Medicine Marrow Pg No 497-506 (Rheumatology)
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Questions and Answers

Which of the following is a systemic manifestation of Polyarteritis Nodosa?

  • Weight loss (correct)
  • Eosinophilia > 10%
  • Asthma
  • Pulmonary infiltrates

    • Neutrophil predominant inflammation is a characteristic of Polyarteritis Nodosa.

    True

    What is the age group most commonly affected by Polyarteritis Nodosa?

    40-60 years

    Polyarteritis Nodosa is characterized by transmural necrotizing inflammation without __________.

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

    Match the following associations with their description:

    <p>Hepatitis B = 30% of PAN cases have this Hairy cell leukemia = Associated condition Aneurysms = Possible vascular complication Mononeuritis multiplex = Involvement of multiple single motor nerves</p> Signup and view all the answers

    Which treatment is considered the drug of choice (DOC) for rheumatologic conditions?

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

    The female to male ratio for Takayasu Arteritis is 2:1.

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

    What is the age range for patients typically diagnosed with Temporal Arteritis?

    <blockquote> <p>50 yr</p> </blockquote> Signup and view all the answers

    The treatment option used for patients with severe renal artery stenosis is __________.

    <p>Percutaneous transluminal renal angiogram with stenting</p> Signup and view all the answers

    Match the following features with their corresponding arteritis:

    <p>Female to Male Ratio: 2:1 = Temporal Arteritis Age Range: &gt;50 yr = Temporal Arteritis Female to Male Ratio: 9:1 = Takayasu Arteritis Age Range: &lt;50 yr = Takayasu Arteritis</p> Signup and view all the answers

    Which of the following is NOT a category of ANCA-associated vasculitis?

    <p>Churg-Strauss syndrome</p> Signup and view all the answers

    The target antigens involved in ANCA-associated vasculitis include Proteinase 3 (PR3) and Myeloperoxidase (MPO).

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

    What triggers the antibody-mediated process in ANCA-associated vasculitis?

    <p>Infection, specifically lipopolysaccharide present in the antigen.</p> Signup and view all the answers

    ANCA associates with neutrophils to activate the __________ complement pathway.

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

    Match the steps in the ANCA-mediated process to their descriptions:

    <ol> <li>Degranulation of Neutrophil = a. Neutrophils release antimicrobial substances</li> <li>NETosis = b. Formation of Neutrophil extracellular traps</li> <li>Endothelial injury = c. Damage to blood vessel lining</li> <li>Stimulation of ANCA production = d. Increase in antibodies against neutrophils</li> </ol> Signup and view all the answers

    Which of the following conditions is NOT a possible diagnosis based on the chest X-ray findings?

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

    Microscopic Polyangiitis (MPA) shows evidence of granulomas.

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

    What is the demographic characteristic of patients most commonly affected by Microscopic Polyangiitis?

    <p>Elderly males aged 50-65 years</p> Signup and view all the answers

    The primary renal manifestation of Microscopic Polyangiitis is __________.

    <p>Rapidly progressive glomerulonephritis (RPGN) type 3</p> Signup and view all the answers

    Match the following symptoms with their corresponding classification in Microscopic Polyangiitis:

    <p>Fatigue = Major Systemic Manifestation Myalgia = Major Systemic Manifestation Skin manifestations = Minor Systemic Manifestation Minimal neuropathy = Minor Systemic Manifestation</p> Signup and view all the answers

    Which of the following features corresponds to a positive score in the Granulomatosis with Polyangiitis (GPA) criteria?

    <p>Granuloma on biopsy</p> Signup and view all the answers

    Microscopic Polyangiitis (MPA) has a positive correlation for an eosinophil count of ≥1 x 10^6/L.

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

    What is the required total score for classification in Granulomatosis with Polyangiitis (GPA)?

    <p>25</p> Signup and view all the answers

    Churg-Strauss Syndrome is characterized by necrotizing vasculitis and __________ necrosis.

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

    Match the following conditions with their primary features:

    <p>Granulomatosis with Polyangiitis (GPA) = Pauci-immune glomerulonephritis Microscopic Polyangiitis (MPA) = Primarily affects older adults Churg-Strauss Syndrome (EGPA) = Necrotizing vasculitis with asthma Eosinophilic gastroenteritis = Gastrointestinal symptoms with eosinophilia</p> Signup and view all the answers

    Which of the following is considered the gold standard for assessing vascular conditions?

    <p>Conventional angiography</p> Signup and view all the answers

    MR angiography is the only method used for diagnosing vascular issues.

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

    What is the primary purpose of conducting a CT angiogram?

    <p>To visualize and assess vascular structures, specifically for conditions like stenosis.</p> Signup and view all the answers

    Which of the following treatments is preferred for patients with Systemic Lupus Erythematosus (SLE) instead of Azathioprine?

    <p>Mycophenolate mofetil</p> Signup and view all the answers

    The presence of a ________ can indicate vascular disease when examining the subclavian or aortic region.

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

    Match the imaging technique with its primary characteristic:

    <p>MR angiography = Best measurement of vessel wall thickness CT angiogram = Detailed visualization of vascular structures Conventional angiography = Gold standard for intervention planning Ultrasound = Non-invasive assessment of blood flow</p> Signup and view all the answers

    Cyclophosphamide can be administered either intravenously or orally with the same efficacy.

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

    Name one indication for plasma exchange in the treatment of GPA or MPA.

    <p>Serum creatinine &gt; 5.5 mg/dL</p> Signup and view all the answers

    The combination of low dose steroid and __________ is used for the maintenance phase of GPA and MPA treatment.

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

    Match the following features with their corresponding type of vasculitis:

    <p>Blood nasal discharge, ulcers, crusting = Granulomatosis with Polyangiitis (GPA) Pauci-immune glomerulonephritis on biopsy = Microscopic Polyangiitis (MPA) Positive CANCA or anti-PR3 ANCA = Granulomatosis with Polyangiitis (GPA) Positive PANCA or anti-MPO ANCA = Microscopic Polyangiitis (MPA)</p> Signup and view all the answers

    What condition is most commonly associated with both nasal septal ulcers and crusting?

    <p>Granulomatosis with Polyangiitis</p> Signup and view all the answers

    Cough and hemoptysis are symptoms of diffuse alveolar hemorrhage.

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

    What is the primary renal manifestation associated with rapidly progressive glomerulonephritis?

    <p>Type 3 RPGN</p> Signup and view all the answers

    Recurrent epistaxis is a major systemic manifestation associated with _______ tract involvement.

    <p>upper respiratory</p> Signup and view all the answers

    Match the minor systemic manifestations with their respective descriptions:

    <p>Arthralgia = Joint pain similar to that seen in SLE Peripheral neuropathy = Nerve damage causing weakness or pain Palpable purpura = Skin lesions due to small blood vessel leakage Scleritis = Inflammation of the sclera in the eye</p> Signup and view all the answers

    What does a positive result for True C-ANCA suggest?

    <p>Renal Limited vasculitis</p> Signup and view all the answers

    Indirect immunofluorescence can show two patterns associated with antibodies.

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

    Name one drug that can induce vasculitis.

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

    The confirmation test for vasculitis is called __________.

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

    Which pattern is associated with Anti-PR3 antibody?

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

    Match the following types of vasculitis with their associated antibodies:

    <p>GPA = Anti-PR3 antibody MPA = Other factors EGPA = Unknown Renal Limited vasculitis = True P-ANCA</p> Signup and view all the answers

    Granuloma is a definitive finding in necrotizing vasculitis.

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

    What is a characteristic positive finding in necrotizing vasculitis?

    <p>Necrotizing vasculitis (d/t neutrophils)</p> Signup and view all the answers

    Which feature is considered a hallmark of small vessel vasculitis?

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

    Granulomatosis with Polyangiitis is more common in males than females.

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

    What type of vessel is primarily involved in medium vessel vasculitis?

    <p>Muscular artery</p> Signup and view all the answers

    The type of antibodies predominantly associated with Granulomatosis with Polyangiitis is _____.

    <p>C-ANCA</p> Signup and view all the answers

    Match the types of vasculitis with their corresponding features:

    <p>Small Vessel Vasculitis = Purpura, Petechiae Medium Vessel Vasculitis = Nodules, Gangrene Large Vessel Vasculitis = Bruit, Asymmetry of pulse GPA = Necrotising vasculitis + Granuloma</p> Signup and view all the answers

    What is one of the features of large vessel vasculitis?

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

    In small vessel vasculitis, non-blanchable pin-point macules develop due to capillary inflammation.

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

    At what age group does Granulomatosis with Polyangiitis commonly occur?

    <blockquote> <p>40 years</p> </blockquote> Signup and view all the answers

    Mononeuritis multiplex is a feature of _____ vessel vasculitis.

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

    Which of the following is NOT a feature of small vessel vasculitis?

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

    Study Notes

    Small Vessel Vasculitis

    • ANCA-associated vasculitis is a type of small vessel vasculitis.
    • Categories include:
      • Granulomatosis with polyangiitis (GPA)
      • Microscopic polyangiitis (MPA)
      • Allergic granulomatosis with polyangiitis (AGPA)
      • Eosinophilic granulomatosis with polyangiitis (EGPA)
    • Target antigens include:
      • Proteinase 3 (PR3)
      • Myeloperoxidase (MPO)
    • Location of target antigens is within the azurophilic granules of resting neutrophils (lysosomes of monocytes)
    • Pathogenesis:
      • Triggered by infection with Lipopolysaccharide present in the antigen.
      • Leads to priming of neutrophils: expression of PR3 & MPO on the surface of neutrophils
      • Activates the alternate complement pathway: ANCA activates the alternate complement pathway
      • Degranulation of neutrophils
      • NETosis: Defective killing of necrotic debris, release of neutrophilic contents to trap antigens
    • Forms Neutrophil extracellular traps (NETs)
    • NET components are not degraded effectively, resulting in increased exposure of NET components
    • Stimulation of ANCA production, causing ANCA to attack primed neutrophils.
    • Endothelial injury and inflammation occur as a consequence.

    Granulomatosis with Polyangiitis (GPA)

    • Features include: Necrotizing vasculitis + Fibrinoid necrosis (Neutrophilic) + Granuloma
    • Antibodies: CCC-ANCA (70%) > PPP-ANCA (25%)
    • Age group: >40 years
    • Sex ratio: Male = Female
    • HLA DP BI 04 is associated with GPA
    • ANCA titres are a predictor of relapse
    • Upper respiratory tract (95% involvement):
      • Midline nasal deformities: Nasal septal ulcers, crusting, perforation, saddle nose deformity
      • Recurrent epistaxis
      • Serous otitis media (B/D)
      • Recurrent sinusitis (due to Staphylococcus aureus)
      • Conductive or sensorineural hearing loss
      • Subglottic stenosis
    • Lower respiratory tract (90%):
      • Thick-walled cavitating nodules
      • Diffuse alveolar hemorrhage (DAH): Cough, hemoptysis, filling opacity, Hemosiderin-laden macrophages in BAL
    • Renal (60%): Rapidly Progressive Glomerulonephritis: Type 3 RPGN, Pauci-immune
    • Minor Systemic Manifestations
      • Joints: Arthralgia (can mimic SLE)
      • CNS: Peripheral neuropathy
      • Skin: Cutaneous non-thrombocytopenic palpable purpura
      • Ocular:
        • Scleritis
        • Orbital pseudotumor
        • Lacrimal involvement (Rare)
    • Constitutional symptoms: Fatigue, weight loss, myalgia (50-60%)
    • ACR Classification criteria:
      • Nasal involvement: Blood nasal discharge, ulcers, crusting, congestion or blockage, or septal defect/perforation
      • Ear involvement: Cartilaginous involvement (cartilage inflammation of the ear or nose, hoarse voice or stridor, endobronchial involvement, or saddle nose deformity)
      • Hearing loss: Conductive or sensorineural hearing loss
      • ANCA: CANCA or anti-PR3 ANCA positive
      • Lung involvement: Pulmonary nodules, mass, cavitation on chest imaging. Fibrosis or interstitial lung disease on chest imaging.

    Microscopic Polyangiitis (MPA)

    • Demographics:
      • Age group: Elderly (50-65 years)
      • Sex ratio: Male > Female
    • Pathology: Necrotizing vasculitis, fibrinoid necrosis (neutrophilic), no granuloma
    • Antibodies: p-ANCA > c-ANCA
    • Major Systemic Manifestations:
      • Fatigue
      • Weight Loss
      • Myalgia
    • Minor Systemic Manifestations:
      • Ocular: Unlikely
      • Joint: Not Significant
      • Skin: More common than GPA
      • CNS: Minimal neuropathy
    • Lower Respiratory Tract (50%): Diffuse alveolar hemorrhage (DAH)
    • Upper Respiratory Tract (30%): Sinusitis (Staphylococcus aureus)
    • Renal (100%): Most common, RPGN type 3
    • Microscopic Polyangiitis (MPA) ACR Classification criteria:
      • Nasal involvement: Nasal bloody discharge, ulcers, crusting, congestion or blockage, or nasal septal defect/perforation.
      • ANCA: PANCA or anti-MPO ANCA positive.
      • Kidney involvement: Pauci-immune glomerulonephritis on biopsy.

    Churg-Strauss Syndrome (EGPA/AGPA)

    • Features: Necrotizing vasculitis + Fibrinoid necrosis + Extravascular granuloma
    • Eosinophilic
    • Predominantly ANCA negative, ANCA +ve: Bad prognosis
    • Age group: Young individuals
    • Sex Ratio: Male = Female
    • Asthmatic Phase:
      • Seen in adolescents
      • Recurrent allergic rhinitis + Asthma
      • Migratory lung infiltrations in CXR
    • High Titter ANCA +ve cases manifestations:
      • DAH
      • RPGN
    • Vasculitic Phase:
      • Eosinophilic gastroenteritis
      • Eosinophilic myocarditis (most common cause of death)
      • Severe neuropathy: Can involve cranial nerves
      • Mononeuritis multiplex: may involve medium vessels
      • Skin lesions: Predominant
    • Prognosis: Bad

    Clinical Features of Small Vessel Vasculitis

    • Small Vessel Vasculitis:
      • Involvement: Capillaries, post capillary venules
      • Layer: Superficial papillary dermis
      • Features: Purpura, Petechiae, Bulla, Urticaria
    • Medium Vessel Vasculitis:
      • Involvement: Muscular artery
      • Layer: Reticular dermis
      • Features: Nodules, Gangrene, Mononeuritis multiplex
    • Large Vessel Vasculitis:
      • Involvement: -
      • Layer: -
      • Features: Bruit, Asymmetry of pulse

    Takayasu Arteritis

    • F:m ratio - 9:1
    • Age range - # Polyarteritis Nodosa (PAN)
    • Lesion in Takayasu arteritis → Stenosis
    • ACR Classification criteria:
      • Age < 40 years
      • Claudication of upper extremities (≥ 10 mmHg)
      • Subclavian/aortic bruit
      • Angiographic evidence of stenosis
    • Investigations:
      • MR angiography: IOC for diagnosis and follow-up, provides the best measurement of vessel wall thickness
      • CT angiogram
      • Conventional angiography: Gold standard, Performed if vessel intervention is planned

    Polyarteritis Nodosa (PAN)

    • Transmural necrotizing inflammation without granuloma.
    • Neutrophil predominant.
    • Focal and segmental necrosis.
    • ANCA –ve
    • Medium vessel involved:
      • Age group: 40-60 years
      • Sex: M = F
    • Associations:
      • Hepatitis B: 30/1 rule. 30% of PAN cases have Hepatitis B. 1% of Hepatitis B cases have PAN.
        • Hairy cell leukemia
      • Aneurysms
    • Systemic Manifestations:
      • Constitutional symptoms:
        • Weight loss
        • Fever
        • Myalgia
        • First symptom
      • Lesions: Nodule, ulcer, gangrene.
      • Hemoptysis (D/t involvement of bronchial artery)
      • Renal failure (D/t renal artery stenosis): Abdominal pain
      • Testicular pain (D/t involvement of testicular artery)
      • Mononeuritis multiplex: Involvement of >1 single motor nerve, Sudden onset.

    Management of GPA & MPA

    • Induction:
      • Methylprednisolone: IV 500mg - 1g x 3 days, oral 1 mg/kg/day, taper over 2-3 months to 5mg/day.
      • Cyclophosphamide: IV or oral (same efficacy), side effects: Gonadal toxicity
      • Rituximab: If unwilling for cyclophosphamide: Used in relapse. Minimal disease: Steroid + methotrexate.
    • Maintenance:
      • Low dose steroid + Azathioprine
      • Note: In SLE, Mycophenolate mofetil (mmF) is preferred to Azathioprine.
    • Plasma exchange indications:
      • DAH (ongoing)
      • Serum creatinine > 5.5 mg/dL
      • CNS involvement

    Screening and Confirmation Tests for Vasculitis

    • Screening: Indirect immunofluorescence, patterns observed: cytoplasmic and perinuclear. Cytoplasmic pattern associated with Anti-PR3 antibody, perinuclear pattern associated with other factors.
    • Confirmation: ELISA, a positive result for True C-ANCA suggests certain causes.
    • Causes of Positive C-ANCA:
      • True P-ANCA can lead to Renal Limited vasculitis, which can be further categorized as GPA > MPA > EGPA.
      • Other negative cases: Autoimmune hepatitis Type-1º sclerosing cholangitis, Rheumatoid arthritis, Inflammatory bowel disease, Infective endocarditis, and Cystic fibrosis.
    • Causes of Negative C-ANCA:
      • Drug-Induced Causes: Hydralazine, Propylthiouracil, Levamisole adulterated cocaine, minocycline
      • Other causes: Goodpasture syndrome
      • Unknown causes: When the result is not against MPO, there may not be a clear cause.

    Necrotizing Vasculitis

    • Findings:
      • Necrotizing vasculitis (d/t neutrophils): This is a positive finding.
      • Fibrinoid necrosis (d/t extravasation of RBC): This may or may not be present (±).
      • Granuloma: This may or may not be present (±).

    Note on Specific Conditions

    • 1° biliary cirrhosis: associated with Anti-mitochondrial antibody
    • Neutrophil-mediated vasculitis
    • Leukocytoclasis: In conjunction with Immune complex mediated vasculitis
    • Biopsy & Immunofluorescence of skin: Preferred method of diagnostic approach. A significant finding may be the absence of deposits, suggesting Pauci immune.

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    Description

    Test your knowledge on small vessel vasculitis, focusing on ANCA-associated types including GPA, MPA, AGPA, and EGPA. Explore the target antigens, pathogenesis, and the role of neutrophils in this condition. This quiz will challenge your understanding of the complexities involved in small vessel vasculitis.

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