Systemic Lupus Erythematosus

Author(s):  
Josephina A. Vossen

Chapter 40 discusses systemic lupus erythematosus (SLE), which is an autoimmune connective tissue disorder with a wide range of clinical features. SLE follows a relapsing and remitting course and predominantly affects women. The clinical manifestations are highly variable, ranging from indolent to fulminant. Arthritis is one of the most common manifestations of SLE. Diagnosis is based on clinical assessment supported by laboratory testing. Imaging of the musculoskeletal system is used primarily for the assessment of symptomatic joints. The initial imaging evaluation of SLE arthropathy is with radiographs of symptomatic joints, usually the hands. Treatment ranges from corticosteroids to immunosuppressive agents.

2021 ◽  
Vol 7 (3) ◽  
pp. 28-34
Author(s):  
Yu. Minakova ◽  
M. Silenko ◽  
O. Ivanova

Damage to the nervous system (neurolupus) is one of the most common clinical manifestations of systemic lupus erythematosus (SLE) in childhood, and is also considered as an unfavorable prognostic criterion for the course of this disease. Neurolupus is characterized by a wide range of clinical manifestations in both children and adult patients, which is due in most cases to a common pathogenetic mechanism - the formation of systemic microvasculitis. The non-specificity and variability of neuropsychiatric symptoms, which may appear already at the onset of the disease, significantly complicate the early diagnosis of SLE and necessitate a close acquaintance of the pediatrician with neurolupus polymorphism in children.


2008 ◽  
Vol 22 (1) ◽  
pp. 29-52 ◽  
Author(s):  
Catherine A. Heyneman

Systemic lupus erythematosus (SLE) is a complex autoimmune disease with variable clinical manifestations that is characterized by flares and periods of relative quiescence. The disease occurs approximately 10 times more frequently in women and is more prevalent among certain ethnic groups. The etiology is complex and dependent upon an interaction of genetic, hormonal, and environmental factors. Corticosteroids and immunosuppressive agents have transformed the outlook for patients with lupus. Unfortunately, the increased lifespan unmasked an accelerated process of atherosclerosis and cardiovascular disease. Early mortality is usually attributable to active lupus, but deaths late in the disease process are often secondary to thrombotic events. Advancements in the understanding of molecular and cellular mechanisms involved in the pathogenesis have resulted in development of novel therapies. Immunomodulatory drugs developed for other diseases are being investigated for use in specific manifestations of lupus. Individualization of treatment and lifelong monitoring are required in most patients.


2021 ◽  
Vol 2 (1) ◽  
pp. 22-27
Author(s):  
Margarita A. Gromova ◽  

Background. Due to multi-organ lesions and a wide range of clinical manifestations, human immunodeficiency virus (HIV infection) resembles systemic connective tissue diseases, particularly systemic lupus erythematosus (SLE). Similarity of immune disorders in patients with HIV infection and SLE may lead to misdiagnosis resulting in wrong tactics of treatment (prescribing immunosuppressive therapy instead of high-dose antiretroviral therapy). Aim. To report the HIV infection and SLE differential diagnosis challenges. Results. By January 2021, the analysis of national literature revealed two cases of HIV infection in young women, initially misdiagnosed with suspected SLE. In addition to two patients described in literature, one more female patient with SLE manifestations combined with advanced stage of HIV infection was examined. Conclusion. HIV and SLE differential diagnosis is the ultimate challenge for clinicians. Physicians should be especially apprehensive about HIV infection when making the diagnosis of SLE. SLE therapy in patients with HIV infection should be appointed according to strict indications.


Kardiologiia ◽  
2019 ◽  
Vol 59 (12) ◽  
pp. 92-96
Author(s):  
N. A. Kosheleva ◽  
N. M. Nikitina ◽  
E. U. Andreeva

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology characterized by a wide range of clinical manifestations with damage to various organs and systems of the body. There are bad prognostic factors for SLE: damage to the heart, kidney, central nervous system, the development of hematological crises and secondary antiphospholipid syndrome. A number of authors consider systemic lupus erythematosus a “new” risk factor for atherosclerosis. The overall risk of myocardial infarction (MI) in patients with SLE is 10 times higher than in the general population. The article presents clinical case report of the development of myocardial infarction in a woman with SLE, receiving therapy for secondary antiphospholipid syndrome.


2019 ◽  
Vol 16 (6) ◽  
pp. 29-35
Author(s):  
Alina Dima ◽  
Bianca Dumitrescu ◽  
Daniela Nicoleta Popescu ◽  
Magda Pârvu

AbstractSystemic lupus erythematosus (SLE) is considered the prototype of autoimmune diseases, the most complex autoimmune pathology and it is characterized by a wide range of immune processes, important antibodies production as well as an impressive spectrum of clinical manifestations. The great variety of lupus signs and symptoms caused difficulties in establishing well-defined classification criteria, as well as sustaining the clinical diagnosis.In 2019, a joint initiative of European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) released a new set of classification criteria for SLE, worldwide SLE experts were involved, this being the largest SLE classification effort up to date.


Lupus ◽  
2016 ◽  
Vol 26 (7) ◽  
pp. 746-755 ◽  
Author(s):  
N Glesse ◽  
P Vianna ◽  
L M G Paim ◽  
M C C Matte ◽  
A K K Aguiar ◽  
...  

Background Systemic lupus erythematosus (SLE) is an autoimmune disease marked by the disruption of the immune homeostasis. Patients exhibit a wide range of clinical manifestations, and environmental and genetic factors are involved in SLE pathogenesis. Evidence suggests that abnormalities in the cellular and molecular events that coordinate apoptosis may favour the generation of autoantigens involved in autoimmunity. In this way, the apoptotic deregulation may be affected by polymorphic variants in apoptotic-related genes. Methods We analyzed FAS, FASL, BCL-2 and BAX polymorphisms in order to correlate to SLE susceptibility and clinical features. A total of 427 SLE patients from the Hospital de Clínicas de Porto Alegre and 543 controls from southern Brazil were evaluated. Results We observed higher frequencies of the FASL –844CC genotype and –844C allele, as well as of the FASL-844C/IVS2nt-124A haplotype in African-derived SLE patients when compared to controls ( P < 0.001). FASL –844C, which is related to high FasL expression, could contribute to increased apoptosis and to the breakdown of immunological tolerance, favouring autoantibody production and inflammation. On the other hand, the BAX –248GA genotype and the –248A allele , related to low protein expression, were observed as a protective factor against SLE in this same population. The rate of apoptosis and cell death was evaluated in peripheral lymphocytes, and SLE patients presented a higher percentage of dead lymphocytes (CD3+Annexin V+ 7-AAD+) compared to the control group. Conclusion Our data support a role for apoptosis in SLE susceptibility.


2020 ◽  
Vol 14 (4) ◽  
pp. 23-30
Author(s):  
E. A. Aseeva ◽  
S. K. Solovyev ◽  
N. Yu. Nikishina ◽  
G. M. Koilubaeva ◽  
T. A. Lisitsyna ◽  
...  

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations. Numerous observations and surveys of patients have shown that the most common symptom of SLE is fatigue complaints in 51 to 90% of patients.Objective: to determine the significance of fatigue in the general health status of RENAISSANCE cohort patients with SLE who were hospitalized in the Clinic, V.A. Nasonova Research Institute of Rheumatology.Patients and methods. The investigation included SLE patients aged 18 years and older who met the 2012 SLICC criteria. The standard examination accepted in the management of patients with SLE was made. Disease activity was determined by SLEDAI-2K; irreversible lesions in various organs were identified using the SLICC damage index. The SF-36 and the LupusQoL questionnaires were used to assess health-related quality of life (HRQOL) and the FACIT-Fatigue scale was applied to measure fatigue.Results and discussion. The investigation enrolled 328 patients, mainly women (91%); the mean age was 34.4±11.5 years; the duration of the disease was 106.3±97.9 months. In this group, moderate and high disease activities (SLEDAI-2K scores of 6–10 and 11–19, respectively) were observed at approximately the same frequency. At the time of inclusion, more than half (56.5%) of the patients already had various irreversible organ lesions. At Visit 1, the FACIT-Fatigue scale showed that fatigue was present in 148 (45%) of the 328 patients. According to the presence of fatigue, the patients were divided into two groups. Group 1 included 148 patients with fatigue; Group 2 consisted of 180 patients without fatigue. The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and anti-DNA antibody levels were significantly higher in the fatigue group (p=0.01 and p=0.02, respectively); the patients also had decreased HRQOL according to 7 LupusQol domains (p<0.001). The patients with fatigue were significantly more likely to receive intravenous glucocorticoids and rituximab. At 12 months after the start of treatment, the patients with fatigue were found to have a statistically significant reduction in disease activity, as well as normalization of anti-DNA antibody levels, improvements in HRQOL according to the LupusQol domains, and less severity of fatigue according to the FACIT-Fatigue scale.Conclusion. Fatigue was detected in almost half (45–53%) of SLE patients. It is associated with a higher disease activity by SLEDAI-2K and with a high anti-DNA antibody level. The patients with fatigue are observed to have an obvious worsening of HRQOL according to all LupusQol domains.


2020 ◽  
Vol 8 (11) ◽  
pp. 791-798
Author(s):  
Khalid Abdullah Alghamdi ◽  
◽  
Ahmed Saeed Almaqati ◽  
Nuha Adnan Meraiani ◽  
Nawal Bassuni ◽  
...  

We report a case of 40-year-old female who is known to have systemic lupus erythematosus (SLE) presenting with severe cognitive impairment with lymphopenia, proteinuria, and evidence of SLE serological activity. Initially, she was managed as a case of neuropsychiatric systemic lupus erythematosus (NPSLE) with Cyclophosphamide and pulse steroids. However, she has been deteriorating clinically in forms of right sided hemiparesis, blindness, and aphasia despite normalization of complements and anti-double stranded DNA antibodies levels. Diagnosis of progressive multifocal leukoencephalopathy (PML) was made based on her clinical manifestations with brain MRI findings of subcortical white matter lesions and detection of John Cunningham virus (JCV) in cerebrospinal fluid analysis. Immunosuppressive agents were discontinued aiming for immune system restoration.


2010 ◽  
Vol 2010 ◽  
pp. 1-6 ◽  
Author(s):  
Massimiliano Sarra ◽  
Giovanni Monteleone

Systemic Lupus Erythematosus (SLE) is an autoimmune disorder characterized by excessive production of a variety of autoantibodies and a wide range of clinical manifestations. Pathogenesis of SLE is complex and not fully understood. There is however evidence that B and T cells are critical to the development of disease, and that T cell-derived cytokines are involved in the SLE-associated inflammatory response. One such cytokine seems to be interleukin (IL)-21, the latest identified member of the -chain-related cytokine family. IL-21 has an important role in the control of the growth, survival, differentiation, and function of both T and B cells, and excessive production of IL-21 has been associated with the development of multiple immune-mediated diseases. Here we review data supporting the involvement of IL-21 in the pathogenesis of SLE.


Author(s):  
Arya B. Mohabbat ◽  
Christopher M. Wittich

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown cause that affects multiple organ systems. Disease susceptibility is conferred by an interaction between genetics, aberrant immunologic mechanisms, hormonal influences, and environmental factors. SLE has a wide range of heterogeneous clinical manifestations and is characterized by disease flares and remissions. There is a broad spectrum of disease severity, leading to significant morbidity and increased mortality.


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