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Questions and Answers
Which autoimmune disease is characterized by antibodies to acetylcholine receptors?
Which autoimmune disease is characterized by antibodies to acetylcholine receptors?
What target organ is primarily affected by autoimmune hemolytic anemia?
What target organ is primarily affected by autoimmune hemolytic anemia?
In which autoimmune disease are antimitochondrial antibodies found?
In which autoimmune disease are antimitochondrial antibodies found?
Which of the following autoimmune diseases targets the myelin sheath?
Which of the following autoimmune diseases targets the myelin sheath?
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Which autoimmune condition involves autoantibodies against smooth muscle?
Which autoimmune condition involves autoantibodies against smooth muscle?
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Which factor is NOT considered an etiology of autoimmune disease?
Which factor is NOT considered an etiology of autoimmune disease?
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What is the primary effect of loss of self-tolerance in the immune system?
What is the primary effect of loss of self-tolerance in the immune system?
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Which HLA allele has the strongest association with ankylosing spondylitis?
Which HLA allele has the strongest association with ankylosing spondylitis?
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What demographic is more likely to develop autoimmune diseases due to hormonal influence?
What demographic is more likely to develop autoimmune diseases due to hormonal influence?
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What mechanism describes how microbial infections can lead to autoimmunity?
What mechanism describes how microbial infections can lead to autoimmunity?
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Which of the following autoantibodies is associated with systemic lupus erythematosus (SLE)?
Which of the following autoantibodies is associated with systemic lupus erythematosus (SLE)?
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In systemic lupus erythematosus (SLE), which organ system is commonly affected?
In systemic lupus erythematosus (SLE), which organ system is commonly affected?
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What triggers immune responses in tissue trauma relating to autoimmune disorders?
What triggers immune responses in tissue trauma relating to autoimmune disorders?
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Which autoantibody is most specifically associated with systemic lupus erythematosus (SLE)?
Which autoantibody is most specifically associated with systemic lupus erythematosus (SLE)?
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What type of staining is characterized by uniform staining of the entire nucleus?
What type of staining is characterized by uniform staining of the entire nucleus?
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In patients with scleroderma, which type of ANA pattern is most commonly observed?
In patients with scleroderma, which type of ANA pattern is most commonly observed?
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What is the primary environmental risk factor associated with rheumatoid arthritis?
What is the primary environmental risk factor associated with rheumatoid arthritis?
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What is the hallmark pattern of anti-dsDNA antibodies most specific for SLE?
What is the hallmark pattern of anti-dsDNA antibodies most specific for SLE?
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Which autoantibody is characterized by an antibody produced by patients with rheumatoid arthritis directed towards the Fc region of IgG?
Which autoantibody is characterized by an antibody produced by patients with rheumatoid arthritis directed towards the Fc region of IgG?
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Which option describes the speckled ANA pattern?
Which option describes the speckled ANA pattern?
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Which laboratory test result is commonly associated with drug-induced lupus?
Which laboratory test result is commonly associated with drug-induced lupus?
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What is the standard substrate used for the indirect immunofluorescent assay for ANAs?
What is the standard substrate used for the indirect immunofluorescent assay for ANAs?
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Which of the following antibodies is diagnostic for systemic lupus erythematosus?
Which of the following antibodies is diagnostic for systemic lupus erythematosus?
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Which symptoms are characteristic of the CREST syndrome associated with scleroderma?
Which symptoms are characteristic of the CREST syndrome associated with scleroderma?
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What is a common systemic effect of drug-induced lupus?
What is a common systemic effect of drug-induced lupus?
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Which autoantibody is specifically associated with rheumatoid arthritis, aside from rheumatoid factor?
Which autoantibody is specifically associated with rheumatoid arthritis, aside from rheumatoid factor?
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Which of the following medications is NOT commonly implicated in drug-induced lupus?
Which of the following medications is NOT commonly implicated in drug-induced lupus?
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What feature characterizes the nucleolar ANA pattern?
What feature characterizes the nucleolar ANA pattern?
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Which antibody pattern is indicative of drug-induced lupus?
Which antibody pattern is indicative of drug-induced lupus?
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What is the typical clinical progression of symptoms in drug-induced lupus once the offending drug is discontinued?
What is the typical clinical progression of symptoms in drug-induced lupus once the offending drug is discontinued?
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Which specific antibody is associated with polymyositis?
Which specific antibody is associated with polymyositis?
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What does the presence of antihistone antibodies support in a clinically evaluated patient?
What does the presence of antihistone antibodies support in a clinically evaluated patient?
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Which antibody pattern is associated with systemic sclerosis?
Which antibody pattern is associated with systemic sclerosis?
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Study Notes
Autoimmunity
- Autoimmunity is a condition where the body's immune system attacks its own tissues and organs.
- This can be caused by autoimmune antibodies or T-cell-mediated immune responses.
- The immune system's ability to tolerate self-antigens is called self-tolerance.
- The loss of self-tolerance is thought to be the cause of autoimmune diseases.
- There is an association between HLA and the risk of developing a specific autoimmune disorder.
- HLA-B27 allele is strongly linked to the development of ankylosing spondylitis.
- Women are 2.7 times more likely to develop autoimmune diseases than men.
- This may be due to the stimulatory effects of female hormones.
- Tissue trauma can release cryptic antigens, which are hidden antigens that can trigger an immune response in lymphocytes.
- Microbial infections can trigger autoimmunity through molecular mimicry, where microbial antigens resemble self-antigens.
Systemic Autoimmune Diseases
- Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect various organs, including the joints, skin, kidneys, blood cells, brain, heart, and lungs.
- SLE can cause nonspecific symptoms like fatigue, weight loss, malaise, fever, and anorexia.
- It can also lead to symmetrical arthritis, erythematous rash, and other systemic effects.
- Drug-induced lupus is caused by certain medications, including procainamide, hydralazine, chlorpromazine, isoniazid, quinidine, methyldopa, and oral contraceptives.
- Symptoms of drug-induced lupus usually disappear when the offending drug is discontinued.
Antinuclear Antibodies (ANAs)
- ANAs are a heterogeneous group of antibodies with different antigen specificities.
- The most specific antibody for SLE is double-stranded DNA (dsDNA) antibody, which can produce a peripheral (rim) or homogenous pattern in immunofluorescence testing.
- Antihistone antibodies are associated with drug-induced SLE.
- Extractable nuclear antigens (ENAs) are a group of antigens that can be extracted from mammalian tissues.
- Examples of ENAs include ribonucleoproteins (RNP), Smith (Sm) antigen, SS-A (Ro), SS-B (La), Scl-70, Jo-1, and PM-1.
- ENAs are commonly detected through indirect immunofluorescent assays using Hep-2 cells.
- Homogenous pattern: Uniform staining of the entire nucleus, associated with anti-dsDNA, anti-histones, and anti-DNP antibodies.
- Peripheral pattern: Greater staining intensity around the outer circle of the nucleus, seen with anti-dsDNA antibodies.
- Speckled pattern: Discrete fluorescent speckles throughout the nuclei, characteristic of anti-ENAs.
- Nucleolar pattern: Prominent staining of the nucleoli within the nuclei, seen with antibodies to RNA and RNP, mainly in Scleroderma.
- Centromere pattern: Numerous discrete speckles seen in CREST patients (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias).
Rheumatoid Arthritis
- Rheumatoid arthritis (RA) is a chronic, symmetric, erosive arthritis affecting peripheral joints and potentially other organs like the heart and lungs.
- Cigarette smoking is the strongest environmental risk factor for RA.
- Autoantibodies associated with RA include rheumatoid factor (RF) and antibodies to CCP.
- Rheumatoid factor (RF) is an IgM antibody produced by RA patients directed against the Fc region of IgG.
- Anti-CCP antibodies are specifically associated with RA and target cyclic citrullinated proteins.
Organ-Specific Autoimmune Diseases
- Addison's disease: Target: Adrenal glands, autoantibodies: Anti-adrenal antibodies.
- Autoimmune hemolytic anemia: Target: RBCs, autoantibodies: Anti-RBC antibodies.
- Autoimmune hepatitis: Target: Liver, autoantibodies: Smooth muscle antibodies (AIH-1), anti-liver kidney microsomal antibody (anti-LKM-1; AIH-2), anti-liver cytosol type 1 antibody (anti-LC-1; AIH-2).
- Autoimmune thrombocytopenic purpura (ITP): Target: Platelets, autoantibodies: Antiplatelet antibodies.
- Celiac disease: Target: Small intestines, autoantibodies: Anti-transglutaminase (tTG), antibodies to deaminated gliadin peptides (DGPs), endomysial antibodies.
- Goodpasture's syndrome: Target: Kidneys and lungs, autoantibodies: Antiglomerular basement membrane antibodies.
- Graves' disease: Target: Thyroid gland, autoantibodies: Thyroid stimulating hormone receptor antibodies (TRAbs), antithyroglobulin, antithyroid peroxidase (TPO).
- Hashimoto's thyroiditis: Target: Thyroid gland, autoantibodies: Antithyroglobulin, antithyroid peroxidase (TPO).
- Multiple sclerosis (MS): Target: Myelin sheath of nerves, autoantibodies: Antibodies to myelin basic protein.
- Myasthenia gravis (MG): Target: Nerve-muscle synapse, autoantibodies: Antibodies to acetylcholine receptors (AChR), anti-muscle-specific kinase, antibody to the lipoprotein (LRP4).
- Pernicious anemia: Target: Stomach, autoantibodies: Parietal cell antibody, anti-intrinsic factor.
- Poststreptococcal glomerulonephritis: Target: Kidneys, autoantibodies: Streptococcal antibodies that cross-react with kidney tissue.
- Primary biliary cirrhosis: Target: Intrahepatic bile ducts, autoantibodies: Antimitochondrial antibodies (AMA).
- Rheumatic fever: Target: Heart, autoantibodies: Streptococcal antibodies that cross-react with cardiac tissue.
- Scleroderma: Target: Connective tissues, autoantibodies: Antinuclear antibodies, anti-Scl-70, anticentromere antibody.
- Sjögren's syndrome: Target: Eyes, mouth, autoantibodies: Antinuclear antibodies, rheumatoid factor, anti-salivary duct antibodies, anti-lacrimal gland antibodies.
- Type 1 Diabetes mellitus: Target: Pancreas, autoantibodies: Anti-insulin, islet cell antibodies, anti-IA-2 and anti-IA-2ßA, antibodies to glutamic acid phosphatase.
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Description
This quiz covers the fundamentals of autoimmunity, including the mechanisms behind autoimmune diseases, the role of the immune system, and factors contributing to susceptibility. Discover the link between genetics and systemic autoimmune disorders, as well as the impact of environmental triggers. Challenge your knowledge on key concepts like self-tolerance and microbial mimicry.