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Questions and Answers
Which of the following is a systemic manifestation of Polyarteritis Nodosa?
Which of the following is a systemic manifestation of Polyarteritis Nodosa?
Neutrophil predominant inflammation is a characteristic of Polyarteritis Nodosa.
Neutrophil predominant inflammation is a characteristic of Polyarteritis Nodosa.
True
What is the age group most commonly affected by Polyarteritis Nodosa?
What is the age group most commonly affected by Polyarteritis Nodosa?
40-60 years
Polyarteritis Nodosa is characterized by transmural necrotizing inflammation without __________.
Polyarteritis Nodosa is characterized by transmural necrotizing inflammation without __________.
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Match the following associations with their description:
Match the following associations with their description:
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Which treatment is considered the drug of choice (DOC) for rheumatologic conditions?
Which treatment is considered the drug of choice (DOC) for rheumatologic conditions?
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The female to male ratio for Takayasu Arteritis is 2:1.
The female to male ratio for Takayasu Arteritis is 2:1.
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What is the age range for patients typically diagnosed with Temporal Arteritis?
What is the age range for patients typically diagnosed with Temporal Arteritis?
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The treatment option used for patients with severe renal artery stenosis is __________.
The treatment option used for patients with severe renal artery stenosis is __________.
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Match the following features with their corresponding arteritis:
Match the following features with their corresponding arteritis:
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Which of the following is NOT a category of ANCA-associated vasculitis?
Which of the following is NOT a category of ANCA-associated vasculitis?
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The target antigens involved in ANCA-associated vasculitis include Proteinase 3 (PR3) and Myeloperoxidase (MPO).
The target antigens involved in ANCA-associated vasculitis include Proteinase 3 (PR3) and Myeloperoxidase (MPO).
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What triggers the antibody-mediated process in ANCA-associated vasculitis?
What triggers the antibody-mediated process in ANCA-associated vasculitis?
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ANCA associates with neutrophils to activate the __________ complement pathway.
ANCA associates with neutrophils to activate the __________ complement pathway.
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Match the steps in the ANCA-mediated process to their descriptions:
Match the steps in the ANCA-mediated process to their descriptions:
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Which of the following conditions is NOT a possible diagnosis based on the chest X-ray findings?
Which of the following conditions is NOT a possible diagnosis based on the chest X-ray findings?
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Microscopic Polyangiitis (MPA) shows evidence of granulomas.
Microscopic Polyangiitis (MPA) shows evidence of granulomas.
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What is the demographic characteristic of patients most commonly affected by Microscopic Polyangiitis?
What is the demographic characteristic of patients most commonly affected by Microscopic Polyangiitis?
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The primary renal manifestation of Microscopic Polyangiitis is __________.
The primary renal manifestation of Microscopic Polyangiitis is __________.
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Match the following symptoms with their corresponding classification in Microscopic Polyangiitis:
Match the following symptoms with their corresponding classification in Microscopic Polyangiitis:
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Which of the following features corresponds to a positive score in the Granulomatosis with Polyangiitis (GPA) criteria?
Which of the following features corresponds to a positive score in the Granulomatosis with Polyangiitis (GPA) criteria?
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Microscopic Polyangiitis (MPA) has a positive correlation for an eosinophil count of ≥1 x 10^6/L.
Microscopic Polyangiitis (MPA) has a positive correlation for an eosinophil count of ≥1 x 10^6/L.
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What is the required total score for classification in Granulomatosis with Polyangiitis (GPA)?
What is the required total score for classification in Granulomatosis with Polyangiitis (GPA)?
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Churg-Strauss Syndrome is characterized by necrotizing vasculitis and __________ necrosis.
Churg-Strauss Syndrome is characterized by necrotizing vasculitis and __________ necrosis.
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Match the following conditions with their primary features:
Match the following conditions with their primary features:
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Which of the following is considered the gold standard for assessing vascular conditions?
Which of the following is considered the gold standard for assessing vascular conditions?
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MR angiography is the only method used for diagnosing vascular issues.
MR angiography is the only method used for diagnosing vascular issues.
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What is the primary purpose of conducting a CT angiogram?
What is the primary purpose of conducting a CT angiogram?
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Which of the following treatments is preferred for patients with Systemic Lupus Erythematosus (SLE) instead of Azathioprine?
Which of the following treatments is preferred for patients with Systemic Lupus Erythematosus (SLE) instead of Azathioprine?
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The presence of a ________ can indicate vascular disease when examining the subclavian or aortic region.
The presence of a ________ can indicate vascular disease when examining the subclavian or aortic region.
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Match the imaging technique with its primary characteristic:
Match the imaging technique with its primary characteristic:
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Cyclophosphamide can be administered either intravenously or orally with the same efficacy.
Cyclophosphamide can be administered either intravenously or orally with the same efficacy.
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Name one indication for plasma exchange in the treatment of GPA or MPA.
Name one indication for plasma exchange in the treatment of GPA or MPA.
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The combination of low dose steroid and __________ is used for the maintenance phase of GPA and MPA treatment.
The combination of low dose steroid and __________ is used for the maintenance phase of GPA and MPA treatment.
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Match the following features with their corresponding type of vasculitis:
Match the following features with their corresponding type of vasculitis:
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What condition is most commonly associated with both nasal septal ulcers and crusting?
What condition is most commonly associated with both nasal septal ulcers and crusting?
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Cough and hemoptysis are symptoms of diffuse alveolar hemorrhage.
Cough and hemoptysis are symptoms of diffuse alveolar hemorrhage.
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What is the primary renal manifestation associated with rapidly progressive glomerulonephritis?
What is the primary renal manifestation associated with rapidly progressive glomerulonephritis?
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Recurrent epistaxis is a major systemic manifestation associated with _______ tract involvement.
Recurrent epistaxis is a major systemic manifestation associated with _______ tract involvement.
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Match the minor systemic manifestations with their respective descriptions:
Match the minor systemic manifestations with their respective descriptions:
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What does a positive result for True C-ANCA suggest?
What does a positive result for True C-ANCA suggest?
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Indirect immunofluorescence can show two patterns associated with antibodies.
Indirect immunofluorescence can show two patterns associated with antibodies.
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Name one drug that can induce vasculitis.
Name one drug that can induce vasculitis.
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The confirmation test for vasculitis is called __________.
The confirmation test for vasculitis is called __________.
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Which pattern is associated with Anti-PR3 antibody?
Which pattern is associated with Anti-PR3 antibody?
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Match the following types of vasculitis with their associated antibodies:
Match the following types of vasculitis with their associated antibodies:
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Granuloma is a definitive finding in necrotizing vasculitis.
Granuloma is a definitive finding in necrotizing vasculitis.
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What is a characteristic positive finding in necrotizing vasculitis?
What is a characteristic positive finding in necrotizing vasculitis?
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Which feature is considered a hallmark of small vessel vasculitis?
Which feature is considered a hallmark of small vessel vasculitis?
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Granulomatosis with Polyangiitis is more common in males than females.
Granulomatosis with Polyangiitis is more common in males than females.
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What type of vessel is primarily involved in medium vessel vasculitis?
What type of vessel is primarily involved in medium vessel vasculitis?
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The type of antibodies predominantly associated with Granulomatosis with Polyangiitis is _____.
The type of antibodies predominantly associated with Granulomatosis with Polyangiitis is _____.
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Match the types of vasculitis with their corresponding features:
Match the types of vasculitis with their corresponding features:
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What is one of the features of large vessel vasculitis?
What is one of the features of large vessel vasculitis?
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In small vessel vasculitis, non-blanchable pin-point macules develop due to capillary inflammation.
In small vessel vasculitis, non-blanchable pin-point macules develop due to capillary inflammation.
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At what age group does Granulomatosis with Polyangiitis commonly occur?
At what age group does Granulomatosis with Polyangiitis commonly occur?
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Mononeuritis multiplex is a feature of _____ vessel vasculitis.
Mononeuritis multiplex is a feature of _____ vessel vasculitis.
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Which of the following is NOT a feature of small vessel vasculitis?
Which of the following is NOT a feature of small vessel vasculitis?
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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
- Hepatitis B: 30/1 rule. 30% of PAN cases have Hepatitis B. 1% of Hepatitis B cases have PAN.
- 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.
- Constitutional symptoms:
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.
