SLIDE 1 Hypersensitivity. This Week's Lecture Will Elaborate on Concepts
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Vaccination and Autoimmune Disease: What Is the Evidence?
REVIEW Review Vaccination and autoimmune disease: what is the evidence? David C Wraith, Michel Goldman, Paul-Henri Lambert As many as one in 20 people in Europe and North America have some form of autoimmune disease. These diseases arise in genetically predisposed individuals but require an environmental trigger. Of the many potential environmental factors, infections are the most likely cause. Microbial antigens can induce cross-reactive immune responses against self-antigens, whereas infections can non-specifically enhance their presentation to the immune system. The immune system uses fail-safe mechanisms to suppress infection-associated tissue damage and thus limits autoimmune responses. The association between infection and autoimmune disease has, however, stimulated a debate as to whether such diseases might also be triggered by vaccines. Indeed there are numerous claims and counter claims relating to such a risk. Here we review the mechanisms involved in the induction of autoimmunity and assess the implications for vaccination in human beings. Autoimmune diseases affect about 5% of individuals in Autoimmune disease and infection developed countries.1 Although the prevalence of most Human beings have a highly complex immune system autoimmune diseases is quite low, their individual that evolved from the fairly simple system found in incidence has greatly increased over the past few years, as invertebrates. The so-called innate invertebrate immune documented for type 1 diabetes2,3 and multiple sclerosis.4 system responds non-specifically to infection, does not Several autoimmune disorders arise in individuals in age- involve lymphocytes, and hence does not display groups that are often selected as targets for vaccination memory. -
Goodpasture Syndrome
orphananesthesia Anaesthesia recommendations for Goodpasture syndrome Disease name: Goodpasture syndrome ICD 10: M31.0 Synonyms: Goodpasture’s syndrome (GS), anti-glomerular basement membrane disease, crescentic glomerulonephritis type 1, GPS Disease summary: Goodpasture syndrome is a rare and organ-specific autoimmune disease (Gell and Coombs classification type II). It is mediated by anti-glomerular basement membrane (anti-GBM) antibodies [7]. The disease was first described by Dr. Ernest Goodpasture in 1919 [6], whereby the glomerular basement membrane was first identified as antigen in 1950s. More than one decade later, researchers succeeded in defining the association between antibodies taken from diseased kidneys and nephritis [7]. The disorder is characterised by autoantibodies targeting at the NC1-domain of the α3 chain of type IV collagen in the glomerular and alveolar basement membrane with activation of the complement cascade among other things [7,17]. The exclusive location of this α3 subunit in basement membranes only in lung and kidney is responsible for the unique affection of these two organs in GPS [7]. Nevertheless, the aetiology and the triggering stimuli for anti-GBM production remain unknown. Due to the fact that patients with specific human leukocyte antigen (HLA) types are more susceptible, a genetic predisposition HLA-associated seems possible [4,7]. However, because this strongly associated allele is frequently present, there seem to be additional behavioural or environmental factors influencing immune response and disease expression. The latter may include respiratory infections (e.g., influenza A2), exposition to hydrocarbon fumes, organic solvents, metallic dust, tobacco smoke, certain drugs (i.e., rifampicin, allopurinol, cocaine), physical damage to basement membrane (e.g., lithotripsy or membranous glomerulonephritis) as well as lymphocyte-depletion therapy (such as alemtuzumab), but unequivocal evidence is lacking [4,7,9,17]. -
Goodpasture's Syndrome: an Analysis of 29 Cases
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector Kidney International, Vol. 13 (1978), pp. 492—504 Goodpasture's syndrome: An analysis of 29 cases CLINTON A. TEAGUE, PETER B. DOAK, IAN J. SIMPSON, STEPHEN P. RAINER, and PETER B. HERDSON The Departments of Pathology and Medicine, University of Auckland School of Medicine, Auckland, New Zealand Goodpasture's syndrome: An analysis of 29 cases. The patho- ation between lung hemorrhage and glomeruloneph- logic features of 29 cases of Goodpasture's syndrome occurring during a 13-yr period in Auckland have been reviewed and corre- ntis classically affecting young men. They referred to lated with clinical findings. There were 20 males and nine females such a patient reported about 40 years previously by in the series; two of the males and three of the females were Goodpasture [2]. During the past two decades, a Maoris. Age at the time of onset of symptoms ranged from 17 to 75 great deal has been learned about the immunological yr, with about 76% of the patients being from 17 to 27 yr of age. Sixteen (55%) of the patients died from less than a week up to aspects of several forms of glomerulonephritis, in- about two years following the onset of symptoms, and the remain- cluding the anti-glomerular basement membrane ing 13 are alive from 30 weeks to 14 yr after initial presentation. (anti-GBM) type of disease with cross-reactivity be- Underlying renal disease varied from mild focal glomerulitis to end-stage glomerulonephritis by light microscopy, but characteris- tween lung and kidney which is a feature of Good- tic glomerular changes were seen in all specimens examined by pasture's syndrome [3]. -
ANCA--Associated Small-Vessel Vasculitis
ANCA–Associated Small-Vessel Vasculitis ISHAK A. MANSI, M.D., PH.D., ADRIANA OPRAN, M.D., and FRED ROSNER, M.D. Mount Sinai Services at Queens Hospital Center, Jamaica, New York and the Mount Sinai School of Medicine, New York, New York Antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis is the most common primary sys- temic small-vessel vasculitis to occur in adults. Although the etiology is not always known, the inci- dence of vasculitis is increasing, and the diagnosis and management of patients may be challenging because of its relative infrequency, changing nomenclature, and variability of clinical expression. Advances in clinical management have been achieved during the past few years, and many ongoing studies are pending. Vasculitis may affect the large, medium, or small blood vessels. Small-vessel vas- culitis may be further classified as ANCA-associated or non-ANCA–associated vasculitis. ANCA–asso- ciated small-vessel vasculitis includes microscopic polyangiitis, Wegener’s granulomatosis, Churg- Strauss syndrome, and drug-induced vasculitis. Better definition criteria and advancement in the technologies make these diagnoses increasingly common. Features that may aid in defining the spe- cific type of vasculitic disorder include the type of organ involvement, presence and type of ANCA (myeloperoxidase–ANCA or proteinase 3–ANCA), presence of serum cryoglobulins, and the presence of evidence for granulomatous inflammation. Family physicians should be familiar with this group of vasculitic disorders to reach a prompt diagnosis and initiate treatment to prevent end-organ dam- age. Treatment usually includes corticosteroid and immunosuppressive therapy. (Am Fam Physician 2002;65:1615-20. Copyright© 2002 American Academy of Family Physicians.) asculitis is a process caused These antibodies can be detected with indi- by inflammation of blood rect immunofluorescence microscopy. -
Difference Between Hypersensitivity and Autoimmunity Key Difference – Hypersensitivity Vs Autoimmunity
Difference Between Hypersensitivity and Autoimmunity www.differencebetwee.com Key Difference – Hypersensitivity vs Autoimmunity Autoimmunity is an adaptive immune response mounted against self-antigens. In simple terms, when your body is acting against its own cells and tissues, this is called an autoimmune reaction. An exaggerated and inappropriate immune response to an antigenic stimulus is defined as a hypersensitivity reaction. Unlike autoimmune reactions that are triggered only by the endogenous antigens, hypersensitivity reactions are triggered by both endogenous and exogenous antigens. This is the key difference between hypersensitivity and autoimmunity. What is Hypersensitivity? An exaggerated and inappropriate immune response to an antigenic stimulus is defined as a hypersensitivity reaction. The first exposure to a particular antigen activates the immune system and, antibodies are produced as a result. This is called sensitization. Subsequent exposures to the same antigen give rise to hypersensitivity. Few important facts regarding the hypersensitivity reactions are given below They can be elicited by both exogenous and endogenous agents. They are a result of an imbalance between the effector mechanisms and the countermeasures that are there to control any inappropriate execution of an immune response. The presence of a genetic susceptibility increases the likelihood of hypersensitivity reactions. The manner in which hypersensitivity reactions harm our body is similar to the way that the pathogens are destroyed by immune reactions. Figure 01: Allergy According to the Coombs and Gell classification, there are four main types of hypersensitivity reactions. Type I- Immediate Type/ Anaphylactic Mechanism Vasodilation, edema, and contraction of smooth muscles are the pathological changes that take place during the immediate phase of the reaction. -
Anti-Glomerular Basement Membrane (GBM) Disease (Goodpasture's Syndrome)
Patient information – Goodpasture’s Syndrome Anti-Glomerular Basement Membrane (GBM) Disease (Goodpasture’s Syndrome) What is it? Goodpasture’s Syndrome is a type of vasculitis (inflammation of blood vessels), which affects the kidneys and the lungs. What causes it? The body normally produces antibodies to fight off infection and disease. In this case, your body makes an antibody that can attack and damages a membrane in your kidneys and lungs. What symptoms might I have? You may feel short of breath and cough up blood. The kidney damage may cause blood-stained or frothy urine or actual kidney failure. Is it serious? Without treatment, the condition can be life-threatening. In some cases, it may be too advanced for treatment to save the kidneys and dialysis will be necessary. However, powerful treatment is now very successful in saving lives and kidney function. Inpatient treatment As soon as diagnosis is made (on blood test or kidney biopsy), treatment will start with Prednisolone (steroid) and Cyclophosphamide (immunosuppressant). · Prednisolone 1mg/Kg of body weight (max 60mg) · IV Cyclophosphamide · Plasma exchange daily for 14 days or until antibody negative Discharge medication [Week One] Prednisolone Inpatient dose Lansoprazole 30mg daily Alendronate (non-dialysis) 70mg weekly Nystatin 1ml four times a day Septrin 480mg daily Anti-GBM Disease (Goodpasture’s Syndrome), March 2019 1 Anti-GBM Disease (Goodpasture’s Syndrome) Outpatient treatment The condition is dangerous, requiring powerful treatment that can have serious side effects. You will be seen often and monitored carefully. Your blood will be checked for its white cell count (WCC) to check how it would respond to infection. -
Conditions Related to Inflammatory Arthritis
Conditions Related to Inflammatory Arthritis There are many conditions related to inflammatory arthritis. Some exhibit symptoms similar to those of inflammatory arthritis, some are autoimmune disorders that result from inflammatory arthritis, and some occur in conjunction with inflammatory arthritis. Related conditions are listed for information purposes only. • Adhesive capsulitis – also known as “frozen shoulder,” the connective tissue surrounding the joint becomes stiff and inflamed causing extreme pain and greatly restricting movement. • Adult onset Still’s disease – a form of arthritis characterized by high spiking fevers and a salmon- colored rash. Still’s disease is more common in children. • Caplan’s syndrome – an inflammation and scarring of the lungs in people with rheumatoid arthritis who have exposure to coal dust, as in a mine. • Celiac disease – an autoimmune disorder of the small intestine that causes malabsorption of nutrients and can eventually cause osteopenia or osteoporosis. • Dermatomyositis – a connective tissue disease characterized by inflammation of the muscles and the skin. The condition is believed to be caused either by viral infection or an autoimmune reaction. • Diabetic finger sclerosis – a complication of diabetes, causing a hardening of the skin and connective tissue in the fingers, thus causing stiffness. • Duchenne muscular dystrophy – one of the most prevalent types of muscular dystrophy, characterized by rapid muscle degeneration. • Dupuytren’s contracture – an abnormal thickening of tissues in the palm and fingers that can cause the fingers to curl. • Eosinophilic fasciitis (Shulman’s syndrome) – a condition in which the muscle tissue underneath the skin becomes swollen and thick. People with eosinophilic fasciitis have a buildup of eosinophils—a type of white blood cell—in the affected tissue. -
Ii Vasclitides
ا يمون وپات وژنز واسکوليتھااکلتا Vascliiitides: The Immunopa tho genes is دکتر محمدمھدی محمدی LMD, PhD, MPH ([email protected]) The 7th International 12th National Congress on Quality Improvement in Clinical Laboratories 28 Farvardin 1393 / 17April 2014 , Tehran, Iran A small spoonful of Terminology (in Farsi بيماريزايی) – .path·o·gen·e·sis, n • – the ppp(roduction and development of disease (events and reactions and other pathologic mechanisms) . Also, pa·thog·e·ny !?! (in Farsi بيماريزايی) _ .path·o·ge·nic·i·ty, n • – the disease-producing capacity of a pathogen. [PATHOGENIC + -ITY] • vir·u·lence, n. – the relative ability of a microorganism to cause disease; degree of pathogenicity. • Vasculitis, n. (vasculitides, plural) – a general term for vessel wall inflammation, systemic or localized, immune-mediated or directly invaded by microbes. • Infections may indirectly generate vasculitis, e.g. through IC formation or by X_reaction. Note that immunosuppressive therapy is only appropriate for --?– type! • WHAT R the IMMUNE MECHANISMS in VASCULITIS? افراط تعادل تفريط Immunodeficiency Allergy (& Infection) Defence Immunodeficiency Surveillance Autoimmunity (& Malignancy) Homostasis ?! Some Inter-related Concepts Immunological Vasculitis Hypersensitivity Auoimmunity Tolerance EDUCATION? Tolerance in different classic Txtbks of Immunology (Abbas, Benjamini, Janeway, Kuby, Roitt, Stites …) • Tolerance (by C&M Immunology): – Unresponsiveness of the adaptive immune system to antigens, as a result of inactivation or death of antigen-specific lymphocytes, induced by exposure to the antigens. – (()Active) Unresponsiveness of the (()HEALTHY) adappytive immune system to (selected) antigens, as a result of (i.e. MECHANISMS:) inactivation or death of antigen-specific lymphocytes, induced by exposure to those antigens. • Tolerance to self antigens is a normal feature of the adaptive immune system, but tolerance to foreign antigens may be induced undtiditiftider certain conditions of antigen exposure. -
African Americans and Lupus
African Americans QUICK GUIDE and Lupus 1 Facts about lupus n People of all races and ethnic groups can develop lupus. n Women develop lupus much more often than men: nine of every 10 It is not people with lupus are women. Children can develop lupus, too. known why n Lupus is three times more common in African American women than lupus is more in Caucasian women. common n As many as 1 in 250 African American women will develop lupus. in African Americans. n Lupus is more common, occurs at a younger age, and is more severe in African Americans. Some scientists n It is not known why lupus is more common in African Americans. Some scientists think that it is related to genes, but we know that think that it hormones and environmental factors play a role in who develops is related to lupus. There is a lot of research being done in this area, so contact the genes, but LFA for the most up-to-date research information, or to volunteer for we know that some of these important research studies. hormones and environmental What is lupus? factors play 2 n Lupus is a chronic autoimmune disease that can damage any part of a role in who the body (skin, joints and/or organs inside the body). Chronic means develops that the signs and symptoms tend to persist longer than six weeks lupus. and often for many years. With good medical care, most people with lupus can lead a full life. n With lupus, something goes wrong with your immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu). -
Tests for Autoimmune Diseases Test Codes 249, 16814, 19946
Tests for Autoimmune Diseases Test Codes 249, 16814, 19946 Frequently Asked Questions Panel components may be ordered separately. Please see the Quest Diagnostics Test Center for ordering information. 1. Q: What are autoimmune diseases? A: “Autoimmune disease” refers to a diverse group of disorders that involve almost every one of the body’s organs and systems. It encompasses diseases of the nervous, gastrointestinal, and endocrine systems, as well as skin and other connective tissues, eyes, blood, and blood vessels. In all of these autoimmune diseases, the underlying problem is “autoimmunity”—the body’s immune system becomes misdirected and attacks the very organs it was designed to protect. 2. Q: Why are autoimmune diseases challenging to diagnose? A: Diagnosis is challenging for several reasons: 1. Patients initially present with nonspecific symptoms such as fatigue, joint and muscle pain, fever, and/or weight change. 2. Symptoms often flare and remit. 3. Patients frequently have more than 1 autoimmune disease. According to a survey by the Autoimmune Diseases Association, it takes up to 4.6 years and nearly 5 doctors for a patient to receive a proper autoimmune disease diagnosis.1 3. Q: How common are autoimmune diseases? A: At least 30 million Americans suffer from 1 or more of the 80 plus autoimmune diseases. On average, autoimmune diseases strike three times more women than men. Certain ones have an even higher female:male ratio. Autoimmune diseases are one of the top 10 leading causes of death among women age 65 and under2 and represent the fourth-largest cause of disability among women in the United States.3 Women’s enhanced immune system increases resistance to infection, but also puts them at greater risk of developing autoimmune disease than men. -
Food Fact Sheet: Food Allergy and Food Intolerance
Food Fact Sheet: Food Allergy and Food Intolerance Having to avoid certain foods in your diet can be difficult. But there are a few simple things you can do to help you manage your food allergies - allowing you to stay safe, continue to participate in fun activities and enjoy your food. What is the difference between food allergy and food intolerance? For some people, eating certain foods can lead to an unpleasant and sometimes dangerous physical reaction. The term used to describe all types of reactions to foods is ‘food hypersensitivity’. A 'food allergy' is a reaction involving the immune system (the body’s defence against foreign bodies). Those that do not involve the immune system are often called a ‘food intolerance’. It is important to identify and manage foods that trigger any symptoms in an appropriate way. Food allergy Proteins within foods can trigger immediate (within two hours) or delayed symptoms (up to several days later). Immediate food allergy (IgE mediated food allergy) Immediate reactions to foods occur when your immune system reacts to a normally harmless protein in food, due to the creation of Immunoglobulin E (IgE). This results in the release of chemicals (e.g. histamine) which trigger allergic symptoms. These symptoms are usually in the skin (itching/swelling), or gut (vomiting, diarrhoea). Other symptoms can include breathing problems and in rare cases an extreme allergic reaction called anaphylaxis. Delayed food allergy (non IgE mediated food allergy) Delayed reactions to foods still involve your immune system, but there is a different type of immune reaction involved. Symptoms typically occur in the gut (vomiting, diarrhoea, constipation) and/or the skin (atopic eczema). -
Allergy, Hypersensitivity, Angioedema, and Anaphylaxis Episode Overview
CrackCast Show Notes –Allergy and Anaphylaxis – October 2017 www.canadiem.org/crackcast Chapter 109 – Allergy, Hypersensitivity, Angioedema, and Anaphylaxis Episode Overview Key Points: 1. A history of sudden urticarial rash accompanied by respiratory difficulty, abdominal pain, or hypotension, strongly favors the diagnosis of anaphylaxis. 2. Epinephrine is the first-line treatment in patients with anaphylaxis: give it immediately. 3. There are no absolute contraindications to the use of epinephrine in the setting of anaphylaxis. 4. Antihistamines and corticosteroids are second- and third-line agents in the management of anaphylaxis and should not replace or precede epinephrine. 5. Consider prolonged observation or admission for patients who: a. Experience protracted anaphylaxis, hypotension, or airway involvement; b. Receive IV epinephrine or more than two doses of IM epinephrine; c. Or have poor outpatient social support. 6. Patients discharged after an anaphylactic event should be prescribed an EpiPen and instructed on its use. 7. Patients with refractory hypotension may require glucagon (receiving beta-blockage) or a continuous IV epinephrine infusion. 8. Non-histaminergic angioedema (non-allergic angioedema) does not typically respond to epinephrine and antihistamines. New drugs, including berinert, icatibant, ecallantide, and Ruconest have been approved for use in HAE. FFP has been used with varying success in HAE, ACID, and ACE inhibitor–induced angioedema. NOTE: ACID: acquired C1 esterase deficiency (ACED) Core Questions: 1. List the four types of Gell and Coombs classifications of immune reaction and give examples of each 2. List four etiologic agents causing anaphylaxis by immunologic mechanisms 3. List six mediators of anaphylaxis and their physiologic actions and clinical manifestations 4.