SYMPOSIUM 3: Systemic Inflammatory Disease (MTz, SS, CT)
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Questions and Answers

Which term is also used to describe C1 inhibitor deficiency?

  • Recurrent attacks
  • Hereditary angioedema (correct)
  • Fatal airway swelling
  • Cutaneous and submucosal swelling

    • What is the prevalence of C1 inhibitor deficiency?

  • 1:100,000 (correct)
  • 1:50,000
  • 1:25,000
  • 1:200,000

    • Who first described C1 inhibitor deficiency?

  • Michael D Tarzi
  • William Osler (correct)
  • 24 year old woman
  • Immunology Lecturer

    • What are the key clinical features of C1 inhibitor deficiency?

    <p>Recurrent attacks of cutaneous and submucosal swelling</p> Signup and view all the answers

    What is the acute treatment of choice for C1 inhibitor deficiency?

    <p>Intravenous C1 inhibitor concentrate</p> Signup and view all the answers

    What is the potential consequence of C1 inhibitor deficiency?

    <p>Potential for fatal airway swelling</p> Signup and view all the answers

    What is the role of C1 inhibitor concentrate in the treatment of C1 inhibitor deficiency?

    <p>Acute treatment</p> Signup and view all the answers

    What is the pathophysiology of C1 inhibitor deficiency?

    <p>Deficiency of C1 inhibitor</p> Signup and view all the answers

    Who should be tested for C1 inhibitor deficiency?

    <p>Those with recurrent attacks of swelling</p> Signup and view all the answers

    How can C1 inhibitor deficiency be diagnosed?

    <p>Blood test</p> Signup and view all the answers

    Which of the following is NOT a common feature of angioedema?

    <p>Delayed onset after a trigger</p> Signup and view all the answers

    What is one of the causes of spontaneous angioedema?

    <p>C1 inhibitor deficiency</p> Signup and view all the answers

    What is the inheritance pattern of C1 inhibitor deficiency?

    <p>Autosomal dominant</p> Signup and view all the answers

    What is the major negative regulator of the classical complement pathway?

    <p>C1 inhibitor</p> Signup and view all the answers

    Which component of the complement system binds to antibody constant regions?

    <p>C1q</p> Signup and view all the answers

    What is the role of C1 inhibitor in the classical complement pathway?

    <p>To make C1r and C1s dissociate from C1q</p> Signup and view all the answers

    Which type of HAE is characterized by deletions or missense mutations in the C1 inhibitor gene?

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

    What should be checked if serum C4 levels are found to be very low?

    <p>C1 inhibitor protein levels</p> Signup and view all the answers

    What is the typical cost per treated attack of angioedema?

    <p>£800 to £1200</p> Signup and view all the answers

    How does having a genetic condition like C1 inhibitor deficiency affect one's relationship with potential partners?

    <p>It may cause concerns about the risk of passing on the condition</p> Signup and view all the answers

    Which cells are enriched in the synovium of patients with rheumatoid arthritis?

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

    Which cells are key effectors in rheumatoid arthritis, responsible for phagocytosis and cytokine production?

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

    What is the suggested initiating cause of rheumatoid arthritis?

    <p>Bacterial infections</p> Signup and view all the answers

    What is the composition of synovial tissue in rheumatoid arthritis?

    <p>Macrophages, T lymphocytes, and B lymphocytes</p> Signup and view all the answers

    What is the role of cytokines in rheumatoid arthritis?

    <p>Activate synovial fibroblasts and osteoclasts</p> Signup and view all the answers

    What is the characteristic feature of joint destruction in rheumatoid arthritis?

    <p>Cartilage erosion</p> Signup and view all the answers

    What is the suggested role of Th17 cells in rheumatoid arthritis?

    <p>Activate synovial fibroblasts and osteoclasts</p> Signup and view all the answers

    What cells are responsible for the production of auto-antibodies associated with rheumatoid arthritis?

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

    What is the suggested genetic risk factor for rheumatoid arthritis?

    <p>HLA-DRB1 alleles</p> Signup and view all the answers

    What is the characteristic feature of synovial tissue in rheumatoid arthritis?

    <p>Synovium hyperplasia</p> Signup and view all the answers

    Which of the following is a clinical marker of rheumatoid arthritis (RA)?

    <p>All of the above</p> Signup and view all the answers

    What is the specificity of anti-cyclic citrullinated peptide (CCP) antibodies in diagnosing RA?

    <p>98%</p> Signup and view all the answers

    What is the role of anti-CCP antibodies in the pathogenesis of RA?

    <p>They enhance the development and severity of inflammation in the presence of mild synovitis</p> Signup and view all the answers

    Which process is responsible for the detection of citrullinated self proteins in RA patients by anti-CCP antibodies?

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

    Which of the following regions in the body is NOT commonly affected by RA?

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

    What percentage of patients with RA have rheumatoid factor (RF)?

    <p>60-70%</p> Signup and view all the answers

    What is the incidence of RA in North America?

    <p>0.9-1.1 cases per 100 inhabitants</p> Signup and view all the answers

    What is the concordance rate of RA in monozygotic twins?

    <p>12-15%</p> Signup and view all the answers

    Which gene accounts for 30-50% of the overall genetic risk of RA?

    <p>HLA-DRB1</p> Signup and view all the answers

    What is one of the environmental factors associated with an increased risk of RA?

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

    Which of the following best describes rheumatoid arthritis (RA)?

    <p>RA is an autoimmune disease characterized by chronic inflammation of the synovial joints.</p> Signup and view all the answers

    What is the prevalence of rheumatoid arthritis worldwide?

    <p>1 in 100 people</p> Signup and view all the answers

    Which of the following antibodies is often present in individuals with rheumatoid arthritis?

    <p>Both Rheumatoid factor (RF) and Cyclic citrullinated peptide (anti-CCP) antibodies</p> Signup and view all the answers

    What are the characteristic symptoms of rheumatoid arthritis?

    <p>Morning stiffness, swelling, heat, redness, and pain</p> Signup and view all the answers

    What is the impact of rheumatoid arthritis on mortality and life expectancy?

    <p>Rheumatoid arthritis increases mortality and decreases life expectancy.</p> Signup and view all the answers

    What is the goal of treatment for rheumatoid arthritis?

    <p>Restoration of natural fluctuations in inflammation</p> Signup and view all the answers

    What is the role of cytokines in rheumatoid arthritis?

    <p>Cytokines cause bone erosion and cartilage damage.</p> Signup and view all the answers

    What is the difference between healthy inflammation and pathological inflammation?

    <p>Healthy inflammation is acute and regulated, while pathological inflammation is chronic and unregulated.</p> Signup and view all the answers

    What is the impact of chronic inflammation on tissue?

    <p>Chronic inflammation causes tissue damage.</p> Signup and view all the answers

    What is the term used to describe immune responses to self-antigens in rheumatoid arthritis?

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

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