Medicine Marrow Pg No 437-446 (ECG)
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Questions and Answers

Which of the following conditions are extrahepatic manifestations of HCV? (Select all that apply)

  • Porphyria cutanea tarda (correct)
  • Cryoglobulinemia (correct)
  • Systemic lupus erythematosus
  • Cutaneous lichen planus (correct)

    • HLA DR3 is associated with Sjogren's syndrome and other autoimmune diseases.

    True

    What is one clinical symptom of Sjogren's syndrome?

    Dry eye (keratoconjunctivitis sicca)

    In Sjogren's syndrome, auto antibody formation leads to autoimmune lymphocytic __________.

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

    Match the following components related to Sjogren's syndrome with their roles:

    <p>Anti-m (muscarinic) = Treatment with cholinergic agonist IL-18 positive macrophages = Painless parotid enlargement HLA DR3 = Genetic predisposition T cell (Th) = Involvement in inflammation</p> Signup and view all the answers

    Which of the following treatments is recommended for a patient with SpO2 less than 90%?

    <p>Oxygen therapy</p> Signup and view all the answers

    Calcium channel blockers are part of the standard treatment in the acute management of ACS.

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

    What are the two options for antiplatelet therapy after discharge?

    <p>Ticagrelor 90mg BD for 1 year or Clopidogrel 75mg OD for 1 year.</p> Signup and view all the answers

    The recommended dose of Morphine for ACS management is _____ .

    <p>as needed</p> Signup and view all the answers

    Match the following medications with their respective doses:

    <p>Aspirin = 75mg daily for lifetime Rosuvastatin = 20mg daily for lifetime Prasugrel = 10mg daily for 1 year Ticagrelor = 90mg twice daily for 1 year</p> Signup and view all the answers

    What is the primary clinical hallmark of Sjogren's syndrome?

    <p>Dry eyes and dry mouth</p> Signup and view all the answers

    Sjogren's syndrome affects more men than women.

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

    What are the two classifications of Sjogren's syndrome based on association with connective tissue disorders?

    <p>Primary Sjogren's and Secondary Sjogren's</p> Signup and view all the answers

    Sjogren's syndrome can present as an extrahepatic manifestation of ______.

    <p>Hepatitis C virus (HCV)</p> Signup and view all the answers

    Match the following features of Sjogren's syndrome with their descriptions:

    <p>Glandular = Affects glands Extraglandular = Affects other body systems Primary Sjogren's = Associated with rheumatoid arthritis (RA) Secondary Sjogren's = Can be triggered by other CTDs</p> Signup and view all the answers

    What is the primary indication for using GP IIb – IIIa inhibitors?

    <p>Downstream bailout therapy in cath lab</p> Signup and view all the answers

    ACE inhibitors should be avoided if there are signs of heart failure or evidence of low output state.

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

    What is the common dosage for Clopidogrel in a scenario where thrombolysis is not available?

    <p>300 mg</p> Signup and view all the answers

    Patients receiving __________ should also start an ACE inhibitor within 24 hours.

    <p>β-blocker</p> Signup and view all the answers

    Match the following contraindications of thrombolysis with their descriptions:

    <p>History of intracranial hemorrhage = Cannot undergo thrombolysis Severe uncontrolled hypertension = High risk of bleeding Recent significant closed head trauma = Increased risk of intracranial hemorrhage Intracranial surgery within 2 months = Recent major surgery contraindication</p> Signup and view all the answers

    Which of the following is NOT a symptom of Sjogren's Syndrome?

    <p>Skin rashes</p> Signup and view all the answers

    Positive Anti-Ro/La antibodies indicate a good prognosis in Sjogren's cases.

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

    What is a common manifestation of Sjogren's Syndrome besides dry mouth?

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

    Children with Sjogren's may experience recurrent episodes of acute ______ due to duct blockade.

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

    Match the following predictors of extraglandular Sjogren to their details:

    <p>Low complement = Indicates immune system involvement Anti-Ro = Associated with Sjogren's and SLE Rheumatoid factor = Can be positive in Sjogren's Cryoglobulins = Linked to B-cell lymphoma risk</p> Signup and view all the answers

    What is the potency percentage of Reteplase?

    <p>80%</p> Signup and view all the answers

    Thrombolysis is recommended to be administered within 6-12 hours for better outcomes.

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

    What is the loading dose of Aspirin in thrombolysis?

    <p>325 mg</p> Signup and view all the answers

    The primary fibrin specificity of Tenecteplase is _____.

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

    Match the thrombolytic agents with their features:

    <p>Streptokinase = Antigenic with a risk of allergy Tenecteplase = Highest fibrin selectivity Reteplase = Very short half-life Alteplase = 75% potency</p> Signup and view all the answers

    What is the hallmark histopathological finding in minor salivary gland biopsies for diagnosis of Sjogren's syndrome?

    <p>Focal lymphocytic sialadenitis</p> Signup and view all the answers

    CD20 is a marker used to identify T cell infiltration.

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

    What test is used to diagnose dry eyes in patients suspected of having Sjogren's syndrome?

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

    The clinical feature characterized by a gritty, sandy feeling in the eyes is associated with __________ dysfunction.

    <p>meibomian gland</p> Signup and view all the answers

    Match the following immunohistochemical stains with the cell types they identify:

    <p>CD20 = B cells CD3 = T cells</p> Signup and view all the answers

    Which complication is associated with a proximal LAD lesion?

    <p>Ventricular Tachycardia</p> Signup and view all the answers

    Electrical complications are more common with RCA lesions than with LAD lesions.

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

    What is the primary management strategy for mechanical complications following STEMI?

    <p>Reduce afterload and refer to CTVS.</p> Signup and view all the answers

    Electromechanical dissociation is characterized by a lack of ______ and ______.

    <p>blood pressure, pulse</p> Signup and view all the answers

    Match the following complications with their descriptions:

    <p>Free wall rupture = Electromechanical dissociation and coagula tamponade Septal rupture = Pain and new murmur in left lower sternal border RVMI = Hypotension Cardiogenic Shock = 40% myocardium damage</p> Signup and view all the answers

    What is the maximum dose of Clopidogrel for adults?

    <p>300 mg</p> Signup and view all the answers

    High blood pressure is associated with unsuccessful thrombolysis.

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

    What medication is recommended to be given after thrombolysis to prevent recurrence?

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

    The primary aim of PCI is to restore normal epicardial coronary flow, which is indicated by __________.

    <p>TIMI 3</p> Signup and view all the answers

    Match the following medications or treatments with their associated use in managing cardiac conditions:

    <p>Ticagrelor = Best choice for PCI LMWH = Anticoagulant after thrombolysis Nitrate = Treatment for Prinzmetal's angina Aspirin = Used in combination with statin during PCI</p> Signup and view all the answers

    What is the most common form of interstitial lung disease associated with severe manifestations in rheumatology?

    <p>Non-specific Interstitial Pneumonia (NSIP)</p> Signup and view all the answers

    Raynaud phenomenon is often seen following the appearance of sicca symptoms in a majority of patients.

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

    What characterizes small vessel vasculitis in rheumatological conditions?

    <p>Palpable purpura</p> Signup and view all the answers

    The presence of preserved _________ levels can indicate features of B cell lymphoma.

    <p>C3</p> Signup and view all the answers

    Match the following manifestations with their respective descriptions:

    <p>Truncal ataxia = CNS involvement with ganglionopathy Interstitial Lung Disease = Mainly Non-specific Interstitial Pneumonia (NSIP) Primary biliary cirrhosis = Autoimmune destruction of the intrahepatic bile duct Vasculitis = Characterized by palpable purpura</p> Signup and view all the answers

    Study Notes

    Sjogren's Syndrome

    • A multisystem autoimmune inflammatory connective tissue disorder
    • Primarily affects middle-aged women with female-to-male ratio of 9:1
    • Classified into primary and secondary based on association with other connective tissue disorders, with primary Sjogren's being more common and associated with rheumatoid arthritis, systemic lupus erythematosus, and idiopathic inflammatory myopathies.
    • Classified into glandular and extraglandular based on pathology, with glandular affecting glands and extraglandular affecting other body systems
    • Hallmark clinical feature is dry eyes and dry mouth
    • Can present as an extrahepatic manifestation of Hepatitis C virus
    • Salivary glands are involved in 90% of cases, with parotid glands being most commonly affected
    • Painless, symmetric salivary gland swelling is a common feature
    • Swelling is a result of lymphocytic infiltration of the salivary glands with periductal and perivascular inflammatory cells.
    • The inflammation is driven by T-cell activation with Th1 subtype promoting B-cell activation

    Salivary Gland Swelling

    • Asymmetric painful swelling with palpable nodules suggests six cell lymphoma, an extranodal marginal zone B-cell lymphoma
    • Lymphoma is associated with cryoglobulinemia, low complements (decreased C3 and C4)

    Anti-Ro/La Antibodies

    • Can be positive in 5% of individuals who develop Sjogren's syndrome after 10-15 years.
    • In diagnosed Sjogren's cases, positive Anti-Ro/La antibodies indicate extraglandular disease, poor prognosis (early onset, longer duration), risk for B-cell lymphoma, and salivary gland enlargement

    ### Other Symptoms

    • Oral thrush
    • Altered taste
    • Childhood Sjogren: Recurrent episodes of acute parotitis due to duct blockade
    • Dry mouth, deep red tongue, parotid gland swelling

    Clinical Manifestation

    • Fatigue
    • Arthralgia/arthritis: Jaccoud arthropathy (non-erosive)

    Predictors of Extraglandular Sjogren's

    • Low complement (decreased C3 & C4)
    • Anti-Ro (RoSA)/La (SS-A, SS-B)
    • Rheumatoid factor: Positive
    • Cryoglobulins

    Salivary Gland Biopsy

    • Major salivary gland: Benign lymphoepithelial lesions (B cells)
    • Minor salivary gland (gold standard): Focal lymphocytic sialadenitis (T cells)

    ### Immunohistochemical Stains

    • B cell infiltration: CD20
    • T cell infiltration: CD3

    Dry Eyes

    • Non-specific
    • Aqueous and lipid (meibomian gland dysfunction) defect
    • Irritation, gritty, sandy feeling, glares
    • Diagnosis: Schirmer test

    Role of HLA-DR3

    • Associated with genetic predisposition to Sjogren's Syndrome
    • Also associated with other diseases such as Systemic Lupus Erythematosus (SLE)

    ### Severe Manifestations

    • CNS: Ganglionopathy (Dorsal root ganglion involvement) → Truncal ataxia
    • Lung: Risk of Interstitial Lung Disease (ILD)
      • Most common: Non-specific Interstitial Pneumonia (NSIP)
      • Characterized by Lymphocytic Interstitial Pneumonia (LIP), ground-glass opacities (GGO), cysts, and nodules
    • Liver & Biliary Tree:
      • Can be extrahepatic manifestation of hepatitis C virus (HCV)
      • Primary biliary cirrhosis/cholangitis (autoimmune destruction of the intrahepatic bile duct)
    • Renal:
      • Tubulointerstitial kidney disease: Risk of distal renal tubular acidosis
      • Minor risk for chronic kidney disease (CKD)
    • Vascular:
      • Raynaud phenomenon:
        • Often precedes sicca symptoms in one-third of patients
        • Can cause critical limb ischemia
      • Vasculitis:
        • Small vessel vasculitis (most common)
        • Hallmark: Palpable purpura
        • Medium vessel vasculitis (severe form):
          • Characterized by ulceration and gangrene
          • Associated with Membranoproliferative Glomerulonephritis (MPGN) and neuropathy
    • Lymphadenopathy: Rule out lymphoma

    Features Indicative of B Cell Lymphoma

    • Persistent, asymmetric, painful, nodular parotid enlargement
    • Associated lymphadenopathy
    • Cryoglobulinemia
    • Decreased C3 levels
    • Increased rheumatoid factor (Ro/La) titers
    • Purpura
    • Leukopenia

    Immunohistochemistry

    • CD20 and PAX5 stain positive in B-cell lymphoma

    Acute Coronary Syndrome (ACS) - Evaluation and Management

    • Painful condition, with increased pain in nature, duration (greater than 20 min) and frequency, and not relieved by Nitrates/Rest
    • Diagnosis should be made rapidly and followed by prompt treatment

    Treatment

    • Oxygen (if SpO2 < 90%)
    • Aspirin 325mg (Chewable, non-enteric)
    • Rosuvastatin 40mg
    • S/L nitrate 5mg, repeat 3 times
    • Morphine
    • Percutaneous Coronary Intervention (PCI) within 24 hours

    High Risk Patients

    • Individuals at high risk for ACS complications should undergo PCI within 2 hours of diagnosis

    Intervention Based Guidelines

    • PCI is the treatment of choice (TOC)
    • Angiography within 24 hours, high risk patients within 2 hours
    • Platelet aggregation (P2Y12) inhibitors should be given in the cath lab

    Peri-Interventional Anticoagulation and Antiplatelet

    • Enoxaparin 0.5mg/kg bolus dose until patient reaches the cath lab
    • Prasugrel 60mg/Ticagrelor 180mg/Clopidogrel 600mg

    Post Discharge

    • Aspirin 75mg for lifetime
    • Rosuvastatin 20mg for lifetime
    • Ticagrelor 90mg BD x 1 year or Clopidogrel 75mg OD x 1 year
    • Prasugrel 10mg x 1 year (Best)

    Role of Other Drugs

    • GP IIb – IIIa inhibitors: Abciximab, Tirofiban, Eptifibatide

    • Use: Downstream bailout therapy (in cath lab if no TIMI 3 or MPG 3 flow: High thrombus burden)

    • ACE inhibitors and β-blockers:

      • Survival advantage if initiated within the first 24 hours
      • Avoided if signs of heart failure/evidence of low output state

    Thrombolysis and PCI

    • Thrombolysis and PCI are both effective treatment options for acute coronary syndromes (ACS).
    • PCI is superior to thrombolysis, as it has a lower risk of electrical and mechanical complications but should be performed within 12 hours.
    • In the absence of PCI, thrombolysis should be considered within 6-12 hours from onset of symptoms
    • The optimal time window for thrombolysis is within 1-2 hours of symptom onset.

    Agents Used in Thrombolysis

    • Streptokinase
      • Non-specific fibrin specificity
      • 50% potency
      • Highest risk of allergy
      • Lowest risk of intracranial hemorrhage
      • Dose: 1.5 million U in 100ml NS over 1 hour
    • Tenecteplase (Bolus)
      • Specific (max) fibrin specificity
      • 75% potency
      • Short half-life (10-12 min)
      • Highest fibrin selectivity
      • Dose: 0.5 mg/kg
    • Reteplase (Bolus)
      • Specific fibrin specificity
      • 80% potency
      • Very short half-life (4-8 min)
      • Dose: 10U x 2 doses 30min apart
    • Alteplase
      • Specific fibrin specificity
      • 75% potency

    ### Thrombolysis Versus PCI

    • Thrombolysis and PCI are equally effective in the first hour of ACS.
    • PCI is the preferred treatment option after thrombolysis.
    • Thrombolysis is recommended within 6 to 12 hours of ACS symptom onset.
    • Benefits of thrombolysis outweigh the risks within 6 to 12 hours; beyond that, the risks outweigh the benefits.

    Pre-Hospital Thrombolysis

    • Pre-hospital thrombolysis can result in a better outcome.

    Right Ventricular Myocardial Infarction (RVMI)

    • Hypotension, clear lung, and raised jugular venous pressure (JVP)

    ### Inferior Wall Myocardial Infarction (IWMI)

    • Bradycardia (due to proximal occlusion), and V1-V2 discordance

    Loading Dose in Thrombolysis

    • Aspirin: 325 mg, non-enteric chewable preparation
    • Statins
      • Atorvastatin: 40-80 mg
      • Rosuvastatin: 20-40 mg

    Complications of ST-Segment Elevation Myocardial Infarction (STEMI)

    • Hemodynamic complications
      • RVMI: Hypotension
      • Acute LVF:
        • 25% myocardium damage
        • Proximal LAD lesion
    • Cardiogenic Shock
      • 40% myocardium damage
      • Proximal LAD lesion
    • Electrical complications:
      • RCA lesion: Atrial arrhythmias
      • Proximal LAD lesion: Ventricular Tachycardia (VT), Ventricular Fibrillation (V.Fibrillation)
    • Mechanical Complications:
      • Tear of infarcted tissue, within 24 hours or 3-5 days
      • Treatment: Reduce afterload - CTVS referral
    • Presentation
      • Free wall rupture:
        • Electromechanical dissociation: No blood pressure, no pulse, coagula tamponade
        • Poor prognosis
      • Septal rupture (Rare):
        • After 3-5 days: Pain + New murmur in left lower sternal border + Thrill
      • Acute mitral regurgitation: (RCA involvement - Posteromedial papillary muscle involved), Presents as acute pulmonary edema

    NSTEMI Management (ESC)

    • Pathogenesis
      • Fixed plaque (Asymptomatic): Chronic stable angina
      • Plaque rupture (Elderly & comorbid population): Critical/Subtotal occlusion
        • ST/T changes
        • Platelet rich thrombus (Fibrin content - No role of thrombolytics)

    MX Algorithm (PCI Center)

    • Available
      • Aspirin 325 mg
      • Ticagrelor 180 mg or Clopidogrel 600mg
      • Rosuvastatin 40 mg
    • Not Available (Thrombolysis)
      • Clopidogrel 300 mg + Tenecteplase 0.5 mg/kg.
      • Repeat ECG after 60-90 mins
    • Cath lab: Start β blocker & ACE inhibitor (within 24 hrs)

    Cath Lab

    • TIMI 3/MPG 3: Normal
    • Pharmacoinvasive approach: Anticoagulants (Enoxaparin 30mg iv)
    • Rescue PCI
    • CCU and start:
      • Rosuvastatin 20 mg lifetime
      • Aspirin 75 mg lifetime
      • Clopidogrel 75 mg x 1 year

    Contraindications of Thrombolysis

    • History of intracranial hemorrhage/structural cerebrovascular mass/stroke within 3 months (Except acute stroke within 4.5 hours)
    • Bleeding disorders/Aortic dissection
    • Significant closed head or facial trauma within 3 months
    • Intracranial/Intraspinal surgery within 2 months
    • Severe uncontrolled hypertension (HTN)
    • Streptokinase C/1 if used within 6 months

    ### Medical Notes

    •  ECG (438)
    • Active space
    • Clopidogrel (P.Y.inhibitors):
      • 300 mg (adult dose)
      • Age > 75 years: 75 mg
      • Blood cholesterol monitoring 3-6 months
    • Nitrate: 5 mg SL/3 tablets (Prinzmetal's angina)

    In PCI

    • Aspirin and statin: Same dose
    • Ticagrelor: 180 mg (best)
    • Aim of PCI:
      • Restore normal epicardial coronary flow (TIMI 3)
      • To attain normal microvascular flow (MPG 3)

    Changes Post Thrombolysis

    • Hypotension + Bradycardia ↓ Vessels reopening
    • BP:
      • High BP: Associated with successful thrombolysis (more drug - plaque contact)
      • Low BP (Bad prognosis): Due to
        • Hypovolemia
        • RVMI (IWMI) Cardiogenic shock
          • IVC diameter to be assessed
          • IV fluids
          • Intra-aortic balloon pump
    • Prevention of recurrence:
      • Post thrombolysis - Raw areas exposed → Anticoagulants given.
      • LMWH: Enoxaparin 30mg bolus & 1 mg/kg SC BD till revascularization / 8 days (ultra fractionated heparin)

    ### Note: 

    • Young thrombus responds better if lysed within 1-2 hours.
    • If primary PCI cannot be performed after STEMI diagnosis, fibrinolytic therapy is recommended within 12 hours.

    Medicine v1.0 Marrow 8.0 2024

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    Description

    Explore the key features of Sjogren's Syndrome, an autoimmune disorder primarily affecting middle-aged women. This quiz covers its classifications, hallmark symptoms such as dry eyes and mouth, and the involvement of salivary glands. Understand the impact of this condition on various body systems and its association with other connective tissue disorders.

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