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.

scholarly journals Polyglandular Autoimmune Syndrome Type 3D : A Case Report

2022 ◽  
Author(s):  
Fadel Fikri
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Disease Patient ◽  
The Body ◽  
Systemic Lupus ◽  
Metabolic System ◽  
Polyglandular Autoimmune Syndrome ◽  
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. Autoimmune Polyglandular Syndrome (PGAS),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: &lt; 1.75). Patient was diagnosed with systemic lupus erythematosus (SLE) and grave's 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.

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 Cytokines as Biomarkers in Systemic Lupus Erythematosus: Value for Diagnosis and Drug Therapy

2021 ◽  
Vol 22 (21) ◽  
pp. 11327
Author(s):  
Helena Idborg ◽  
Vilija Oke
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Lupus Erythematosus ◽  
Autoimmune Response ◽  
Pathophysiological Mechanism ◽  
Cell Nuclei ◽  
Systemic Lupus ◽  
Necrosis Factor Alpha ◽  
Organ Systems ◽  
Extracellular Structures

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease. The disease is characterized by activation and dysregulation of both the innate and the adaptive immune systems. The autoimmune response targets self-molecules including cell nuclei, double stranded DNA and other intra and extracellular structures. Multiple susceptibility genes within the immune system have been identified, as well as disturbances in different immune pathways. SLE may affect different organs and organ systems, and organ involvement is diverse among individuals. A universal understanding of pathophysiological mechanism of the disease, as well as directed therapies, are still missing. Cytokines are immunomodulating molecules produced by cells of the immune system. Interferons (IFNs) are a broad group of cytokines, primarily produced by the innate immune system. The IFN system has been observed to be dysregulated in SLE, and therefore IFNs have been extensively studied with a hope to understand the disease mechanisms and identify novel targeted therapies. In several autoimmune diseases identification and subsequent blockade of specific cytokines has led to successful therapies, for example tumor necrosis factor-alpha (TNF-α) inhibition in rheumatoid arthritis. Authors of this review have sought corresponding developments in SLE. In the current review, we cover the actual knowledge on IFNs and other studied cytokines as biomarkers and treatment targets in SLE.

scholarly journals Graves’ Disease and Evans’ Syndrome as First Manifestation of Systemic Lupus Erithematosus

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A930-A930
Author(s):  
Andrés Alberto Gómez-Noronha ◽  
Eddy López-Huamanrayme ◽  
Carmen Cecilia Quiroa-Alfaro
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Heart Rate ◽  
Autoimmune Disease ◽  
Thyroid Hormones ◽  
Lupus Erythematosus ◽  
Thyroid Disease ◽  
Hellp Syndrome ◽  
Graves Disease ◽  
Evans Syndrome ◽  
Systemic Lupus

Abstract Background: Graves’ disease is the most common cause of hyperthyroidism triggered by antibodies called thyroid-stimulating immunoglobulin (TSI) which stimulates an overproduction of thyroid hormones. Evans’ syndrome is a rare condition characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura. Systemic lupus erythematosus (SLE) is also an autoimmune disease with extreme heterogeneity and potentially involvement of any organ or system. It is well known when a patient is diagnosed with an autoimmune disease, it is about time to show up other manifestations of another one, just as it happened in this case report. Clinical Case: A 31-year-old pregnant woman (22 weeks) was admitted to the obstetric emergency room due to headaches, weakness and tinnitus. During anamnesis, she said she was diagnosed with hypertension several weeks before she was pregnant. At physical examination, a 160/100 mm/Hg blood pressure and a heart rate over 100 bpm were found. Initial tests were solicited congruent with severe thrombocytopenia (20 000/mm3) and severe anemia (6 gr/dl), there was also a modest increase in transaminases levels. Transfusion support was needed and a “HELLP syndrome” was diagnosed. Gynecologists decided to perform an emergency hysterotomy and the end of pregnancy. During the post-operative care and the following days, the patient persisted with an average of 100 bpm heart rate and hypertension despite of the use of antihypertensive medication. Physicians also noticed the presence of malar rash and goiter. Thyroid hormones levels where requested and the results were consistent with primary hyperthyroidism (TSH: &lt;0.005 Mu/L, FT4: &gt;100 pmol/L). Further tests were required such as TSI (positive), a thyroid scintigraphy (high thyroid uptake), antinuclear antibodies (ANA: + 1/160 speckled pattern, anti- Smith: +) and extractable nuclear antigen antibodies (ENA) panel. Grave’s disease and SLE were diagnosed. Rheumatologists suggested that the diagnosis of HELLP Syndrome was unclear and they strongly believed that thrombocytopenia and anemia during pregnancy were part of Evans’s syndrome and at the same time of SLE. Antithyroid drugs (thiamazol), beta blockers (propranolol) hydroxychloroquine and corticoids (prednisone) were given to the patient with an excellent clinical and biochemical response. Conclusion: A 25% of patients with SLE can be diagnosed with an autoimmune thyroid disease, such as Graves’ disease (1). Frequent evaluation of thyroid hormones and antithyroid antibodies should be performed in patients with SLE, especially when there are related symptoms of a thyroid disorder. References: 1.Chan AT, Al-Saffar Z, Bucknall RC. Thyroid disease in systemic lupus erythematosus and rheumatoid arthritis. Rheumatology (Oxford). 2001;40:353---4.

scholarly journals Gut Microbiota in Human Systemic Lupus Erythematosus and a Mouse Model of Lupus

2017 ◽  
Vol 84 (4) ◽  
Author(s):  
Xin M. Luo ◽  
Michael R. Edwards ◽  
Qinghui Mu ◽  
Yang Yu ◽  
Miranda D. Vieson ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Disease ◽  
Gut Microbiota ◽  
Lupus Erythematosus ◽  
Bacterial Species ◽  
Human Study ◽  
The Body ◽  
Systemic Lupus ◽  
Content Type ◽  
Active Disease

ABSTRACT Gut microbiota dysbiosis has been observed in a number of autoimmune diseases. However, the role of the gut microbiota in systemic lupus erythematosus (SLE), a prototypical autoimmune disease characterized by persistent inflammation in multiple organs of the body, remains elusive. Here we report the dynamics of the gut microbiota in a murine lupus model, NZB/W F1, as well as intestinal dysbiosis in a small group of SLE patients with active disease. The composition of the gut microbiota changed markedly before and after the onset of lupus disease in NZB/W F1 mice, with greater diversity and increased representation of several bacterial species as lupus progressed from the predisease stage to the diseased stage. However, we did not control for age and the cage effect. Using dexamethasone as an intervention to treat SLE-like signs, we also found that a greater abundance of a group of lactobacilli (for which a species assignment could not be made) in the gut microbiota might be correlated with more severe disease in NZB/W F1 mice. Results of the human study suggest that, compared to control subjects without immune-mediated diseases, SLE patients with active lupus disease possessed an altered gut microbiota that differed in several particular bacterial species (within the genera Odoribacter and Blautia and an unnamed genus in the family Rikenellaceae ) and was less diverse, with increased representation of Gram-negative bacteria. The Firmicutes / Bacteroidetes ratios did not differ between the SLE microbiota and the non-SLE microbiota in our human cohort. IMPORTANCE SLE is a complex autoimmune disease with no known cure. Dysbiosis of the gut microbiota has been reported for both mice and humans with SLE. In this emerging field, however, more studies are required to delineate the roles of the gut microbiota in different lupus-prone mouse models and people with diverse manifestations of SLE. Here, we report changes in the gut microbiota in NZB/W F1 lupus-prone mice and a group of SLE patients with active disease.

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.

Systemic Lupus Erythematosus

2016 ◽  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Symptomatic Treatment ◽  
The Body ◽  
Systemic Autoimmune Disease ◽  
Systemic Lupus ◽  
Specific Section ◽  
Onset Systemic Lupus Erythematosus ◽  
Management Issues

Systemic lupus erythematosus (SLE) is a systemic, autoimmune disease that can affect any part of the body, causing the immune system to attack the body’s cells and tissue, and resulting in inflammation and tissue damage. It is characterized by the presence of autoreactive B and T cells and the production of a broad, heterogeneous group of autoantibodies (autoAb); the absence of a unique presentation makes its diagnosis difficult, even for qualified clinicians. Systemic Lupus Erythematosus focuses on providing a practical approach to the assessment and management of patients with this complex, multisystem, autoimmune disease, in order to improve the diagnosis and treatment of the disease and its complications. It provides detail on the history and epidemiology of SLE alongside comprehensive sections on clinical features, treatment, and special situations. As well as detailing the challenging management issues of SLE, this title provides an overview of the numerous investigations specific to the condition, assessment of disease activity, symptomatic treatment, patient education, and biologic therapies. A specific section on juvenile-onset systemic lupus erythematosus also provides the practising clinician with the knowledge needed to manage this distinct and aggressive stage of SLE.

scholarly journals An Early Lupus Pleuritis With Large Pleural Effusion Due to Systemic Lupus Erythematosus in a 17-year-old Girl

2020 ◽  
Author(s):  
Siavash Abedi ◽  
Ali Sharifpour ◽  
Masoud Aliyali ◽  
Fatemeh Niksolat ◽  
Seyyed Abbas Hashemi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immune System ◽  
Pleural Effusion ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Initial Symptom ◽  
Systemic Lupus

Systemic Lupus Erythematosus (SLE) known as “lupus” is an autoimmune disease that mostly affects women. In this disorder, the body’s immune system attacks different tissues.Manifestations and symptoms vary among individuals from mild to severe. One of the most common manifestations of lupus is pleuritis with pleural effusion; however, its manifestationas the initial symptom of the disease is remarkably rare. Herein, we present a 17-year-old female with SLE, which was diagnosed with early pleural effusion regarding rheumatologiccounseling subjected to treatment

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....

scholarly journals Hepatitis Lupus in Systemic Lupus Erythematosus in Male Patients

2021 ◽  
Vol 7 (2) ◽  
pp. 428-434
Author(s):  
Andikha Putra ◽  
Raveinal
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Abdominal Ultrasound ◽  
The Body ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Methyl Prednisolone ◽  
Organ Systems ◽  
Lupus Hepatitis

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease characterized by autoantibodies against the cell nucleus and involves many organ systems in the body with unknown etiologies and various clinical manifestations, disease course and prognosis. SLE can be found at all ages, generally appearing at age 9-58 years with a peak at age 28 years. It is more common in women with a ratio of women to men 15: 1 to 22: 1. The highest incidence and prevalence of SLE was found in North America 23.2 / 100.000 population / year and 241 / 100.0000 population. In Indonesia, there has been an increase in visits to SLE patients from 17.9-27.2% in 2015 to 30.3-58% in 2017. One of the manifestations of SLE is hepatitis lupus, which is inflammation of the liver tissue. Lupus hepatitis can occur in 20-50% of patients with SLE. It was reported that a 20-year-old man presented with complaints of pain in the joints of the right and left hands which increased since 1 week. The patient also complained of reddish patches on the face, hair loss and mouth sores. Physical examination revealed anemic eye conjunctiva, malar rash, oral ulcer. During the joint examination, there was tenderness in bilateral MCP and PIP. The abdominal examination revealed hepatomegaly. Investigations revealed anemia, thrombocytopenia, increased liver function. Abdominal ultrasound revealed hepatomegaly. ANA profile examination was positive for anti RNP, anti-sm, and anti- ribosomal protein antibodies. The patient was diagnosed with Systemic Lupus Erythematosus with lupus hepatitis according to the ACR (American College of Rheumatology) criteria in which the patient had 6 criteria. The patient was given therapy with 2x125 mg of intravenous methyl prednisolone for 3 days and hydroxychloroquine 1x200 mg orally and other symptomatic drugs.

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