial Findings of Systemic Lupus Erythematosus

Retroperitoneal Fibrosis and Aortitis as the Initial Findings of Systemic Lupus Erythematosus

Retroperitoneal Fibrosis and Aortitis as the Initial Findings of Systemic Lupus Erythematosus

Retroperitoneal Fibrosis and Aortitis as the Initial Findings of Systemic Lupus Erythematosus

Thin-section chest CT findings in systemic lupus erythematosus with antiphospholipid syndrome: A comparison with systemic lupus erythematosus without antiphospholipid syndrome

To assess thin-section chest CT findings in systemic lupus erythematosus (SLE) with antiphospholipid syndrome (APS), in comparison with SLE without APS. We retrospectively reviewed the medical records and thin-section CT findings of 17 consecutive patients with an established diagnosis of SLE with APS, comparing with 37 consecutive SLE patients without APS, between 2004 and 2008, and patients who had other autoimmune disease, such as Sjögren syndrome, were excluded. No significant differences were seen between the two groups in age, gender, smoking habits, or history of steroid pulse and biological therapy. CT images of 2mm thickness obtained with a 16- or 64-detector row CT were retrospectively evaluated by two radiologists in consensus on ultra high-resolution gray-scale monitors. The frequency of thin-section CT abnormalities was higher in SLE with APS group (82%) than in SLE without APS group (43%). Ground-glass opacity (59%), architectural distortion (47%), reticulation (41%), enlarged peripheral pulmonary artery (29%), and mosaic attenuation (29%) were significantly more common in the SLE with APS group than in the SLE without APS group (Fisher's exact test, p<0.01). SLE patients with APS have increased prevalence of thin-section chest CT abnormalities than those without APS.

Relationship between cardiac symptoms, myocardial perfusion defects and coronary angiography findings in systemic lupus erythematosus

Coronary angiography is generally regarded as the 'gold standard' test for diagnosing coronary artery disease (CAD). We sought to determine the relationship between cardiac symptoms and findings of coronary angiography and myocardial perfusion scintigraphy (MPS) in patients with systemic lupus erythematosus (SLE). Medical records of all SLE patients who underwent coronary angiography while attending our clinic over 24 years were reviewed, noting the indication for the test and its findings. Among patients who had MPS within 6 months prior to coronary angiography, a contingency table was used to rate the agreement between the two tests. Among the 35 patients who underwent coronary angiography, 31 had the test to investigate cardiac symptoms. Among the symptomatic patients, 17 (55%) had an abnormal angiogram with one or more plaques, while 14 (45%) had normal angiograms. All four asymptomatic patients had normal angiograms. Compared to those with normal angiograms, patients with abnormal angiograms had a higher mean number of cardiovascular risk factors per patient (1.6 ± 1.4 vs. 0.6 ± 1.0, p = 0.02). Twenty-four patients had both angiography and MPS. Overall, the agreement between angiography and MPS was poor (κ = 0, p = 0.0008), with 14 (58.3%) patients having perfusion defects and normal angiograms. A proportion of SLE patients with cardiac symptoms do not have plaques on coronary angiography. Overall there is poor agreement between the findings of coronary angiography and MPS in SLE, suggesting mechanisms of ischemia other than plaques.

Linfadenopatia e lúpus eritematoso sistêmico

Lymphadenopathy is a benign finding in systemic lupus erythematosus (SLE), commonly seen in young patients with cutaneous involvement and constitutional symptoms, with good response to corticosteroids. Reactive follicular hyperplasia is the most frequent finding in biopsies. We report the case of a patient with recurrent episodes of lymphadenopathy associated with hepatosplenomegaly, fever, and weight loss since the age of 13 years. The patient also developed arthritis, hypertension, proteinuria, cardiomyopathy, and peripheral neuropathy. His condition was investigated extensively without diagnostic clarification; he was treated, empirically, for tuberculosis. The patient received a diagnosis of SLE only five years after the original presentation and received the specific treatment. Early diagnosis in those cases is difficult because laboratory exams may not show the presence of auto-antibodies and low complement levels.

Renal Disease in Systemic Lupus Erythematosus With Emphasis on Classification of Lupus Glomerulonephritis: Advances and Implications

Systemic lupus erythematosus is an autoimmune disease with protean clinical and pathologic manifestations involving almost all organs in the body. There is a high incidence of renal involvement during the course of the disease, with varied renal pathologic lesions and diverse clinical features. A renal biopsy examined by routine light microscopy, immunofluorescence, and electron microscopy contributes toward diagnosis, prognostic information, and appropriate management. (1) To review the clinical and various pathologic features of renal lesions in systemic lupus erythematosus patients. (2) To introduce the International Society of Nephrology and Renal Pathology Society Classification of Lupus Glomerulonephritis. A literature review, illustrations with original artwork, and tabulation of clinical and pathologic data of cases obtained from the authors' renal biopsy files examined during the last 8 years were used. The International Society of Nephrology/ Renal Pathology Society-sponsored Classification of Lupus Glomerulonephritis proposes standardized definitions of the various pathologic findings, describes clinically relevant lesions, incorporates prognostic parameters, and recommends a uniform way of reporting the renal biopsy findings. Lupus glomerulonephritis is divided into 6 classes primarily based on the morphologic lesions, extent and severity of the involvement, immune complex deposition, and activity and chronicity. Special emphasis is laid on describing qualitative as well as quantitative morphologic data and to include the accompanying tubulointerstitial disease and different vascular lesions, which have prognostic and therapeutic significance. This classification is intended to facilitate a higher degree of reproducibility, resulting in better patient care and more effective future clinical and translational research. Renal biopsy findings in systemic lupus erythematosus add new and independent parameters of prognostic significance to established clinical and genetic factors.

Recurrent Headache and MRI Findings in Systemic Lupus Erythematosus

Headache in patients with systemic lupus eryhtematosus (SLE) is considered a common neurological finding, although the relationship is unclear. Another obscure point is the relationship between headache and neuroradiologic findings in these patients. In this study, we aimed to evaluate the correlation between headache characteristics and intracranial lesions in SLE patients. Forty-eight SLE patients were chosen from those referred to our clinic depending on the American Collage of Rheumatology (ACR) criteria at the same time or after the diagnosis of SLE. Headache classification was done regarding the ICD-II criteria in the patients. Headache severity was assessed by visual analog scale (VAS), and subjects with VAS > or = 4 were included in the study. Patients were divided into two groups according to magnetic resonance imaging (MRI) findings: abnormal MRI (lesion positive) and normal MRI (lesion negative). On MRI, intracranial lesions were detected in 37.5% (n = 18) of the patients, and no lesion was found in 62.5% (n = 30). Headache characteristics were as tension type in 54.1% (n = 26) and migraine like in 39.6% (n = 19) of all patients. Imaging findings were mostly as periventricular and subcortical focal lesions, ranging from 3-22 mm in diameter. A significant correlation was found between abnormal MRI findings with advanced age and prolonged disease duration (p = 0.018, p = 0.016). As a conclusion, a detailed neurologic evaluation and radiologic investigation, if necessary, should be performed in SLE patients with prolonged disease and advanced age, regardless of headache characteristics.

MR Manifestations of the Brain in Neuropsychiatric Systemic Lupus Erythematosus Patients

Purpose: The primary goal of this study was to evaluate the MR findings of systemic lupus erythematosus (SLE) patients with neuropsychiatric symptoms. Materials and Methods: The MR images of 38 patients with SLE were evaluated based on the presence of the following abnormal lesions: the locations of the abnormal signal intensity lesions in the white matter, infarctions, a small vessel vasculopathy, leukoencephalopathy, hemorrhage, abscess, and other lesions. Results: The MR images showed an abnormality in 22 of 38 (58%) episodes. Abnormal signal intensities were noted in the subcortical and periventricular white matter in six cases, acute territorial infarctions in five cases, multiple small acute embolic infarctions in four cases and a brain abscess in two cases. A reversible posterior leukoencephalopathy was found in one case. In addition, another patient had vasogenic edema with focal central cytotoxic edema at the pons. The entire cerebral and corpus callosum volumes were significantly smaller in four patients with SLE as compared to the volumes in healthy control subjects. Conclusion: SLE may induce variable MR imaging findings of the CNS. Recognition of the variable findings is helpful for easy diagnosis and prompt treatment.

Cardiac tamponade due to systemic lupus erythematosus in patient with Prader-Willi syndrome after growth hormone therapy

We present a 16-year old girl with Prader-Willi syndrome who developed cardiac tamponade as an initial finding of systemic lupus erythematosus. Until one year prior to this episode, she had received growth hormone treatment for nine years. The association among Prader-Willi syndrome, growth hormone treatment and systemic lupus erythematosus is discussed.

Bone marrow histological findings in systemic lupus erythematosus with hematologic abnormalities: A clinicopathological study

The histopathologic features characterizing the involvement of the bone marrow (BM) in systemic lupus erythematosus (SLE) have not been systematically analyzed to date. The aim of this study was to assess morphologic and immunohistochemical characteristics of BM involvement in SLE. Clinical and serological data of 40 SLE patients with unexplained cytopenias were studied. Ten patients with myelodysplasia of refractory anemia (RA) were used as controls. BM aspiration, BM biopsy (BMB), and immunohistochemistry were carried out in patients and controls. BM fibrosis, BM necrosis, stromal edema, and abnormal localization of immature precursors (ALIP) were assessed according to standard criteria. Dyserythropoiesis and megakaryocytic atypias were uniform findings in SLE patients. The disruption of the normal BM architecture was a predominant SLE BM feature affecting cells of all three hemopoietic lineages, with both erythroid and megakaryocytic precursors tending to assume paratrabecular locations and ALIP aggregates being present in 27 cases. In addition, BM was hypocellular in 23 cases. BM necrotic alterations were evident in 90% of the cases. The density of reticulin content was generally increased. Vascular changes including dilatation of sinuses were manifest and were associated with the presence of necrotic alterations (P = 0.008). Hemoglobin levels correlated inversely with the presence of ALIP (P = 0.016). Upon comparing BMB features between SLE and RA controls there were striking similarities. BMB in patients with SLE and unexplained cytopenias presents a variety of histopathologic findings including BM necrosis, stromal alterations, hypocellularity, dyspoiesis, and distortion of normal BM architecture, characterized primarily by the presence of ALIP aggregates.

TNF-related apoptosis-inducing ligand is involved in neutropenia of systemic lupus erythematosus

AbstractNeutropenia is a common laboratory finding in systemic lupus erythematosus (SLE). However, the molecular mechanism of SLE neutropenia has not been fully explained. In this study, we examined whether TNF-related apoptosis-inducing ligand (TRAIL) is involved in the pathogenesis of SLE neutropenia using samples from SLE patients. Serum TRAIL levels in SLE patients with neutropenia were significantly higher than those of SLE patients without neutropenia and healthy volunteers. Serum TRAIL levels showed a significant negative correlation with neutrophil counts in SLE patients. The expression of TRAIL receptor 3 was significantly lower in SLE patients with neutropenia than in patients without neutropenia or in healthy volunteers. Treatment with glucocorticoids negated the decrease of TRAIL receptor 3 expression on neutrophils of SLE patients. TRAIL may accelerate neutrophil apoptosis of neutrophils from SLE patients, and autologous T cells of SLE patients, which express TRAIL on surface, may kill autologous neutrophils. Interferon gamma and glucocorticoid modulated the expression of TRAIL on T cells of SLE patients and also modulated the expression of cellular Fas-associating protein with death domain–like interleukin-1β–converting enzyme (FLICE)–inhibitory protein (cFLIP), an inhibitor of death receptor signaling, in neutrophils. Thus, our results provide a novel insight into the molecular pathogenesis of SLE neutropenia.

Imaging findings in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an unusually complex autoimmune disease that is encountered in every radiology subspecialty because of its multisystem involvement and the wide age range of affected patients. There are no universally accepted diagnostic imaging criteria for SLE, and in fact, many SLE patients present with systemic findings and laboratory abnormalities and do not require imaging. Nevertheless, radiology plays an ancillary role in the diagnosis and management of this often insidious disease, and knowledge of the spectrum of radiologic findings in SLE and its complications is crucial for proper image interpretation. Imaging is often performed in patients with a known diagnosis of SLE to determine the extent and severity of disease, which depend on the extent of organ involvement, and to monitor complications. In addition, imaging may be important in selected patients with diseases such as pneumonia who present with atypical symptoms due to immunosuppressive therapy.

Diagnostic and prognostic significance of anti-C1q antibodies in systemic lupus erythematosus.

The presence of a wide variety of autoantibodies is a characteristic finding in systemic lupus erythematosus. Autoantibodies against nuclear proteins, such as anti-nuclear and anti-double-stranded DNA antibodies, are used as diagnostic markers in systemic lupus erythematosus. Renal involvement is frequently found in systemic lupus erythematosus and is an important risk factor for death. Therefore, markers for the diagnosis and follow-up of nephritis are very important. Anti-C1q autoantibodies are strongly associated with renal involvement in systemic lupus erythematosus. This study will review recent findings on the pathogenic role and clinical importance of anti-C1q antibodies in lupus nephritis. Recent clinical studies have clearly emphasized the diagnostic relevance of anti-C1q autoantibody levels in patients with lupus nephritis. With a possible negative predictive value of 100%, anti-C1q autoantibodies are the only exclusive antibodies associated with the involvement of a single organ in systemic lupus erythematosus. Next to the clinical findings, the pathogenic significance of anti-C1q antibodies has been shown in an animal model. The deposition of autologous C1q in healthy glomeruli of mice after the infusion of anti-C1q antibodies induces moderate tissue damage. The latest insight into the pathogenesis of anti-C1q autoantibodies in the development of lupus nephritis and the recently demonstrated clinical importance of anti-C1q autoantibodies for the diagnosis of lupus nephritis support the value of further investigations. New diagnostic methods for the detection of anti-C1q and an accurate follow-up of antibody levels might be of use in clinical practice.

Ocular Complications in Systemic Lupus Erythematosus: Choroidal and Retinal Pigment Epithelial Changes

Cotton wool patch and retinal hemorrhage are common ocular findings in systemic lupus erythematosus(SLE). Immunosuppressive treatment with corticosteroids may modify choroidal and retinal findings. We examined ocular complications, mainly choroidal and retinal pigment epithelium (RPE) changes in patients with SLE. Fifty SLE cases[3 males, 47 females, 41 +/- 15 (mean +/- standard deviation) year-old] were examined and treated in the departments of Internal Medicine and Ophthalmology of Osaka University Medical Hospital. Complications included cataract in 16 cases, glaucoma or ocular hypertension in 9 cases, and choroidal and retinal changes in 15 cases. Occlusive retinal vasculitis, retinal pigment epithelial dysfunction, such as pigment epithelial detachment, choroidal neovascularization and multiple posterior pigment epitheliopathy, in 6 cases, and branch retinal vein occlusion in 2 cases were detected as fundus complications. Average time period from the first detection of SLE was 13 years in patients with RPE changes. Long term corticosteroid therapy is probably responsible for the RPE complications.

Functional melanonychia due to involvement of the nail matrix in systemic lupus erythematosus

Longitudinal melanonychia is an unusual finding in systemic lupus erythematosus. We report the case of a patient of Arabic extraction with cutaneous lesions of lupus erythematosus on the cheek and chest and longitudinal melanonychia affecting several fingers. Results of histologic examination of the skin and direct immunofluorescence analysis confirmed the diagnosis of systemic lupus erythematosus. The distal matrix revealed changes suggestive of systemic lupus erythematosus accompanied by increased activity of melanocytes but without melanocytic hyperplasia. In this case, longitudinal melanonychia associated with systemic lupus erythematosus was of functional origin. (J Am Acad Dermatol 2002;47:S187-8.)

Cerebral computed tomography and electroencephalography compared with neuropsychological findings in systemic lupus erythematosus.

Central nervous system involvement was evaluated in 36 patients with systemic lupus erythematosus (SLE) using cerebral computed tomography (CT), electroencephalography (EEG), and a neuropsychological test battery. The purpose was to investigate whether brain dysfunction as assessed by comprehensive neuropsychological investigation is associated with findings of routine investigation methods such as CT and EEG which are available in most hospitals. Abnormal EEG was found in 19%, and CT revealed cerebral atrophy in 47% of SLE patients. Few neuropsychological functions were affected by the presence of abnormal EEG, cerebral atrophy, or infarcts. Significant associations were found only between cortical atrophy and impairment of tactile spatial problem-solving and motor dexterity, and between cortical infarcts and motor dexterity in the dominant hand. The value of conventional EEG in assessing cerebral SLE is negligible, except for identifying epileptic activity and focal pathology. Cerebral CT has little relevance in predicting brain dysfunction as established by neuropsychological assessment in SLE, except for detecting cortical atrophy and infarcts.