Inflammation and Autoimmune Disorders Quiz
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Questions and Answers

Which type of tissue is characterized by stem cells that continuously cycle to regenerate?

  • Quiescent tissues
  • Stable tissues
  • Permanent tissues
  • Labile tissues (correct)

    • What initiates the healing process after a wound occurs?

  • Cellular replication
  • Vascularization
  • Fibrosis
  • Inflammation (correct)

    • Which type of tissue is noted for being quiescent but capable of reentering the cell cycle when required?

  • Permanent tissues
  • Regenerative tissues
  • Stable tissues (correct)
  • Labile tissues

    • How does the liver regenerate after a partial resection?

    <p>Via compensatory hyperplasia</p> Signup and view all the answers

    Which type of tissues has a limited regenerative capacity and cannot regenerate once damaged?

    <p>Permanent tissues</p> Signup and view all the answers

    Which antiphospholipid antibodies are significant in diagnosing antiphospholipid antibody syndrome?

    <p>Anti-β2-glycoprotein I</p> Signup and view all the answers

    What is the most common cause of death in patients with lupus?

    <p>Renal failure</p> Signup and view all the answers

    What body systems are rarely involved in drug-induced lupus characterized by antihistone antibodies?

    <p>CNS and renal systems</p> Signup and view all the answers

    Which symptom is commonly associated with Sjögren syndrome?

    <p>Recurrent dental caries</p> Signup and view all the answers

    Which of the following treatments is first-line for lupus flares?

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

    Which autoantibodies are associated with extraglandular manifestations in Sjögren syndrome?

    <p>Anti-SSA/Ro antibodies</p> Signup and view all the answers

    What is a characteristic consequence of lupus anticoagulant in blood tests?

    <p>Falsely-elevated PTT</p> Signup and view all the answers

    What is the underlying pathology of Sjögren syndrome?

    <p>Lymphocyte-mediated damage</p> Signup and view all the answers

    What specific feature on lip biopsy can assist in diagnosing Sjögren's syndrome?

    <p>Lymphocytic sialadenitis</p> Signup and view all the answers

    Which of the following is a characteristic feature of diffuse scleroderma?

    <p>Early visceral organ involvement</p> Signup and view all the answers

    Which antibody is commonly associated with diffuse scleroderma?

    <p>Anti-Scl-70</p> Signup and view all the answers

    What is a potential risk associated with Sjögren's syndrome?

    <p>Increased risk for B-cell lymphoma</p> Signup and view all the answers

    In which demographic is systemic sclerosis classically seen?

    <p>Middle-aged females</p> Signup and view all the answers

    Which of the following symptoms is characteristic of CREST syndrome?

    <p>Esophageal dysmotility</p> Signup and view all the answers

    What mechanism primarily leads to fibrosis in systemic sclerosis?

    <p>Endothelial dysfunction and vascular damage</p> Signup and view all the answers

    What is a major clinical complication associated with scleroderma affecting the kidneys?

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

    Labile tissues, such as skin and bone marrow, are characterized by stem cells that cycle continuously to regenerate.

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

    Stable tissues are known to regenerate only under conditions of inflammation and cannot reenter the cell cycle otherwise.

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

    The process of wound healing incorporates both regeneration and repair mechanisms.

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

    Permanent tissues, such as neurons, have high regenerative capacity and can completely return to their original state after injury.

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

    Compensatory hyperplasia is the most significant mechanism for liver regeneration following partial resection.

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

    Anti-SSA and anti-SSB antibodies are exclusively found in patients with systemic sclerosis.

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

    Lymphocytic sialadenitis identified through lip biopsy is a key diagnostic criterion for Sjögren's syndrome.

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

    The diffuse type of systemic sclerosis affects only the skin with no early visceral involvement.

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

    CREST syndrome includes sclerodactyly as one of its defining features.

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

    Increased risk for B-cell marginal zone lymphoma associated with Sjögren's syndrome typically manifests as bilateral enlargement of the parotid gland.

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

    Anti-DNA topoisomerase I antibodies are highly associated with limited systemic sclerosis.

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

    Fibroblast activation in systemic sclerosis is a direct result of autoimmune damage to the epidermis.

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

    Sjögren's syndrome is classified as a mixed connective tissue disease with attributes of systemic sclerosis.

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

    Antiphospholipid antibody syndrome can lead to thrombosis in both arterial and venous systems.

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

    Antihistone antibodies are associated with primary Sjögren syndrome.

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

    Lifelong anticoagulation is necessary for individuals diagnosed with antiphospholipid antibody syndrome.

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

    Dry eyes and dry mouth in Sjögren syndrome result from the inflammatory destruction of the lacrimal and salivary glands.

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

    Pregnant women with anti-SSB/La antibodies have a higher risk of delivering infants with congenital heart block.

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

    Sjögren syndrome can be classified as primary or secondary, the latter occurring alongside rheumatoid arthritis.

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

    ANA positivity is required for the diagnosis of drug-induced lupus.

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

    Renal failure and infection are common causes of mortality in patients with lupus, but accelerated coronary atherosclerosis occurs early.

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

    Describe the role of labile tissues in regeneration and provide an example.

    <p>Labile tissues continuously regenerate via stem cells and an example is the skin, which has stem cells in its basal layer.</p> Signup and view all the answers

    Explain the difference between stable and permanent tissues in terms of their regenerative capacity.

    <p>Stable tissues can reenter the cell cycle when needed, while permanent tissues, like neurons, have a limited or no capacity for regeneration.</p> Signup and view all the answers

    What mechanisms underlie the compensatory hyperplasia observed in liver regeneration after partial resection?

    <p>Compensatory hyperplasia involves hepatocytes producing additional cells and then entering quiescence, effectively compensating for the lost liver mass.</p> Signup and view all the answers

    Identify the types of tissues and their regenerative capabilities as outlined in the classification system.

    <p>The three types are labile (continuous regeneration), stable (quiescent but can regenerate), and permanent (minimal regeneration).</p> Signup and view all the answers

    What initiates the wound healing process and what are the primary mechanisms involved?

    <p>Wound healing is initiated by inflammation and primarily occurs through a combination of regeneration and repair mechanisms.</p> Signup and view all the answers

    What role do anti-SSA and anti-SSB antibodies play in pregnant patients with Sjögren's syndrome?

    <p>They indicate an increased risk of delivering infants with congenital heart block.</p> Signup and view all the answers

    How does lymphocytic sialadenitis contribute to the diagnosis of Sjögren's syndrome?

    <p>It is observed on lip biopsy and serves as an additional diagnostic criterion.</p> Signup and view all the answers

    What distinguishes limited from diffuse systemic sclerosis in terms of skin involvement?

    <p>Limited systemic sclerosis has skin involvement confined to hands and face, while diffuse involves the entire skin early on.</p> Signup and view all the answers

    What is the typical demographic for patients with systemic sclerosis?

    <p>Systemic sclerosis predominantly affects middle-aged females between 30 and 50 years old.</p> Signup and view all the answers

    Which organs are commonly affected by diffuse systemic sclerosis?

    <p>Commonly affected organs include the lungs, kidneys, GI tract, and blood vessels.</p> Signup and view all the answers

    What specific antibody is highly associated with diffuse systemic sclerosis?

    <p>Anti-DNA topoisomerase I (anti-Scl-70) antibodies are highly associated.</p> Signup and view all the answers

    What is the role of lupus anticoagulant in the context of antiphospholipid antibody syndrome?

    <p>Lupus anticoagulant contributes to a hypercoagulable state, resulting in increased risk of thrombosis.</p> Signup and view all the answers

    How does drug-induced lupus differ from systemic lupus erythematosus (SLE) in terms of autoantibody profiles?

    <p>Drug-induced lupus is characterized by antihistone antibodies, while SLE typically shows a broader range of autoantibodies.</p> Signup and view all the answers

    What is a significant long-term risk associated with Sjögren's syndrome?

    <p>There is an increased risk of developing B-cell marginal zone lymphoma.</p> Signup and view all the answers

    What are the common clinical manifestations of Sjögren syndrome in patients?

    <p>Patients commonly experience dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).</p> Signup and view all the answers

    What are the hallmark features of CREST syndrome?

    <p>Hallmark features include Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias.</p> Signup and view all the answers

    Describe the significance of anti-SSA/Ro and anti-SSB/La antibodies in pregnant women with Sjögren syndrome.

    <p>These antibodies indicate an increased risk of neonatal lupus and congenital heart block in infants.</p> Signup and view all the answers

    What are the long-term treatment considerations for a patient diagnosed with antiphospholipid antibody syndrome?

    <p>Lifelong anticoagulation therapy is required to prevent recurrent thrombosis.</p> Signup and view all the answers

    What underlying immune mechanism is responsible for the symptoms associated with Sjögren syndrome?

    <p>The symptoms are due to lymphocyte-mediated damage and fibrosis of the lacrimal and salivary glands.</p> Signup and view all the answers

    How does the presence of rheumatoid factor affect the diagnosis of Sjögren syndrome?

    <p>Rheumatoid factor may be present even in the absence of rheumatoid arthritis, complicating the diagnosis.</p> Signup and view all the answers

    What is a potential cause of death in patients with lupus beyond renal failure and infection?

    <p>Accelerated coronary atherosclerosis is another common cause of death in lupus patients.</p> Signup and view all the answers

    Labile tissues, such as skin and bone marrow, possess ______ that continuously cycle to regenerate.

    <p>stem cells</p> Signup and view all the answers

    Stable tissues can reenter the cell cycle to regenerate when necessary, as seen in the ______ after partial resection.

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

    The process of wound healing includes regeneration via both ______ and repair.

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

    Permanent tissues, such as neurons, are characterized by their limited ______ capacity.

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

    Labile tissues are divided into three types based on regenerative capacity: labile, stable, and ______.

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

    Antiphospholipid antibody syndrome results in a ______ state due to antiphospholipid antibodies.

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

    Common causes of drug-induced lupus include procainamide, hydralazine, and ______.

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

    Sjögren syndrome classically presents as dry eyes (keratoconjunctivitis sicca) and dry ______.

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

    Sjögren syndrome can be classified as primary (sicca syndrome) or associated with another autoimmune disorder, especially ______.

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

    Pregnant women with anti-SSA antibodies are at risk for delivering babies with ______ lupus.

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

    First-line treatment for lupus flares includes avoiding exposure to direct sunlight and ______ for flares.

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

    Sjögren syndrome is characterized by the presence of ANA and anti-______ antibodies.

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

    Removal of the offending drug in drug-induced lupus usually results in ______.

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

    Anti-SSA and anti-SSB antibodies are seen in a subset of patients with ______.

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

    Lymphocytic sialadenitis can be confirmed through a ______ biopsy.

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

    Increased risk for B-cell marginal zone lymphoma is associated with Sjögren's syndrome and typically presents as unilateral enlargement of the ______ gland.

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

    Systemic sclerosis is characterized by sclerosis of ______ and visceral organs.

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

    Fibroblast activation in systemic sclerosis primarily leads to deposition of ______.

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

    CREST syndrome is associated with limited type systemic sclerosis, which includes features such as ______ phenomenon.

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

    The diffuse type of systemic sclerosis may involve any organ, commonly affecting the ______ and GI tract.

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

    Anti-DNA topoisomerase I antibodies are highly associated with ______ systemic sclerosis.

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

    Match the following types of tissues with their regenerative characteristics:

    <p>Labile tissues = Continuously cycling stem cells Stable tissues = Quiescent but can reenter cycle Permanent tissues = Limited to no regenerative capability Scar tissue = Replacement of tissue with fibrous connective tissue</p> Signup and view all the answers

    Match the following conditions with their associated laboratory findings:

    <p>Sjögren syndrome = ANA and anti-SSA/SSB positivity Systemic sclerosis = Anti-DNA topoisomerase I antibodies Lupus = Antihistone antibodies and ANAs Antiphospholipid antibody syndrome = Anticardiolipin antibodies and prolonged APTT</p> Signup and view all the answers

    Match the following types of tissue repair processes with their descriptions:

    <p>Regeneration = Replacement with native tissue Repair = Replacement with scar tissue Compensatory hyperplasia = Cell proliferation in response to partial loss Fibrosis = Pathological accumulation of fibrous connective tissue</p> Signup and view all the answers

    Match the following symptoms with the corresponding conditions:

    <p>Dry eyes and mouth = Sjögren syndrome Raynaud's phenomenon = Systemic sclerosis Skin tightening = Diffuse systemic sclerosis Bilateral parotid gland enlargement = Sjögren syndrome</p> Signup and view all the answers

    Match the following regenerative processes with their organ/issue examples:

    <p>Labile tissue regeneration = Skin and bone marrow Stable tissue regeneration = Liver after partial resection Permanent tissue incapacity = Neurons and cardiac muscle Quiescent tissue reentry = Hepatocyte hyperplasia</p> Signup and view all the answers

    Match each clinical feature with its associated condition:

    <p>Lymphocytic sialadenitis = Sjögren's syndrome Calcinosis = CREST syndrome Raynaud phenomenon = Diffuse systemic sclerosis B-cell marginal zone lymphoma = Sjögren's syndrome</p> Signup and view all the answers

    Match the type of scleroderma with its characteristic features:

    <p>Limited type = Skin involvement limited to hands and face Diffuse type = Early visceral involvement CREST syndrome = Anti-Centromere antibodies Sclerodactyly = Skin thickening of fingers</p> Signup and view all the answers

    Match the types of antibodies with their associated conditions:

    <p>Anti-SSA = Associated with Sjögren's syndrome Anti-SSB = Increased risk of congenital heart block Anti-Scl-70 = Common in diffuse systemic sclerosis Anti-Centromere = Common in limited systemic sclerosis</p> Signup and view all the answers

    Match the organ systems commonly affected in diffuse scleroderma:

    <p>Lungs = Pulmonary hypertension Kidneys = Scleroderma renal crisis GI tract = Esophageal dysmotility Skin = Diffused thickening and tightness</p> Signup and view all the answers

    Match the patient demographic with the condition it's commonly associated with:

    <p>Middle-aged females = Systemic sclerosis Pregnant women with anti-SSB antibodies = Risk for congenital heart block Patients with scleroderma = Increased risk for B-cell lymphoma Patients with Sjögren's syndrome = Possible overlap with RA</p> Signup and view all the answers

    Match each growth factor with its role in systemic sclerosis:

    <p>TGF-β = Stimulates collagen deposition PDGF = Promotes fibroblast activation Endothelin = Causes vasoconstriction NO (Nitric Oxide) = Regulates blood vessel dilation</p> Signup and view all the answers

    Match each feature with the system involved in systemic sclerosis:

    <p>Raynaud phenomenon = Vascular system Esophageal dysmotility = Gastrointestinal system Interstitial fibrosis = Pulmonary system Sclerodactyly = Integumentary system</p> Signup and view all the answers

    Match the following autoimmune conditions with their composite features:

    <p>SLE = Lupus nephritis and skin rash Mixed connective tissue disease = Features of SLE, scleroderma, and polymyositis Sjögren's syndrome = Dry eyes and dry mouth Polymyositis = Muscle weakness and inflammation</p> Signup and view all the answers

    Match the following antiphospholipid antibodies with their characteristics:

    <p>Anticardiolipin = False-positive VDRL and RPR syphilis tests Lupus anticoagulant = Falsely elevated PTT Anti-β2-glycoprotein I = IgG or IgM associated with thrombosis Antiphospholipid antibody syndrome = Hypercoagulable state</p> Signup and view all the answers

    Match the following features with their corresponding conditions:

    <p>Sjögren syndrome = Autoimmune destruction of lacrimal and salivary glands Drug-induced lupus = Antihistone antibodies present Lupus = Requires lifelong anticoagulation Anti-SSA/Ro antibodies = Risk of neonatal lupus in pregnancy</p> Signup and view all the answers

    Match the following symptoms with their associated conditions:

    <p>Keratoconjunctivitis sicca = Dry eyes in Sjögren syndrome Xerostomia = Dry mouth in Sjögren syndrome Recurrent dental caries = Common in older women with Sjögren syndrome Arterial thrombosis = Complication of antiphospholipid syndrome</p> Signup and view all the answers

    Match the following drugs with their known associations in drug-induced lupus:

    <p>Procainamide = Commonly causes antihistone antibodies Hydralazine = Known for inducing drug-induced lupus Isoniazid = Associated with lupus-like symptoms Glucocorticoids = First-line treatment for lupus flares</p> Signup and view all the answers

    Match the following autoantibodies with their related disease manifestations:

    <p>Anti-SSA = Associated with neonatal lupus Anti-SSB = Congenital heart block risk Rheumatoid factor = Often present in Sjögren syndrome ANA = Positive by definition in drug-induced lupus</p> Signup and view all the answers

    Match the following potential complications with their respective autoimmune conditions:

    <p>Lupus = Renal failure and infections common Sjögren syndrome = Risk for B-cell lymphoma Antiphospholipid syndrome = Deep venous thrombosis risk Drug-induced lupus = Rare CNS and renal involvement</p> Signup and view all the answers

    Match the following descriptions with their corresponding antibodies:

    <p>Anticardiolipin antibodies = Associated with false-positive syphilis tests Lupus anticoagulant = Causes false elevation in PTT tests Anti-SSA/Ro antibodies = Linked to extraglandular manifestations Anti-ribonucleoprotein antibodies = Characteristic in Sjögren syndrome</p> Signup and view all the answers

    Match the following conditions with their clinical implications:

    <p>Sjögren syndrome = May progress to corneal ulceration Antiphospholipid syndrome = Requires continual anticoagulation Drug-induced lupus = Can remit upon drug removal Anti-β2-glycoprotein I = Associated with arterial and venous thrombi</p> Signup and view all the answers

    Study Notes

    Inflammation, Inflammatory Disorders, and Wound Healing

    • Inflammation is a protective response to tissue injury
    • Inflammation involves the following:
      • Vascular changes: dilation, increased permeability
      • Cellular events: leukocyte recruitment and activation
    • Cardinal signs of inflammation:
      • Rubor (redness)
      • Calor (heat)
      • Tumor (swelling)
      • Dolor (pain)
      • Functio laesa (loss of function)

    Systemic Inflammatory Response Syndrome (SIRS)

    • Characterized by widespread inflammation, often triggered by infection or trauma
    • Clinical criteria:
      • Temperature > 38°C or < 36°C
      • Heart rate > 90 bpm
      • Respiratory rate > 20 breaths/min or PaCO2 < 32 mmHg
      • White blood cell count > 12,000/mm3 or < 4,000/mm3 or > 10% immature bands

    Autoimmune Diseases

    • Characterized by immune system attacking self-antigens
    • Result in chronic inflammation and tissue damage

    Systemic Lupus Erythematosus (SLE)

    • Characterized by autoantibodies against nuclear antigens (ANA)
    • Clinical features:
      • Malar rash
      • Discoid rash
      • Photosensitivity
      • Oral ulcers
      • Arthritis
      • Serositis (pleuritis, pericarditis)
      • Renal involvement (glomerulonephritis)
      • Neurological disorders (seizures, psychosis)
      • Hematological abnormalities (anemia, leukopenia, thrombocytopenia)

    Antiphospholipid Antibody Syndrome

    • Associated with hypercoagulable state due to antiphospholipid antibodies
    • Results in arterial and venous thrombosis, including deep venous, hepatic vein, placental, and cerebral thrombosis
    • Requires lifelong anticoagulation
    • Can be primary or associated with SLE

    Antihistone Antibody

    • Characteristic of drug-induced lupus
    • Common causes: procainamide, hydralazine, and isoniazid
    • Removal of the drug usually results in remission

    Sjögren Syndrome

    • Autoimmune destruction of lacrimal and salivary glands
    • Presents as dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), and recurrent dental caries
    • Can be primary or associated with other autoimmune disorders, especially rheumatoid arthritis
    • Characterized by ANA and anti-ribonucleoprotein antibodies (anti-SSA/Ro and anti-SSB/La)
    • Increased risk for B-cell lymphoma

    Systemic Sclerosis (Scleroderma)

    • Autoimmune disorder with fibrosis of skin and visceral organs
    • Prototype is CREST syndrome:
      • Calcinosis
      • Raynaud phenomenon
      • Esophageal dysmotility
      • Sclerodactyly
      • Telangiectasias

    Mixed Connective Tissue Disease

    • Autoimmune mediated tissue damage with mixed features of SLE, systemic sclerosis, and polymyositis
    • Characterized by ANA along with serum antibodies to U1 ribonucleoprotein

    Wound Healing

    • Healing process initiated by inflammation
    • Involves combination of regeneration and repair

    Regeneration

    • Replacement of damaged tissue with native tissue
    • Tissues classified based on regenerative capacity:
      • Labile (continuous regeneration)
      • Stable (regenerate when necessary)
      • Permanent (cannot regenerate)

    ### Inflammation, Inflammatory Disorders, and Wound Healing

    • Inflammation is a protective response to tissue injury that involves vascular and cellular changes
    • Components of inflammation include redness, warmth, swelling, pain, and loss of function caused by vasodilation, increased vascular permeability, and leukocyte infiltration
    • Chronic inflammation can be a significant factor in many diseases, such as atherosclerosis, cancer, and arthritis
    • Autoimmune diseases result from a failure of the immune system to distinguish self from non-self, leading to the production of antibodies directed against self-antigens
    • Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by circulating autoantibodies against nuclear antigens, particularly antinuclear antibodies (ANA)

    Systemic Lupus Erythematosus (SLE)

    • Affects primarily women (9:1 ratio with men) in the childbearing age
    • Most common symptoms are arthritis, fever, rash, and fatigue
    • Malar rash is a hallmark of the disease, characterized by a butterfly-shaped rash across the cheeks and nose
    • Discoid rash is characterized by red, scaly patches on the skin that can lead to scarring
    • Photosensitivity can be a symptom, exacerbating the rash with sun exposure
    • Oral ulcers are common, appearing as painless sores on the lining of the mouth
    • Serositis commonly involves the pleura and pericardium, causing pleuritis and pericarditis.
    • Renal issues can occur, with glomerulonephritis being a severe manifestation of the disease
    • Neurological involvement can lead to seizures, psychosis, and cognitive dysfunction
    • Hematologic abnormalities can be present with anemia, thrombocytopenia, and leukopenia.
    • Anti-double-stranded (ds) DNA antibodies are very specific for SLE

    ### Antiphospholipid Antibody Syndrome

    • Characterized by a hypercoagulable state due to antiphospholipid antibodies
    • Anticardiolipin antibodies are commonly found, leading to false-positive syphilis screening tests (VDRL and RPR)
    • Anti-β2-glycoprotein I is another common antibody
    • Lupus anticoagulant is a false-positive test for prolonged PTT
    • Results in both arterial and venous thrombosis, including deep vein thrombosis, hepatic vein thrombosis, placental thrombosis (recurrent pregnancy loss), and cerebral thrombosis (stroke)
    • Requires lifelong anticoagulation
    • Often associated with SLE but also frequently occurs as a primary disorder

    ###  Sjögren's Syndrome

    • Autoimmune destruction of lacrimal and salivary glands
    • Classic presentation: dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), and recurrent dental caries; typically affecting women between 50-60 years
    • Can progress to ulceration of the corneal epithelium and oral mucosa
    • Can occur as a primary disorder (sicca syndrome) or be associated with other autoimmune disorders, especially rheumatoid arthritis
    • Rheumatoid factor is frequently present even in the absence of rheumatoid arthritis
    • Characterized by ANA and anti-ribonucleoprotein antibodies (anti-SSA/Ro and anti-SSB/La)
    • Anti-SSA and anti-SSB can cause extraglandular manifestations like neuropathy and are associated with a risk of delivering babies with neonatal lupus and congenital heart block in pregnant women
    • Lymphocytic sialadenitis on lip biopsy is a diagnostic criterion
    • Increased risk for B-cell lymphoma (marginal zone) presenting as unilateral enlargement of the parotid gland late in disease course

    Systemic Sclerosis (Scleroderma)

    • Autoimmune disorder characterized by sclerosis of skin and visceral organs
    • Typically affects middle-aged females (30-50 years)
    • Fibroblast activation leads to collagen deposition
    • Autoimmune damage to mesenchyme is the potential initiating event
    • Endothelial dysfunction leads to inflammation (increased adhesion molecules), vasoconstriction (increased endothelin and decreased NO), and secretion of growth factors (TGF-β and PDGF)
    • Fibrosis, initially perivascular, progresses and causes organ damage

    Limited Type Systemic Sclerosis

    • Skin involvement is limited (hands and face) with late visceral involvement
    • CREST syndrome is a prototype: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias
    • Anti-centromere antibodies are commonly present

    Diffuse Type Systemic Sclerosis

    • Skin involvement is diffuse with early visceral involvement
    • Any organ can be affected
    • Common organs involved include vessels (Raynaud phenomenon), GI tract (esophageal dysmotility and reflux), lungs (interstitial fibrosis and pulmonary hypertension), and kidneys (scleroderma renal crisis)
    • Highly associated with antibodies to DNA topoisomerase I (anti-Scl-70)

    Mixed Connective Tissue Disease

    • Autoimmune-mediated tissue damage with mixed features of SLE, systemic sclerosis, and polymyositis
    • Characterized by ANA and serum antibodies to U1 ribonucleoprotein

    Wound Healing

    • Healing is initiated with the onset of inflammation
    • Involves both regeneration and repair

    Regeneration

    • Replacement of damaged tissue with native tissue, dependent on the regenerative capacity of the tissue
    • Tissues categorized into three types based on regenerative capacity: labile, stable, and permanent
    • Labile tissues possess stem cells that continuously cycle to regenerate the tissue (e.g., small and large bowel, skin, bone marrow)
    • Stable tissues are comprised of quiescent cells (G0) that can reenter the cell cycle to regenerate when needed (e.g., liver regeneration after partial resection)
    • Permanent tissues lack the ability to regenerate and are replaced by fibrous scar tissue (e.g., cardiac muscle, neurons).

    ### Inflammation, Inflammatory Disorders, and Wound Healing

    • Antiphospholipid Antibody Syndrome
      • Characterized by a hypercoagulable state due to antiphospholipid antibodies, especially lupus anticoagulant
      • Results in arterial and venous thrombosis, including deep venous, hepatic vein, placental (recurrent pregnancy loss), and cerebral (stroke).
      • Lifelong anticoagulation is necessary.
      • Associated with Systemic Lupus Erythematosus (SLE); however, it more commonly occurs as a primary disorder.
    • Drug-Induced Lupus
      • Characterized by antihistone antibodies.
      • Common causes include procainamide, hydralazine, and isoniazid.
      • ANA is positive.
      • CNS and renal involvement are rare.
      • Removal of the drug usually results in remission.

    ### Sjögren Syndrome

    • Autoimmune destruction of lacrimal and salivary glands - lymphocyte-mediated damage (type IV HSR) with fibrosis.
    • Classically presents as dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), and recurrent dental caries in an older woman (50-60 years).
    • May progress to ulceration of the corneal epithelium and oral mucosa.
    • Can be primary (sicca syndrome) or associated with another autoimmune disorder, especially rheumatoid arthritis.
    • Rheumatoid factor is often present even when rheumatoid arthritis is absent.
    • Characterized by ANA and anti-ribonucleoprotein antibodies (anti-SSA/Ro and anti-SSB/La).
    • Anti-SSA and anti-SSB are associated with extraglandular manifestations (e.g., neuropathy).
    • Pregnant women with anti-SSA are at risk for delivering babies with neonatal lupus and congenital heart block.
    • Lymphocytic sialadenitis on lip biopsy (minor salivary glands) is an additional diagnostic criterion.
    • Increased risk for B-cell (marginal zone) lymphoma, which presents as unilateral enlargement of the parotid gland late in disease course.

    Systemic Sclerosis (Scleroderma)

    • Autoimmune disorder characterized by sclerosis of skin and visceral organs.
    • Classically presents in middle-aged females (30-50 years).
    • Fibroblast activation leads to deposition of collagen.
    • Autoimmune damage to mesenchyme is a possible initiating event.
    • Endothelial dysfunction leads to inflammation, vasoconstriction, and secretion of growth factors.
    • Fibrosis progresses and causes organ damage.
    • Limited type:
      • Skin involvement is limited (hands and face) with late visceral involvement.
      • Prototype is CREST syndrome: Calcinosis/anti-Centromere antibodies, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias of the skin.
    • Diffuse type:
      • Skin involvement is diffuse with early visceral involvement.
      • Any organ can be involved.
      • Commonly involved organs: vessels (Raynaud phenomenon), GI tract (esophageal dysmotility and reflux), lungs (interstitial fibrosis and pulmonary hypertension), kidneys (scleroderma renal crisis).
      • Highly associated with antibodies to DNA topoisomerase I (anti-Scl-70).

    Mixed Connective Tissue Disease

    • Autoimmune-mediated tissue damage with mixed features of SLE, systemic sclerosis, and polymyositis.
    • Characterized by ANA along with serum antibodies to U1 ribonucleoprotein.

    Wound Healing

    • Basic Principles
      • Healing is initiated when inflammation begins.
      • Occurs via a combination of regeneration and repair.
    • Regeneration
      • Replacement of damaged tissue with native tissue.
      • Dependent on regenerative capacity of tissue.
      • Tissues are divided into three types based on regenerative capacity: labile, stable, and permanent.
      • Labile tissues:
        • Possess stem cells that continuously cycle to regenerate the tissue.
        • Examples: small and large bowel, skin, bone marrow.
      • Stable tissues:
        • Comprised of cells that are quiescent (G0), but can reenter the cell cycle to regenerate tissue when necessary.
        • Example: regeneration of liver by compensatory hyperplasia after partial resection.

    Inflammation, Inflammatory Disorders, and Wound Healing

    • Antiphospholipid antibody syndrome is characterized by a hypercoagulable state due to antibodies against phospholipids.

      • This results in arterial and venous thrombosis, including deep venous thrombosis, hepatic vein thrombosis, placental thrombosis (recurrent pregnancy loss), and cerebral thrombosis (stroke).
      • Lifelong anticoagulation is required.
      • While associated with SLE, it's more commonly a primary disorder.

    • Antihistone antibody is associated with drug-induced lupus.

      • Common causes include procainamide, hydralazine, and isoniazid.
      • CNS and renal involvement are rare.
      • Removal of the drug usually leads to remission.

    Sjögren Syndrome

    • Autoimmune destruction of lacrimal and salivary glands by lymphocyte-mediated damage (type IV HSR) with fibrosis.
    • Presents classically as dry eyes and dry mouth, often with recurrent dental caries in older women (50-60 years).
    • Can be primary (sicca syndrome) or associated with other autoimmune disorders, particularly rheumatoid arthritis.
    • Characterized by ANA and anti-ribonucleoprotein antibodies (anti-SSA/Ro and anti-SSB/La).
      • Anti-SSA and anti-SSB are associated with extraglandular manifestations such as neuropathy.
      • Pregnant women with anti-SSA have a risk of delivering babies with neonatal lupus and congenital heart block.
      • Also found in some SLE patients.
    • Lymphocytic sialadenitis on lip biopsy (minor salivary glands) is a diagnostic criterion.
    • Patients are at increased risk for B-cell (marginal zone) lymphoma.

    Systemic Sclerosis (Scleroderma)

    • Autoimmune disorder with sclerosis of skin and visceral organs.
    • Presents classically in middle-aged females (30-50 years).
    • Fibroblast activation leads to collagen deposition.
      • Autoimmune damage to mesenchyme is a possible initiating event.
      • Endothelial dysfunction leads to inflammation, vasoconstriction, and secretion of growth factors.
      • Fibrosis initially perivascular, progresses causing organ damage.
    • Limited type has limited skin involvement (hands and face) with late visceral involvement.
      • CREST syndrome: Calcinosis, anti-Centromere antibodies, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias.
    • Diffuse type has diffuse skin involvement with early visceral involvement.
      • Can involve any organ but commonly affects vessels, the GI tract, lungs, and kidneys.
      • Highly associated with antibodies to DNA topoisomerase I (anti-Scl-70).

    Mixed Connective Tissue Disease

    • Autoimmune-mediated tissue damage with features of SLE, systemic sclerosis, and polymyositis.
    • Characterized by ANA and serum antibodies to U1 ribonucleoprotein.

    Wound Healing: Basic Principles

    • Healing is initiated when inflammation begins.
    • Occurs via a combination of regeneration and repair.

    Regeneration

    • Replacement of damaged tissue with native tissue.
    • Dependent on the regenerative capacity of the tissue.
    • Tissues are divided into three types based on regenerative capacity: labile, stable, and permanent.
    • Labile tissues: Possess stem cells that continuously cycle to regenerate the tissue.
      • Small and large bowel (stem cells in mucosal crypts).
      • Skin (stem cells in basal layer).
      • Bone marrow (hematopoietic stem cells).
    • Stable tissues: Are comprised of quiescent cells that can reenter the cell cycle to regenerate tissue when necessary.
      • Classic example is regeneration of the liver by compensatory hyperplasia after partial resection.

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    Pathoma 2023 PDF Inflammation & Inflammatory Disorders

    Description

    Test your knowledge on inflammation, its role in wound healing, and the mechanisms underlying autoimmune diseases like Systemic Lupus Erythematosus. This quiz covers essential concepts from the body's inflammatory response, systemic inflammatory response syndrome, and the effects of chronic inflammation. Perfect for medical students and healthcare professionals!

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