Systemic Lupus Erythematosus and Critical Illness

2020 ◽  
Vol 31 (3) ◽  
pp. 296-307
Author(s):  
Erin K. Gasser ◽  
Hildy M. Schell-Chaple

Systemic lupus erythematosus is a chronic autoimmune disorder that causes a wide range of mild to life-threatening conditions that require hospitalization and critical care. The morbidity and mortality of systemic lupus erythematosus are associated with the organ system damage caused by intermittent or chronic disease activity and with the complications of long-term and toxic immunosuppressant medication regimens. This article reviews the epidemiologic, clinical, diagnostic, and therapeutic information essential for critical care clinicians who provide care to patients with systemic lupus erythematosus.

Cells ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 140 ◽  
Author(s):  
Yasuo Nagafuchi ◽  
Hirofumi Shoda ◽  
Keishi Fujio

Systemic lupus erythematosus (SLE) is an autoimmune disorder with a wide range of clinical symptoms. Enormous progress has been made in the immunological and genetic understanding of SLE. However, the biology of disease heterogeneity in SLE has remained largely unexplored. Human immune profiling studies, helped by recent technological advances especially in single-cell and “omics” analyses, are now shedding light on the cellular and molecular basis of clinical symptoms and disease flares in individual patients. Peripheral blood immunophenotyping analysis with flow cytometry or mass cytometry are identifying responsible cell subsets and markers characteristic of disease heterogeneity. Transcriptome analysis is discovering molecular networks responsible for disease activity, disease subtype and future relapse. In this review, we summarize recent advances in the immune profiling analysis of SLE patients and discuss how they will be used for future precision medicine.


2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Mariana Postal ◽  
Lilian TL Costallat ◽  
Simone Appenzeller

Systemic lupus erythematosus (SLE) is a prototypic inflammatory autoimmune disorder characterized by multisystem involvement and fluctuating disease activity. Symptoms range from rather mild manifestations such as rash or arthritis to life-threatening end-organ manifestations. Despite new and improved therapy having positively impacted the prognosis of SLE, a subgroup of patients do not respond to conventional therapy. Moreover, the risk of fatal outcomes and the damaging side effects of immunosuppressive therapies in SLE call for an improvement in the current therapeutic management. New therapeutic approaches are focused on B-cell targets, T-cell downregulation and costimulatory blockade, cytokine inhibition, and the modulation of complement. Several biological agents have been developed, but this encouraging news is associated with several disappointments in trials and provide a timely moment to reflect on biologic therapy in SLE.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Atsushi Sakamoto ◽  
Masao Ogura ◽  
Atsushi Hattori ◽  
Kinji Tada ◽  
Reiko Horikawa ◽  
...  

Abstract Background Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is characterized by bleeding and thrombosis in patients with autoimmune diseases or infections. Paediatric LAHPS exhibits various degrees of bleeding, ranging from mild to severe; however, adrenal haemorrhage due to LAHPS and its long-term clinical course have not been sufficiently described. Case presentation A 9-year-old boy presented with prolonged abdominal pain and abnormal coagulation screening tests. The laboratory tests showed prolonged activated partial thromboplastin time and subsequently revealed the presence of lupus anticoagulant, anti-nuclear antibodies, and hypoprothrombinemia, leading to diagnosis of LAHPS. An enhanced computed tomogram demonstrated nodular lesions in the adrenal glands bilaterally, suggestive of adrenal haemorrhage. Laboratory and clinical manifestations exhibited life-threatening adrenal insufficiency that required hydrocortisone administration. The patient developed systemic lupus erythematosus, diagnosed 12 months later. Conclusions This patient with LAHPS developed rare adrenal failure due to adrenal haemorrhage, a life-threatening event that should be recognized and treated early. In our case, renal dysfunction was also observed when systemic lupus erythematosus was diagnosed 1 year after LAHPS. Our case emphasizes that early recognition of adrenal failure and careful long-term observation is required in patients with autoantibodies.


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):  
Dandyala Pavan Kalyan ◽  
Gajula Sri Teja ◽  
Kallem Sharat Venkat Reddy ◽  
Marina D’souza

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder, which affects the major organs in the human body. Pathophysiology of SLE is unknown. It mainly affects the joints, and restricts their movement. Hydroxychloroquine (HCQ) an anti-malarial drug is used as the first line of drugs used to treat SLE. The major adverse effect of this drug is irreversible retinopathy. The aim of the study was to evaluate the incidence and prevalence of retinopathy in patients with long-term usage of hydroxychloroquine (for more than 1 year).Methods: In patients with SLE, we recorded a review on HCQ induced toxicity among those taking it for longer period (>1 year). All the patients were above 18 years of age. A total data of 210 patients suffering from SLE and taking HCQ for more than one year was collected. Patients were categorized according to gender and dose pattern. Out of 210 patients, 0 patients were found to be retinal toxic induced by HCQ.Results: Suitable statistical tools were used and data was analysed which showed the incidence and prevalence of HCQ induced toxicity. With the results of our study we can understand that incidence and prevalence rates were very low among the subjects.Conclusions: HCQ is said to reduce the risk of disease remission, improves survival, minimizes the risks of vital organ damage, reduces the frequency of flares and has a protective effect on cardiovascular health.  HCQ medication is usually well tolerated.  But irreversible retinopathy is the major effect on long term use of HCQ.  The present study concludes that in the nominal daily dose of 200mg did not reveal any signs of retinal toxicity in 100% of the population tested within 5 years of HCQ treatment suggesting that the toxicity is rare and can be prevented by reducing the dose of the drug.


2022 ◽  
pp. 529-531
Author(s):  
F Altous ◽  
Rupak Protim Patir ◽  
Debashish Kharigapsha ◽  
Bimal Karmakar ◽  
Mozadeed Alom

Systemic lupus erythematosus (SLE) is one of the numerous diseases called “the great imitators” because it frequently resembles or is misdiagnosed as other illnesses. SLE is a chronic autoimmune inflammatory disorder that affects multiple systems and has a wide range of symptoms. The female to male ratio varies between 7 and 15 to 1. A number of observations point to an estrogen impact as the cause of this variance. Because sex hormonal impacts are likely to be modest in youngsters, the female to male ratio is 3:1. Renal involvement and seizures are more common in male SLE patients than photophobia and cutaneous symptoms. Males likewise appear to have a more devastating consequence. As a result, we feel that male lupus patients are uncommon, the illness’s symptoms are life-threatening, and that early discovery of the disease will result in a better outcome for these patients. Here, we report the case of SLE with an unusual presentation in a 44-year-old man from lower Assam to emphasize the importance of a high index of suspicion in such patients.


2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Sriram Krishnamurthy ◽  
Subramanian Mahadevan

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder associated with multiple immunological abnormalities and a wide range of clinical manifestations. Recent progress in genetics has expanded the number of the genes associated with SLE to more than 20 in number and has contributed to improvement of understanding of the pathogenesis of the disease. This has enhanced the development of novel therapeutic targets and biomarkers for individualized and tailor-made clinical management of lupus patients. Despite this knowledge, however, it is a challenge to fully understand the genetic pathogenesis of the disease. The present paper describes the current concepts in the mechanisms, genomics, and pathogenesis of SLE and their implications for management of the disorder. The potential role of gene therapy, biological agents, intravenous immunoglobulin, anti-inflammatory cytokines, and cytokine inhibitors is discussed.


1975 ◽  
Vol 37 (6) ◽  
pp. 924-929 ◽  
Author(s):  
Hideaki YAMAURA ◽  
Masaharu RIKIMARU ◽  
Isamu TAKAHASHI ◽  
Sadao ANAN ◽  
Tomio AKIYAMA ◽  
...  

Lupus ◽  
2021 ◽  
pp. 096120332110047
Author(s):  
Muming Yu ◽  
Yulei Gao ◽  
Heng Jin ◽  
Songtao Shou

Acute pericardial tamponade, which can cause obstructive shock, is a serious life-threatening medical emergency that can be readily reversed by timely identification and appropriate intervention. Acute pericardial tamponade can occur for a number of reasons, including idiopathic, malignancy, uremia, iatrogenic, post-myocardial infarction, infection, collagen vascular, hypothyroidism, and others. Systemic lupus erythematosus (SLE) and hyperthyroidism associated with pericardial tamponade are rarely reported. Here, we report the case of a 20-year-old female patient was final diagnosed of SLE with Graves’ hyperthyroidism.


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