Systemic lupus erythematosus and related disorders

2010 ◽  
pp. 3652-3664 ◽  
Author(s):  
Anisur Rahman ◽  
David A. Isenberg
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
T Lymphocytes ◽  
Complement System ◽  
Lupus Erythematosus ◽  
The Body ◽  
Rheumatic Disorder ◽  
Systemic Lupus ◽  
The Complement System

Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disorder that can present with symptoms in almost any organ or system of the body. It is 10 to 20 times commoner in women than men, and commoner in Afro-Caribbeans than Asians than whites. Aetiology is multifactorial, incorporating genetic, hormonal, and environmental elements. No single abnormality of the immune system can be considered responsible, pathogenesis depending on the interplay of a number of different factors, including autoantibodies, T lymphocytes, cytokines, the complement system, and apoptosis....

Systemic lupus erythematosus and related disorders

2020 ◽  
pp. 4499-4513
Author(s):  
Anisur Rahman ◽  
David A. Isenberg
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Lupus Erythematosus ◽  
Severe Disease ◽  
Skin Lesions ◽  
The Body ◽  
Rheumatic Disorder ◽  
Systemic Lupus ◽  
Mild Disease ◽  
The Complement System

Systemic lupus erythematosus is an autoimmune rheumatic disorder that can present with symptoms in almost any organ or system of the body. It is 10 times commoner in women than men, and commoner in Afro-Caribbeans than in other ethnic groups. Its aetiology is multifactorial, incorporating genetic, hormonal, and environmental elements. No single abnormality of the immune system can be considered responsible, pathogenesis depending on the interplay of several different factors, including autoantibodies, T lymphocytes, cytokines, the complement system, and apoptosis. Common symptoms are constitutional (fatigue, anorexia), musculoskeletal (arthralgia/arthritis, myalgia), dermatological (alopecia, butterfly rash, vasculitic skin lesions, purpura), cardiopulmonary (breathlessness, pleurisy), and neurological (migraine, seizures, depression, psychosis). Treatment for mild disease is NSAID, analgesics and hydroxychloroquine, more severe disease requires corticosteroid and immunosuppressant drugs.

scholarly journals Systemic Lupus Erythematosus Disease: An Overview of the Clinical Approach to Pathogenesis, Diagnosis, and Treatment

2021 ◽  
Vol 4 (2) ◽  
pp. 91-98
Author(s):  
Saurabh Nimesh ◽  
Md. Iftekhar Ahmad ◽  
Shikhka Dhama ◽  
Pradeep Kumar ◽  
Muhammad Akram ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Chronic Disease ◽  
Lupus Erythematosus ◽  
The Body ◽  
Clinical Approach ◽  
Systemic Lupus Erythematosus Disease ◽  
Systemic Lupus ◽  
Organ Systems ◽  
Novel Approaches

The systemic lupus erythematosus (SLE), commonly known as Lupus, is a rare and complex multisystem autoimmune disease where one’s immune system is overactive, and the body attacks its organ systems. SLE is a historically old disease described already in antiquity; it is an example of a chronic disease with physical, psychological, financial, and social implications for individuals diagnosed. It has inspired medical and basic biological scientists that focus on molecular biology, basic immunology, immunopathology, clinical science, genetics, and epidemiology. The syndrome is real in its existence-although hidden behind obstacles, cumbersome for patients and clinicians, and rebellious for scientists. There is currently no cure for SLE. The goal of treatment is to ease symptoms. This article will review information on the general approach to SLE therapy, focusing on currently approved therapies and novel approaches that might be used in the future.

scholarly journals Platelet Activation and Anti-Phospholipid Antibodies Collaborate in the Activation of the Complement System on Platelets in Systemic Lupus Erythematosus

PLoS ONE ◽  
2014 ◽  
Vol 9 (6) ◽  
pp. e99386 ◽  
Author(s):  
Christian Lood ◽  
Helena Tydén ◽  
Birgitta Gullstrand ◽  
Gunnar Sturfelt ◽  
Andreas Jönsen ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Platelet Activation ◽  
Complement System ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
The Complement System

Targeting the complement system in systemic lupus erythematosus and other diseases

2013 ◽  
Vol 148 (3) ◽  
pp. 313-321 ◽  
Author(s):  
Maria-Louise Barilla-LaBarca ◽  
Kiley Toder ◽  
Richard Furie
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Complement System ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
The Complement System

C3 glomerulonephritis and systemic lupus erythematosus: A report of a patient treated with eculizumab and review of the literature

Lupus ◽  
2021 ◽  
pp. 096120332110279
Author(s):  
Ruth Fernandez-Ruiz ◽  
Rebecca B Blank ◽  
Ming Wu ◽  
H Michael Belmont
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Complement System ◽  
Lupus Erythematosus ◽  
Alternative Pathway ◽  
Renal Diseases ◽  
C3 Glomerulonephritis ◽  
Complement Pathway ◽  
Systemic Lupus ◽  
The Complement System

Introduction Activation of the complement pathway by immune complexes is a key feature of systemic lupus erythematosus (SLE) and SLE glomerulonephritis, which translates into low levels of C3 and C4 during active disease. C3 glomerulonephritis (C3GN) is part of a broader group of rare renal diseases, the C3 glomerulopathies, characterized by prominent C3 accumulation in the glomeruli with minimal to no immunoglobulin (Ig) deposition secondary to dysregulation of the alternative pathway of the complement system. Distinguishing lupus nephritis from other complement-mediated kidney disorders, including C3GN, represents a diagnostic challenge with potential therapeutic implications. Methods We report an unusual case of a 55-year-old woman with SLE and previous biopsy-proven class IV lupus nephritis, subsequently diagnosed with C3GN. Furthermore, we review the available literature published from January 2010—March 2021 on the clinical features and management of C3GN in the setting of SLE. Results In addition to our case, very few reports exist in the literature regarding C3GN in association with SLE. The underlying pathogenic mechanism of C3GN consists of dysregulation of the alternative pathway of the complement system, either due to genetic variation in complement-related genes or to acquired autoantibodies targeting C3 or C5 convertases; the latter mechanism could explain the occurrence of C3GN in the setting of autoimmune diseases, although it was not definitively identified in our patient or others with SLE. Similar to some of the previous reports, after suboptimal renal response on mycophenolate mofetil and rituximab, our patient has been successfully treated with eculizumab, thus far with >50% improvement in proteinuria. Conclusions C3GN represents an additional mechanism of renal injury in SLE mediated by alternative complement pathway dysregulation. Although rare, patients with SLE and persistent proteinuria with very low C3 would benefit from expedited renal biopsy to evaluate for C3GN as well as genetic testing, since this entity could require a different therapeutic approach.

scholarly journals Autoimmune Polyglandular Syndrome Type 3-D

2022 ◽  
Author(s):  
Fadel Fikri Suharto ◽  
RM Dewi Anggraini ◽  
Ardianto Tamin ◽  
Della Fitricana ◽  
Nova Kurniati ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Disease Patient ◽  
The Body ◽  
Graves Disease ◽  
Systemic Lupus ◽  
Metabolic System ◽  
Organ Systems

Background: Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease characterized by the presence of autoantibodies against cell nuclei and involves many organ systems in the body. The etiopathology of SLE is thought to involve complex and multifactorial interactions between genetic variation and environmental factors. Hyperthyroidism is a disease due to increased thyroid hormone function followed by signs and symptoms that affect the body's metabolic system. Graves' disease is an autoimmune disease characterized by the presence of antibodies to TSHR (TRAb). Several coexisting autoimmune diseases have been classified under different syndromes. Case Presentation: A woman, 29 years-old, came to office with complaint of chest palpitation. Patient had history of fever, joint pain, hair loss, and malar rash. Patient had been diagnosed with hyperthyroidism for 4 years and regularly taking propylthiouracil 100 mg and propranolol 10 mg. Titer ANA Test 1/100, Anti ds-DNA 68.08, C3-Complement 93 (N: 83-193), C4-Complement 11.2 (N: 15-57), Free T3 7.79 (N: 1.71-3.71), Free -T4 2.50 (N: 0.70-1.48), TSHs 0.0001 (N: 0.350-4.94), TRAb 3.38 (N: < 1.75). Patient was diagnosed with systemic lupus erythematosus (SLE) and graves’ disease. Patient treated with methimazole 10 mg, propranolol 10 mg, myfortic 360 mg, and methylprednisolone 4 mg. Conclusion: Autoimmune Polyendocrine Syndromes (APS) was at first characterized as different endocrine organ diseases related to an immune system disease in a subject. Hence, affiliation between illnesses in APS was noted not to be irregular but in specific combinations in which a few non-endocrine immune system diseases were moreover portion of the disorders.

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