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 (A)</p> Signup and view all the answers

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

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

What is the potential consequence of C1 inhibitor deficiency?

<p>Potential for fatal airway swelling (D)</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 (A)</p> Signup and view all the answers

What is the pathophysiology of C1 inhibitor deficiency?

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

Who should be tested for C1 inhibitor deficiency?

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

How can C1 inhibitor deficiency be diagnosed?

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

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

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

What is one of the causes of spontaneous angioedema?

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

What is the inheritance pattern of C1 inhibitor deficiency?

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

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

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

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

<p>C1q (B)</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 (A)</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 (C)</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 (D)</p> Signup and view all the answers

What is the typical cost per treated attack of angioedema?

<p>£800 to £1200 (D)</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 (B)</p> Signup and view all the answers

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

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

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

<p>Macrophages (D)</p> Signup and view all the answers

What is the suggested initiating cause of rheumatoid arthritis?

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

What is the composition of synovial tissue in rheumatoid arthritis?

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

What is the role of cytokines in rheumatoid arthritis?

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

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

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

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

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

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

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

What is the suggested genetic risk factor for rheumatoid arthritis?

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

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

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

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

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

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

<p>98% (B)</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 (B)</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 (D)</p> Signup and view all the answers

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

<p>Organs (D)</p> Signup and view all the answers

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

<p>60-70% (B)</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 (D)</p> Signup and view all the answers

What is the concordance rate of RA in monozygotic twins?

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

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

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

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

<p>Smoking (B)</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. (B)</p> Signup and view all the answers

What is the prevalence of rheumatoid arthritis worldwide?

<p>1 in 100 people (B)</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 (A)</p> Signup and view all the answers

What are the characteristic symptoms of rheumatoid arthritis?

<p>Morning stiffness, swelling, heat, redness, and pain (C)</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. (A)</p> Signup and view all the answers

What is the goal of treatment for rheumatoid arthritis?

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

What is the role of cytokines in rheumatoid arthritis?

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

What is the impact of chronic inflammation on tissue?

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

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

<p>Autoreactivity (B)</p> Signup and view all the answers

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