blood and immune - de lorenzo

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Questions and Answers

What is the age of the young female patient in the first clinical case?

  • 36 years old
  • 26 years old (correct)
  • 16 years old
  • 46 years old

Which of the following is part of the patient's general history?

  • Reported allergies
  • Diabetes type I
  • Normal menstrual cycle (correct)
  • Smoker

Which autoimmune condition was the patient treated for in 2009?

  • Autoimmune thrombocytopenia (correct)
  • Lupus nephritis
  • Autoimmune hemolytic anemia
  • Rheumatoid arthritis

What symptoms did the patient start experiencing in April 2014?

<p>Hair loss (alopecia) (C)</p> Signup and view all the answers

The swelling of joints and alopecia can be a symptom of what?

<p>An autoimmune etiology (B)</p> Signup and view all the answers

What does alopecia with erythema indicate?

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

What blood analysis result would orient the doctor towards an autoimmune disease?

<p>Positive antinuclear antibodies (ANA) (A)</p> Signup and view all the answers

What does a complete blood count provide information about?

<p>RBC, WBC, hemoglobin, and platelets (A)</p> Signup and view all the answers

Which of the following results from a blood analysis prescribed by the rheumatologist?

<p>Normal renal profile (C)</p> Signup and view all the answers

What does a low hemoglobin level indicate?

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

What does the presence of anti-double stranded DNA antibodies suggest?

<p>That lupus nephritis is possible (A)</p> Signup and view all the answers

What does the presence of anti-Smith antibodies indicate?

<p>They are very specific for lupus (D)</p> Signup and view all the answers

Anemia of chronic disease results in decreased

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

What is prednisone used for?

<p>Treat active inflammatory diseases (C)</p> Signup and view all the answers

What type of medication is Azathioprine?

<p>An immunosuppressive (C)</p> Signup and view all the answers

What are the criteria for autoimmune diseases primarily used for?

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

What does proteinuria in the urine of 24 hours indicate?

<p>That her disease is not controlled (B)</p> Signup and view all the answers

What is the maximum score that can be achieved in SLEDAI-2K?

<p>103 (B)</p> Signup and view all the answers

What is the purpose of Kidney biopsy?

<p>To determine class of lupus nephritis the patient have (C)</p> Signup and view all the answers

What is Mycophenolate mofetil (MMF) primarily given for?

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

Idiopathic inflammatory myopathies are characterized by an autoimmune attack against what?

<p>The skeletal muscle (D)</p> Signup and view all the answers

What is a common symptom of idiopathic inflammatory myopathies?

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

Which of the following is a risk factor for idiopathic inflammatory myopathies?

<p>Genetic predisposition (C)</p> Signup and view all the answers

What type of infections are considered environmental risk factors for myopathies?

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

Which medication is known to sometimes cause immune-mediated necrotizing myopathy?

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

What does muscle cell lysis lead to?

<p>Increased levels of muscle enzymes (D)</p> Signup and view all the answers

What type of muscle is primarily affected in idiopathic inflammatory myopathies?

<p>Skeletal muscle (C)</p> Signup and view all the answers

In healthy muscles, where are macrophages typically located?

<p>In the perimysium and near the epimysium (B)</p> Signup and view all the answers

What is the main function of macrophages in healthy muscle tissue?

<p>To remove dead debris (C)</p> Signup and view all the answers

What process is avoided in physiological skeletal muscle repair?

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

Which of the following is a characteristic of type 1 muscle fibers?

<p>Have a low metabolism that relies on aerobic respiration (D)</p> Signup and view all the answers

Which of the following best describes the usual female to male ratio in idiopathic inflammatory myopathies?

<p>1:1 (A)</p> Signup and view all the answers

What is the name of the connective tissue around the muscle fibers?

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

Based on the text, what is HLA 8.1 ancestral haplotype (HLA-DR3 considered an example of?

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

Which of the following features is observed when cutting the muscle in a cross-section?

<p>The fibers will appear roundish and the nuclei will be centrally located. (C)</p> Signup and view all the answers

Flashcards

What is alopecia?

Hair loss

What causes erythema?

Erythema is caused by vasodilation due to endothelial inflammation.

What do ANA tests check for?

Antinuclear antibodies. If they are positive they direct you towards an autoimmune disease

What might low haemoglobin indicate?

Indicates anaemia, low white blood cell count, and low platelets.

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What are anti-smith antibodies?

Very specific antibodies for lupus but not sensitive at all, so they are not frequently present but when present they are very specific.

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Why perform urine tests in lupus?

Monitor kidney involvement in lupus patients.

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What is Prednisone (PDN)?

Used to treat active inflammatory diseases like lupus.

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What does a 24-hour proteinuria test measure?

Measure proteinuria in urine over 24 hours to quantify kidney damage.

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What is SLEDAI-2K?

A score that helps understand how active the disease of the patient is.

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What does a kidney biopsy help with

Diagnosis of lupus nephritis, tells you if there is lupus nephritis and also what class of lupus nephritis it is

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What does Belimumab do?

Targets B cells (similar to rituximab, which is an anti T 20). Belimumab actually targets the BAFF receptor (B cell activating factor), which is a receptor that activates B cell.

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What are the signs of a good shot for a patient

Stable vasculitic lesions, increased proteinuria, urinary sediment contains less RBCs and WBCs

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What is neuropsychiatric SLE (NPSLE)?

Autoimmune-related inflammation of the brain.

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When are headaches more common?

When a patient has positivity to antiphospholipid antibodies

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Idiopathic Inflammatory Myopathies

A heterogeneous group of disorders characterized by an autoimmune attack against the skeletal muscle.

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Characteristics of Idiopathic Inflammatory Myopathies

Chronic inflammation of the skeletal muscle leading to muscle cell lysis and weakness, marked by increased muscle enzyme levels.

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Environmental Risk Factors

These may include viral infections. They increase the risk of autoimmune diseases.

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Drug-Induced Myopathy

Rarely, drugs, such as statins or corticosteroids, can induce myopathy.

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Epidemiology of Myopathies

Idiopathic inflammatory myopathies are rare diseases affecting people of all ages, with a slight female predominance, except for inclusion body myositis.

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Structure of Skeletal Muscles

Myofibers are grouped into fascicles, surrounded by connective tissue (perimysium) and wrapped by epimysium.

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Immune Response to Muscle Injury

Produce chemokines, recruit immune cells, and produce cytokines, leading to myofiber death and regeneration.

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Mechanism of Self-Perpetuation

Adaptive immunity leads to specific antibody production and chronic inflammation, preventing resolution and causing more damage.

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EULAR-ACR Criteria (2017)

These commonly used criteria recognize subtypes of myositis but are not perfect due to overlapping features.

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Clinical Manifestations

Characterized by muscle weakness, myalgias, and potential extramuscular manifestations like myocarditis.

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Muscle Weakness Pattern

Muscle weakness in idiopathic inflammatory myopathies typically involves proximal muscles, starts in the lower limbs, and can affect upper limbs and pharynx.

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Typical Progression

Subacute onset of symmetrical weakness in proximal muscles, primarily affecting lower limbs initially.

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Creatine Kinase (CK) Levels

Levels are very high, especially in necrotizing myopathy, potentially causing toxic renal failure.

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Histological Features of Myositis

Muscle biopsy shows endomysial inflammation, while dermatomyositis shows perimysial inflammation. Common features include central nuclei, type 2 myofiber prevalence, and MHC I overexpression.

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Immune-Mediated Necrotizing Myopathy

Involves proximal and symmetrical muscle weakness and high CK levels; commonly associated with statins.

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