Oncology and Rheumatology Connective Tissue Diseases III Quiz
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Questions and Answers

What is the primary cause of Raynaud's phenomenon?

  • Restricted blood flow (correct)
  • Increased blood flow
  • Nerve damage
  • Blood vessel inflammation

    • Which of the following is a characteristic clinical manifestation of dermatomyositis?

  • Decreased skin pigmentation
  • Muscle weakness (correct)
  • Increased appetite
  • Increased joint mobility

    • Which of the following is a type of systemic vasculitis?

  • Systemic lupus erythematosus
  • Giant cell arteritis (correct)
  • Rheumatoid arthritis
  • Sjögren's syndrome

    • What is the primary cause of the skin turning blue in Raynaud's phenomenon?

    <p>Blood vessel spasm</p> Signup and view all the answers

    Which of the following is a key feature of the pathology of systemic vasculitides?

    <p>Inflammation of blood vessels</p> Signup and view all the answers

    What is the most common autoantibody associated with dermatomyositis?

    <p>Anti-Mi2 antibodies</p> Signup and view all the answers

    Which of the following is NOT a common systemic symptom of dermatomyositis?

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

    What type of immune cells are involved in the pathogenesis of dermatomyositis?

    <p>CD4+ T-cells and B-cells</p> Signup and view all the answers

    What is the key difference between dermatomyositis and polymyositis?

    <p>Dermatomyositis has a rash, while polymyositis does not.</p> Signup and view all the answers

    Which of the following is a common manifestation of dermatomyositis related to the joints?

    <p>Arthralgia and deforming arthropathy</p> Signup and view all the answers

    What is the significance of anti-P155/P140 antibodies in dermatomyositis?

    <p>They are associated with a higher risk of malignancy.</p> Signup and view all the answers

    What is the cause of polymyositis in adults according to the text?

    <p>Autoimmune factors</p> Signup and view all the answers

    Which type of T cells are thought to directly injure myocytes in polymyositis?

    <p>CD8+ cytotoxic T cells</p> Signup and view all the answers

    In polymyositis, where are CD8+ T cells mainly located?

    <p>In endomysium</p> Signup and view all the answers

    What is a common factor leading to delayed medical advice seeking in patients with DM & PM?

    <p>Presence of skin rash</p> Signup and view all the answers

    How are systemic vasculitides classified based on according to the text?

    <p>By the size of the vessels involved</p> Signup and view all the answers

    Which type of cells are typically found in the inflammatory infiltrate of Kawasaki disease?

    <p>Neutrophils, eosinophils, and mononuclear cells</p> Signup and view all the answers

    Which of the following is a common complication of Kawasaki disease if left untreated?

    <p>Aneurysm or myocardial infarction</p> Signup and view all the answers

    What is the primary cause of Buerger's disease/thromboangiitis obliterans?

    <p>Clots in vessels supplying fingers and toes</p> Signup and view all the answers

    What is the primary risk factor for developing Buerger's disease?

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

    Which of the following is a characteristic histological feature of the small vessel vasculitides?

    <p>Fibrinoid necrosis of the vessel wall</p> Signup and view all the answers

    Which type of ANCA is typically associated with granulomatosis with polyangiitis (Wegener's)?

    <p>Proteinase 3 (PR3) ANCA</p> Signup and view all the answers

    Which of the following is a characteristic clinical feature of granulomatosis with polyangiitis (Wegener's)?

    <p>Chronic sinusitis and nasal ulcers</p> Signup and view all the answers

    Which of the following is a key histological feature of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?

    <p>Necrotizing vasculitis</p> Signup and view all the answers

    Which of the following is a common clinical manifestation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?

    <p>Pulmonary infiltrates and asthma</p> Signup and view all the answers

    Which of the following is a common treatment approach for the small vessel vasculitides?

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

    Which of the following is a characteristic clinical manifestation of Granulomatosis with Polyangiitis (GPA)?

    <p>Granulomatous vasculitis and positive p-ANCA (MPO-ANCA)</p> Signup and view all the answers

    Which of the following is a key feature of Henoch-Schönlein Purpura (HSP)?

    <p>IgA antibodies and immune complex-mediated small vessel vasculitis</p> Signup and view all the answers

    Which of the following is a common clinical presentation of Henoch-Schönlein Purpura (HSP)?

    <p>Skin lesions on the buttocks and legs, progressing from macules to petechiae to palpable purpura</p> Signup and view all the answers

    Which of the following is a characteristic of Granulomatosis with Polyangiitis (GPA)?

    <p>Granulomatous vasculitis and positive p-ANCA (MPO-ANCA)</p> Signup and view all the answers

    Which of the following is a common treatment approach for Granulomatosis with Polyangiitis (GPA)?

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

    Which of the following is a characteristic of Henoch-Schönlein Purpura (HSP)?

    <p>ANCA negativity and IgA antibody involvement</p> Signup and view all the answers

    Study Notes

    Inflammatory Myopathies

    • Dermatomyositis (DM) and Polymyositis (PM) are inflammatory myopathies
    • DM:
      • Characterized by skin manifestations, including mechanic's hand, Gottron's papules, and heliotrope rash
      • May also have systemic symptoms such as fever, malaise, weight loss, and Raynaud's phenomenon
      • Can lead to malignant tumors in adults
      • Autoantibodies: ANA, anti-Mi2, anti-helicase, anti-Jo1, and anti-P155/P140
    • PM:
      • Characterized by bilateral proximal muscle weakness, similar to DM but without a rash
      • Cause is unknown, may involve viruses and autoimmune factors
      • Different pathogenesis: CD8+ cytotoxic T cells probably directly injuring myocytes
      • Anti-Jo1 in 20% of patients, poor prognosis with interstitial lung disease

    Systemic Vasculitides

    • Classification based on the size of the vessels involved
    • Large vessel vasculitis:
      • Takayasu arteritis
    • Medium vessel vasculitis:
      • Kawasaki disease
      • Buerger's disease/thromboangiitis obliterans
    • Small vessel vasculitis:
      • ANCA-associated vasculitides:
        • Microscopic polyangiitis
        • Granulomatosis with polyangiitis (Wegener's granulomatosis)
        • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
      • IgA vasculitis (Henoch-Schonlein purpura)

    Kawasaki Disease

    • Mucocutaneous lymph node syndrome
    • Acute febrile illness in infants and children (< 4 years)
    • Mucocutaneous symptoms and cervical lymph node enlargement
    • Treatment: IVIG

    Buerger's Disease/Thromboangiitis Obliterans

    • Affects medium and small vessels
    • Heavy smokers, males, 20-40 years
    • Pathogenesis: Clots in vessels supplying fingers and toes
    • Patients develop ulcers on fingers and toes, often co-exists with Raynaud's phenomenon
    • Treatment: Smoking cessation

    Small Vessel Vasculitides

    • Microscopic polyangiitis:
      • Leukocytoclastic or hypersensitivity vasculitis
      • Transmural, necrotizing vasculitis
      • Organs involved: skin, mucous membranes, lungs, GI, kidneys, heart, and brain
      • Treatment: glucocorticoids, cyclophosphamide
    • Granulomatosis with polyangiitis/Wegener's granulomatosis:
      • Classical clinical triad: kidney, nasopharynx, and lungs
      • Biopsy: focal necrotizing vasculitis, granulomatous change, and PR3-ANCA positive
      • Rapidly fatal if left untreated
      • Treatment: glucocorticoids, cyclophosphamide
    • Eosinophilic granulomatosis with polyangiitis/Churg-Strauss syndrome:
      • Necrotizing vasculitis, granulomatous vasculitis
      • Strongly associated with asthma, eosinophilia, and pulmonary infiltrates
      • Supportive therapy and glucocorticoids
    • Henoch-Schonlein purpura:
      • IgA antibodies directly target endothelial cells
      • Small vessel IgA and immune complex-mediated vasculitis
      • Presentation: skin lesions on buttocks and leg, progressing from blanching macules to petechiae to palpable purpura
      • Diagnosis: clinical, as lab results often normal
      • Treatment: supportive therapy, +/- corticosteroids for GI symptoms

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    Connective Tissue Diseases III (Inflammatory Myopathies & Systemic Vasculitides) 2024 PDF

    Description

    Test your knowledge on inflammatory myopathies, systemic vasculitides, and Raynaud's phenomenon. Learn about the changes in skin color due to restricted blood flow and vascular reactions.

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