Autoimmune Diseases Overview
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Questions and Answers

What is autoimmunity?

Disorders in which immune responses are targeted toward self-antigens, resulting in damage to organs and tissues in the body.

What is the primary reason for the development of autoimmune diseases?

  • Loss of self-tolerance (correct)
  • Increased sunlight exposure
  • Enhanced physical activity
  • Improper diet

    • Women are more likely than men to develop autoimmune diseases.

    True

    What environmental factor has the strongest link to rheumatoid arthritis?

    <p>Cigarette smoking</p> Signup and view all the answers

    The strongest link found with HLA is between the HLA-B27 allele and the development of __________.

    <p>ankylosing spondylitis</p> Signup and view all the answers

    Which of the following is NOT a systemic autoimmune disease?

    <p>Myasthenia gravis</p> Signup and view all the answers

    What are antinuclear antibodies (ANAs)?

    <p>A heterogeneous group of antibodies that have different antigen specificities.</p> Signup and view all the answers

    Match the following autoimmune diseases with their target organs:

    <p>Rheumatoid arthritis = Peripheral joints Hashimoto's thyroiditis = Thyroid gland Type 1 Diabetes mellitus = Pancreas Goodpasture's syndrome = Kidneys and Lungs</p> Signup and view all the answers

    The primary autoantibody associated with systemic lupus erythematosus (SLE) is __________.

    <p>Anti-dsDNA</p> Signup and view all the answers

    Drug-induced lupus is associated with specific medications and symptoms disappear once the drug is stopped.

    <p>True</p> Signup and view all the answers

    Which of the following clinical features is least likely to be associated with drug-induced lupus?

    <p>Skin ulcers</p> Signup and view all the answers

    What laboratory finding is expected in patients with drug-induced lupus?

    <p>Leukopenia</p> Signup and view all the answers

    Which antibody pattern is associated with drug-induced systemic lupus erythematosus (SLE)?

    <p>Homogenous</p> Signup and view all the answers

    Which drug is most commonly implicated in causing drug-induced lupus?

    <p>Chlorpromazine</p> Signup and view all the answers

    Which of the following antibodies is most specific for systemic lupus erythematosus (SLE)?

    <p>Anti-dsDNA</p> Signup and view all the answers

    Which of the following statements about the complement levels in drug-induced lupus is true?

    <p>Complement levels are low</p> Signup and view all the answers

    What is the common symptom that disappears after discontinuation of the offending drug in drug-induced lupus?

    <p>Erythematous rash</p> Signup and view all the answers

    Which type of antinuclear antibodies (ANAs) gives a coarse speckled pattern and is associated with a specific disease?

    <p>Anti-Sm</p> Signup and view all the answers

    An increase in which of the following laboratory tests is consistent with the presence of systemic lupus erythematosus (SLE)?

    <p>Erythrocyte sedimentation rate (ESR)</p> Signup and view all the answers

    Which of the following is least likely to be a systemic effect of drug-induced lupus?

    <p>Weight gain</p> Signup and view all the answers

    What outcome results from the loss of self-tolerance in autoimmune disease?

    <p>Immune response directed against self-antigens</p> Signup and view all the answers

    Which factor is associated with a higher risk of developing autoimmune diseases in women compared to men?

    <p>The stimulatory effect of female hormones</p> Signup and view all the answers

    How do microbial infections potentially trigger autoimmune diseases?

    <p>Through molecular mimicry</p> Signup and view all the answers

    What is the effect of inflammation or tissue trauma in the context of autoimmune disease?

    <p>Triggers the release of cryptic antigens</p> Signup and view all the answers

    Which of the following autoantibodies is NOT typically associated with systemic lupus erythematosus (SLE)?

    <p>Anti-citrullinated protein</p> Signup and view all the answers

    Which statement best describes the underlying issue in autoimmune diseases?

    <p>It confuses self-antigens as foreign substances</p> Signup and view all the answers

    Which allele has the strongest association with ankylosing spondylitis?

    <p>HLA-B27</p> Signup and view all the answers

    Which body systems can be affected by the inflammation caused by systemic lupus erythematosus?

    <p>Skin, kidneys, and heart</p> Signup and view all the answers

    What distinguishes autoimmune diseases from other types of immune disorders?

    <p>They trigger responses against self-tissues</p> Signup and view all the answers

    Which of the following is a true statement regarding the genetics of autoimmune diseases?

    <p>There is a genetic predisposition that increases risk for specific disorders.</p> Signup and view all the answers

    Which of the following antibodies is primarily associated with a homogenous pattern in indirect immunofluorescent assays?

    <p>Anti-dsDNA</p> Signup and view all the answers

    In the context of indirect immunofluorescent assays, what characterizes a speckled pattern?

    <p>Numerous discrete speckles throughout the nucleus</p> Signup and view all the answers

    Which autoimmune disease is associated with antibodies to acetylcholine receptors?

    <p>Myasthenia gravis</p> Signup and view all the answers

    What is the role of rheumatoid factor (RF) in rheumatoid arthritis?

    <p>It targets the Fc region of IgG.</p> Signup and view all the answers

    What is the main autoantibody implicated in autoimmune thrombocytopenic purpura?

    <p>Antiplatelet antibody</p> Signup and view all the answers

    Which autoantibody is specifically associated with rheumatoid arthritis and is directed towards cyclic citrullinated proteins?

    <p>Anti-CCP</p> Signup and view all the answers

    Which disease primarily affects the adrenal glands and is associated with antibodies to adrenals?

    <p>Addison's disease</p> Signup and view all the answers

    Which disease is characterized by the presence of numerous discrete speckles in cell nuclei with a specific relation to CREST syndrome?

    <p>Scleroderma</p> Signup and view all the answers

    In which condition do patients show antibodies against the glomerular basement membrane?

    <p>Goodpasture's syndrome</p> Signup and view all the answers

    What is the most widely used test for detecting antinuclear antibodies (ANAs)?

    <p>Indirect immunofluorescent assay</p> Signup and view all the answers

    Which feature is most characteristic of nucleolar staining in immunofluorescent assays?

    <p>Prominent staining of the nucleoli</p> Signup and view all the answers

    Which of the following autoimmune diseases is characterized by autoantibodies in the small intestine?

    <p>Celiac disease</p> Signup and view all the answers

    What autoimmune disease is strongly linked to cigarette smoking as its environmental risk factor?

    <p>Rheumatoid arthritis</p> Signup and view all the answers

    Which autoimmune disease primarily affects the pancreas?

    <p>Type 1 Diabetes mellitus</p> Signup and view all the answers

    What type of antibodies are seen in patients with autoimmune hepatitis?

    <p>Smooth muscle antibodies</p> Signup and view all the answers

    What pattern is primarily observed in patients with anti-dsDNA antibodies?

    <p>Homogeneous pattern</p> Signup and view all the answers

    Which of the following conditions has autoantibodies that are primarily associated with the Scl-70 antigen?

    <p>Scleroderma</p> Signup and view all the answers

    Which autoimmune disease can manifest with dry eyes and dry mouth?

    <p>Sjogren's syndrome</p> Signup and view all the answers

    Which autoantibody is associated with pernicious anemia?

    <p>Anti-intrinsic factor</p> Signup and view all the answers

    What is the primary autoantibody found in patients with autoimmune hemolytic anemia?

    <p>Antibodies to RBCs</p> Signup and view all the answers

    Study Notes

    Autoimmune Diseases

    • Autoimmune diseases occur when the immune system attacks the body's own tissues and organs.
    • Autoimmune responses can be mediated by autoantibodies or T-cells.
    • Loss of self-tolerance is thought to be the root cause of autoimmune diseases.
    • Genetic factors play a role: HLA alleles are associated with specific autoimmune disorders.
    • Hormonal influences also contribute: Women are more susceptible to autoimmune diseases than men.
    • Tissue trauma can trigger immune responses by releasing cryptic antigens.
    • Microbial infections can contribute through molecular mimicry, where microbial antigens resemble self-antigens.

    Systemic Autoimmune Diseases

    • Systemic Lupus Erythematosus (SLE): Characterized by inflammation affecting various organs.
    • SLE can cause fatigue, weight loss, arthritis, skin rashes, and kidney issues.
    • Drug-induced lupus occurs as a side effect of certain medications and typically resolves upon discontinuation of the offending drug.

    Laboratory Tests for SLE

    • CBC: Often shows leukopenia (low white blood cell count) and anemia.
    • Platelet Count: Thrombocytopenia (low platelet count) is common.
    • ESR: Elevated.
    • CRP: Normal to low.
    • Complement: Low.
    • Antinuclear antibodies (ANAs): A group of antibodies with diverse antigen specificities.
    • Double-stranded DNA (dsDNA) antibodies: Highly specific for SLE.
    • Antihistone antibodies: Suggestive of drug-induced SLE.
    • Extractable nuclear antigens (ENAs): A group of nuclear antigens targeted by autoantibodies in SLE and other autoimmune diseases.

    Immunofluorescence Patterns of ANAs

    • Homogenous pattern: Uniform staining of the nucleus, associated with anti-dsDNA, anti-histones, and anti-DNP.
    • Peripheral pattern: Greater staining intensity around the outer circle of the nucleus, indicative of anti-dsDNA.
    • Speckled pattern: Discrete fluorescent speckles throughout the nuclei, characteristic of anti-ENAs.
    • Nucleolar pattern: Prominent staining of nucleoli within the nuclei, associated with antibodies to RNA and RNP.
    • Centromere pattern: Numerous discrete speckles, seen in CREST syndrome.

    Rheumatoid Arthritis (RA)

    • A chronic, symmetrical, erosive arthritis primarily affecting peripheral joints.
    • Can also affect organs like the heart and lungs.
    • The strongest environmental risk factor is cigarette smoking.
    • Autoantibodies:
      • Rheumatoid factor (RF): An IgM antibody targeting the Fc region of IgG.
      • Antibodies to cyclic citrullinated proteins (anti-CCP): Specifically associated with RA.

    Organ-Specific Autoimmune Diseases

    • Addison's disease: Targets adrenal glands.
    • Autoimmune hemolytic anemia: Targets red blood cells (RBCs).
    • Autoimmune Hepatitis: Targets the liver.
      • Autoantibodies:
        • Smooth muscle antibodies (AIH-1)
        • Anti-liver kidney microsomal antibody (anti-LKM-1)
        • Anti-liver cytosol type 1 antibody (anti-LC-1)
    • Autoimmune thrombocytopenic purpura: Targets platelets.
    • Celiac disease: Targets small intestines.
    • Goodpasture's syndrome: Targets kidneys and lungs.
    • Grave's disease: Targets thyroid gland.
    • Hashimoto's thyroiditis: Targets thyroid gland.
    • Multiple sclerosis: Targets myelin sheath of nerves.
    • Myasthenia gravis: Targets nerve-muscle synapse.
    • Pernicious anemia: Targets stomach.
    • Poststreptococcal glomerulonephritis: Targets kidneys.
    • Primary biliary cirrhosis: Targets intrahepatic bile ducts.
    • Rheumatic fever: Targets heart.
    • Scleroderma: Targets connective tissues.
    • Sjogren's syndrome: Targets eyes and mouth.
    • Type 1 Diabetes mellitus: Targets pancreas.

    Autoimmunity

    • Immune responses are targeted toward self-antigens, resulting in organ and tissue damage.
    • Causes can be autoimmune antibodies or T-cell mediated responses.

    Etiology of Autoimmune Disease

    • Loss of self-tolerance, which is the immune system's ability to accept self-antigens without reacting.
    • Genetics: HLA association with risk of developing autoimmune disorders, HLA-B27 allele strongly linked to ankylosing spondylitis.
    • Hormonal Influence: Women are more likely to develop autoimmune diseases due to the stimulatory effect of female hormones.
    • Tissue Trauma: Inflammation or trauma releases cryptic antigens, making them accessible to lymphocytes and triggering immune responses.
    • Microbial Infections: Microbes can cause autoimmune diseases through molecular mimicry, where microbial antigens resemble self-antigens.

    Systemic Lupus Erythematosus (SLE)

    • Autoimmune disease where the body's immune system attacks its own tissues and organs.
    • Inflammation can affect multiple body systems, including joints, skin, kidneys, blood cells, brain, heart, and lungs.
    • Clinical Signs: Fatigue, weight loss, malaise, fever, anorexia, symmetrical arthritis, erythematous rash, and other systemic effects.
    • Drug-induced lupus: Common implicated drugs include procainamide, hydralazine, chlorpromazine, isoniazid, quinidine, methyldopa, and oral contraceptives. Symptoms disappear when the drug is discontinued.

    Laboratory Tests for SLE

    • CBC: Leukopenia, anemia, Thrombocytopenia
    • ESR: Increased
    • CRP: Normal to Low
    • Complement: Low

    Antinuclear Antibodies (ANAs)

    • Heterogeneous group of antibodies with different antigen specificities.
    • Anti-dsDNA: Peripheral or homogenous pattern, associated with SLE.
    • Anti-ssDNA: Not detected on routine screens, associated with SLE.
    • Antihistone: Homogenous pattern, associated with drug-induced SLE and SLE.
    • Anti-DNP: Homogenous pattern, associated with drug-induced SLE and SLE.
    • Anti-Sm: Coarse speckled pattern, diagnostic for SLE.
    • Anti-SS-A (Ro): Finely speckled pattern, associated with SLE and Sjögren's syndrome.
    • Anti-SS-B (La): Finely speckled pattern, associated with SLE and Sjögren's syndrome.
    • Antinucleolar: Prominent staining of nucleoli, associated with SLE and systemic sclerosis.
    • Anti-Scl-70: Atypical speckled pattern, associated with systemic sclerosis (Scleroderma).
    • Anti-Jo-1: Fine cytoplasmic speckling, associated with polymyositis.
    • Anti-centromere: Discrete speckled pattern, associated with CREST syndrome.

    Tests and Patterns for ANAs

    • Double-stranded DNA (dsDNA) antibodies: Peripheral or homogenous pattern, most specific for SLE.
    • Antihistone antibodies: Supports the diagnosis of drug-induced SLE.
    • Extractable nuclear antigens (ENAs): Isolated in saline extracts of mammalian tissues, examples include RNP, Sm, SS-A (Ro), SS-B (La), Scl-70, Jo-1, and PM-1.
    • Indirect immunofluorescent assay: Most widely used for ANAs, highly sensitive, detects a wide range of antibodies, inexpensive and easy to perform. Hep-2 is the substrate used.
    • Homogenous pattern: uniform staining of the entire nucleus, associated with Anti-dsDNA, Anti-histones, and Anti-DNP, found in SLE, drug-induced SLE and other AIDs.
    • Peripheral pattern: Greater staining intensity around the outer circle of the nucleus, seen when anti-dsDNA is present, most specific for SLE.
    • Speckled pattern: Discrete fluorescent specs throughout the nuclei, characteristic of anti-ENAs.
    • Nucleolar pattern: Prominent staining of nucleoli, due to antibodies to RNA and RNP, mainly seen in Scleroderma.
    • Centromere pattern: Numerous discrete speckles, seen in CREST patients with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.

    Rheumatoid Arthritis (RA)

    • Chronic, symmetric, and erosive arthritis of the peripheral joints, affecting multiple organs like the heart and lungs.
    • Strongest environmental risk is cigarette smoking.
    • Autoantibodies:
      • Rheumatoid factor (RF)
      • Antibodies to CCP
    • Rheumatoid factor: IgM antibody directed towards the Fc region of IgG.
    • Anti-CCP: Anti-cyclic citrullinated proteins, specifically associated with RA.

    Organ-Specific Autoimmune Diseases

    • Addison's disease:
      • Target: Adrenal glands
      • Autoantibodies: Antibodies to adrenals
    • Autoimmune hemolytic anemia:
      • Target: RBCs
      • Autoantibodies: Antibodies to RBCs
    • 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:
      • Target: Platelets
      • Autoantibodies: Antiplatelet antibody
    • 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 of the lungs and kidneys
    • Grave's disease:
      • Target: Thyroid gland
      • Autoantibodies:
        • Thyroid stimulation hormone receptor antibodies (TRAbs)
        • Antithyroglobulin
        • Antithyroid peroxidase (TPO)
    • Hashimoto's thyroiditis:
      • Target: Thyroid gland
      • Autoantibodies:
        • Antithyroglobulin
        • Antithyroid peroxidase (TPO)
    • Multiple sclerosis:
      • Target: Myelin sheath of nerves
      • Autoantibodies: Antibodies to myelin basic protein
    • Myasthenia gravis:
      • 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
    • 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 and 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
        • Antibody to glutamic acid phosphatase

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    Description

    This quiz explores the mechanisms and factors involved in autoimmune diseases, including systemic lupus erythematosus. Understand how the immune system can fail and the roles of genetics, hormones, and infections in these conditions. Test your knowledge on the characteristics and triggers of autoimmune disorders.

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