Vasculitis Flashcards
Vasculitis is an autoimmune inflammatory condition where immune cells mistake antigens on the endothelium for foreign antigens. What is the called?
1 - immune tolerance
2 - molecular mimicry
3 - cross reactivity
4 - all of the above
2 - molecular mimicry
In vasculitis damaged endothelium tries to repair itself. Which of the does not occur during this healing process?
1 - tissue factor and collagen are exposed to coagulation factors
2 - increased risk of coagulation
3 - walls become thinner and weaker, increasing risk of aneurysms
4 - fibrin is deposited into vessel walls
5 - walls become more elastic
5 - walls become more elastic
- walls actually become stiffer due to all the other factors such as fibrin deposits
Which of the following is NOT a general symptom of vasculitis?
1 - fever
2 - weight loss
3 - anaemia
4 - fatigue
5 - myalgia/arthralgia
3 - anaemia
There are generic symptoms patients with vasculitis can present with, such as fever, weight loss and fatigue. Which of the following specific organs can be affected?
1 - ENT = nasal bridge collapse
2 - Skin = purpura rash (non-blanching), can cause necrosis
3 - Oral = mucus membrane ulcers, necrosis and haemorrhage in gums
4 - Neural = cerebral vasculitis (white matter on T2 scan)
5 - all of the above
5 - all of the above
Vasculitis is an autoimmune inflammatiion of the blood vessels. Vasculitis can be classified into 3 categories. Which of the following is NOT one of these categories?
1 - Large Vessel
2 - Medium Vessel
3 - Moderate Vessel
4 - Small Vessel
3 - Moderate Vessel
- Large Vessel = Takayasu’s, Giant Cell arteries
- Medium Vessel = Polyarteritis nodosa, Kawasaki’s
- Small = Henoch Schonlein purpura, EGPA and GPA, MPA
What is the most common vasculitis?
1 - Takayasu’s
2 - Giant Cell arteritis
2 - Polyarteritis nodosa
4 - Kawasaki’s
2 - Giant Cell arteritis
- form of large vessel vasculitis
Giant cell arteritis, a form of large vessel vasculitis is the most common vasculitis. What age does this occur in most commonly?
1 - 20-35 y/o
2 - 35-50 y/o
3 - >50 y/o
4 - >60 y/o
- > 50 years
- peak 70-79
Patients with giant cell arteritis can experience headaches and tenderness of the skull. Which blood vessel and its location is most commonly affected?
1 - internal carotid artery near the ear
2 - temporal artery at temple of head
3 - ophthalmic artery near eyes
4 - facial artery near mandible bone
2 - temporal artery at temple of head
Patients with giant cell arteritis can experience headaches and tenderness of the skull, with the temporal artery at temple of head most commonly affected. However, if the ophthalmic artery near eyes is affected what can this cause?
1 - blindness
2 - diplopia
3 - painless
4 - all of the above
4 - all of the above
- if not treated quickly with corticosteroids this can cause ischaemia and blindness
Patients with giant cell arteritis can experience headaches and tenderness of the skull, with the temporal artery at temple of head most commonly affected. However, if the facial artery near mandible bone is affected what can this cause?
1 - pain called claudication
2 - paralysis
3 - hypoesthesia
4 - all of the above
1 - pain called claudication
What blood measures can be elevated in giant cell arteritis, a form of large cell vasculitis?
1 - ESR and creatine kinase
2 - CRP and lactate dehydrogenase
3 - CRP and creatine kinase
4 - CRP and ESR
4 - CRP and ESR
- ESR is really high
If a biopsy was taken from a patient with suspected giant cell vasculitis we would see giant cells. What are these cells though?
1 - megakaryocytes
2 - lymphocytes grouped together
3 - reed-sternberg cells
4 - monocytes packed together
4 - monocytes packed together
- form a granuloma
- found in internal elastic lamina
In giant cell arteritis, a form of large vessel vasculitis why can ultrasound and/or biopsy not be definitive form of diagnosis when assessing the temporal artery?
1 - operator dependent skill set
2 - part of artery sampled may be healthy
3 - not sufficient to effectively diagnose
2 - part of artery sampled may be healthy
- vasculitis is segmental, so parts are healthy
- so may miss the damaged arteries
How can we diagnose vasculitis using imagery?
1 - biopsy or ultrasound
2 - MRI
3 - X-ray
4 - CT scan
1 - biopsy or ultrasound
How can we treat giant cell arteries?
1 - NSAIDS given immediately
2 - strong dose of steroids given immediately
3 - DMARDs given immediately
4 - biologics given immediately
2 - strong dose of steroids given immediately
Takayasus arteritis is a form of large vessel arteritis. The presentation is very similar to giant cell arteritis, except for which 2 key differences?
1 - affects asian women <40 y/o
2 - affects large vessels of legs only
3 - affects men <40 y/o
4 - affects the blood vessels branching off the aortic arch, specifically the branches
1 - affects asian women <40 y/o
4 - affects the blood vessels branching off the aortic arch, specifically the branches
Takayasus arteritis is a form of large vessel arteritis that typically affects the blood vessels branching off the aortic arch, specifically the branches. What symptoms can this present with?
1 - weak or lack of pulses in upper extremities
2 - visual symptoms
3 - neurological symptoms
4 - all of the above
4 - all of the above
If a biopsy was taken from a patient with suspected takayasus cell vasculitis we would see giant cells. What are these cells though?
1 - megakaryocytes
2 - lymphocytes grouped together
3 - reed-sternberg cells
4 - monocytes packed together
4 - monocytes packed together
- form a granuloma
- found in internal elastic lamina
- this is the same as in giant cell arteritis
In giant cell arteritis, a form of large cell vasculitis, why would we want to do a chest X-ray?
1 - ensure lungs are ok
2 - common carotid artery could be affected
3 - aorta could be inflamed
4 - check on the heart
3 - aorta could be inflamed
How can we treat takayasus cell arteries?
1 - NSAIDS given immediately
2 - strong dose of steroids given immediately
3 - DMARDs given immediately
4 - biologics given immediately
2 - strong dose of steroids given immediately
Polyarteritis nodosa (PAN) is a vasculitis that affects medium size blood vessels, particularly muscular arteries. In PAN immune cells damage the endothelium of blood vessels confusing the antigens present for what?
1 - hepatitis B
2 - bacteria
3 - hepatitis C
4 - multiple myeloma
1 - hepatitis B
Polyarteritis nodosa (PAN) is a vasculitis that affects medium size blood vessels, particularly muscular arteries. In PAN which layers of the blood vessels are affected?
1 - tunic intima
2 - tunica media
3 - tunica adventitia
4 - all layers
4 - all layers
- has a transmural effect like crohns
Polyarteritis nodosa (PAN) is a vasculitis that affects medium size blood vessels, particularly muscular arteries. In PAN there is a transmural affect of all layers. What then happens to the vessel wall?
1 - leads to vascular wall death
2 - fibrosis is laid down
3 - blood vessels become stiff called fibrinoid necrosis
4 - fibrinosed vessel wall becomes weak and is at risk of aneurysms
4 - fibrinosed vessel wall becomes weak and is at risk of aneurysms
Polyarteritis nodosa (PAN) is a vasculitis that affects medium size blood vessels, particularly muscular arteries. In PAN there is a transmural affect of all layers. This can cause vessel wall death that become stiff, narrow and at risk of aneurysm. Which of the following is an example of what PAN can cause?
1 - hypertension if renal arteries are involved
2 - mesenteric ischemia causing pain and bleeding if mesenteric artery is affected
3 - neurological symptoms if blood vessels to the brain are affected
4 - skin lesions if blood vessels supplying the skin are affected
5 - all of the above
5 - all of the above
- essentially can cause end organ ischaemia and the symptoms relate to this
How can we treat Polyarteritis nodosa (PAN) arteries?
1 - NSAIDS given immediately
2 - strong dose of steroids given immediately
3 - DMARDs given immediately
4 - biologics given immediately
2 - strong dose of steroids given immediately
Kawasaki arteritis is a form of medium vessel vasculitis. Which vessels are particularly affected in this condition?
1 - blood vessels of the brain
2 - respiratory blood vessels
3 - coronary blood vessels
4 - renal blood vessels
3 - coronary blood vessels
Kawasaki arteritis is a form of medium vessel vasculitis that predominantly affects the coronary blood vessels. All of the following can be caused EXCEPT which one?
1 - increased risk of coagulation and cardiac ischaemia
2 - weak artery walls due to fibrin deposits increasing aneurysms
3 - thicken blood vessels walls and narrowing lumen and inducing a heart attack
4 - valves become dysfunctional due to reduced blood flow
4 - valves become dysfunctional due to reduced blood flow
Kawasaki arteritis is a form of medium vessel vasculitis that predominantly affects the coronary blood vessels. What age group is Kawasaki arteritis most common in?
1 - children <5 y/o
2 - 15-30 y/o
3 - 20-40 y/o
4 - >50 y/o
1 - children <5 y/o
- boys are more at risk
Although Kawasaki arteritis is self-limiting, meaning it can correct itself, which of the following is not a typical presentation?
1 - aortic dissection
2 - conjunctivitis
3 - rash
4 - adenopathy (cervical)
5 - strawberry tongue
6 - swollen hands and feet
7 - fever
1 - aortic dissection
- CRASHS - burn is the mnemonic
Although Kawasaki arteritis is self-limiting, meaning it can correct itself. All of the following can occur, but how many of these are required to confirm a diagnosis?
- conjunctivitis
- rash
- adenopathy (cervical)
- strawberry tongue
- swollen hands and feet
- fever
1 - all of them
2 - >4 with a fever for 5 days
3 - >3
4 - >2 with a fever for 3 days
2 - >4 with a fever for 5 days
Kawasaki arteritis can cause all of the following except which one?
1 - aneamia
2 - raised WBC
3 - low ESR and CRP
4 - raised LFTs
5 - raised platelets
6 - mononuclear white blood cells in the urine without evidence of bacteria
3 - low ESR and CRP
- these are both typically high as there is inflammation
Which imaging modality can be useful in Kawasaki arteritis?
1 - X-ray
2 - MRI
3 - CT
4 - echocardiogram
4 - echocardiogram
- as Kawasaki arteritis mainly effects coronary blood vessels its important to check the heart
Typically which 2 are used to treat Kawasaki arteritis?
1 - aspirin
2 - strong dose of steroids given immediately
3 - IV immunoglobulins (donor antibodies)
4 - biologics given immediately
1 - aspirin
3 - IV immunoglobulins (donor antibodies)
- form of passive immunity
Kawasaki arteritis is typically treated using aspirin and IVIG (donor antibodies). Why does the patient need to be monitored very closely during treatment?
1 - reyes syndrome
2 - hypersensitivity reaction
3 - anaphylaxis
4 - heart attack
1 - reyes syndrome
- children taking aspirin are at risk of reyes syndrome
In small vessel vasculitis arterioles, capillaries and venules are affected. Which immune cell has been identified as a cause of small cell vasculitis?
1 - B cells
2 - T cells
3 - NK cells
4 - neutrophils
1 - B cells
- specifically targets granuloes (neutrophils, basinophils and eosinophils)
In small vessel vasculitis arterioles, capillaries and venules are affected. B cells produce antibodies against granuoles (neutrophils, basophils and eosinophils). What are these antibodies called?
1 - granuoles antibodies
2 - rheumatoid factor
3 - anti-neutrophilic cytoplasmic antibodies (ANCAs)
4 - MHC-I antibodies
3 - anti-neutrophilic cytoplasmic antibodies (ANCAs)
In small vessel vasculitis arterioles, capillaries and venules are affected. B cells produce antibodies against granuoles called anti-neutrophilic cytoplasmic antibodies (ANCAs). What is the most common form of antibody produced?
1 - IgA
2 - IgG
3 - IgM
4 - IgD
2 - IgG
Granulomatosis with polyangiitis (inflammation of multiple blood vessels) (GPA), previously referred to as Wegners Granulomatosis, is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called what?
1 - cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs)
2 - rheumatoid factor
3 - anti-neutrophilic cytoplasmic antibodies (ANCAs)
4 - MHC-I antibodies
1 - cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs)
Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). cANCAs bind with a specific antigen on granules called what?
1 - MHC-I
2 - elastin
3 - proteinase-3
4 - lipoprotein-3
3 - proteinase-3
- cANCA binding to proteinase-3 causes cytokines to be released onto endothelium causing vasculitis
Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). Which age group is typically affected by GPA?
1 - children <5 y/o
2 - 15-30 y/o
3 - 40-50 y/o
4 - >65 y/o
3 - 40-50 y/o
- typically middle aged men
Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). Which of the following tissue is NOT typically affected by GPA?
1 - nasopharynx
2 - heart
3 - lungs
4 - kidneys
2 - heart
Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). Which of the following is NOT a symptom patients typically present with?
1 - blood mucus from nose
2 - pain from sinusitis
3 - saddle nose
4 - SOB with blood on cough
5 - increased BP and urine output
5 - increased BP and urine output
- causes increased BP and and reduced urine output
Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). Which 2 of the following are used to treat is GPA?
1 - aspirin
2 - strong dose of steroids given immediately
3 - IVIG (donor antibodies)
4 - methotrexate
2 - strong dose of steroids given immediately
- corticosteroids
4 - methotrexate
- relapse is common because cANCA remains
A very similar form of small vessel vasculitis to Granulomatosis with polyangiitis (GPA) is microscopic polyangiitis (MP). What is the key difference of MT when compared to GPA?
1 - only females affected
2 - nasopharynx affected
3 - lungs and kidneys only affected
4 - granulomas present in vessel walls
5 - cANCAs present
3 - lungs and kidneys only affected
- nasopharynx NOT affected
- granulomas NOT present in vessel walls
- cANCAs NOT present, instead perinuclear anti-neutrophilic cytoplasmic antibodies (pANCAs) are present
Microscopic polyangiitis (MP) is a small vessel vasculitis. MP results in a specific type of auto antibody to be released called perinuclear anti-neutrophilic cytoplasmic antibodies (pANCAs). pANCAs bind with a specific antigen on granules called what?
1 - myeloperoxidase
2 - elastin
3 - proteinase-3
4 - lipoprotein-3
1 - myeloperoxidase
Microscopic polyangiitis (MP) is a small vessel vasculitis. MP results in a specific type of auto antibody to be released called perinuclear anti-neutrophilic cytoplasmic antibodies (pANCAs). Which 2 of the following are used to treat is GPA?
1 - aspirin
2 - strong dose of steroids given immediately
3 - IVIG (donor antibodies)
4 - methotrexate
2 - strong dose of steroids given immediately
- corticosteroids
4 - methotrexate
Pulmonary-renal syndrome (PRS) is used to describe the combination of glomerulonephritis and pulmonary haemorrhage as a manifestation of a multisystem autoimmune disease. PRS can then be subdivided into 2 categories based on what?
1 - CRP levels
2 - ESR levels
3 - haematuria
4 - anti-neutrophilic cytoplasmic antibodies (ANCAs)
4 - anti-neutrophilic cytoplasmic antibodies (ANCAs)
- PRS is very dangerous as patients can deteriorate in <48h
Pulmonary-renal syndrome (PRS) is used to describe the combination of glomerulonephritis and pulmonary haemorrhage as a manifestation of a multisystem autoimmune disease. PRS can then be subdivided into 2 categories based on the presence of anti-neutrophilic cytoplasmic antibodies (ANCAs). Which of the following would NOT be ANCA positive?
1 - Granulomatosis with polyangiitis
2 - Microscopic polyangiitis
3 - Systemic Lupus Erythematous
4 - Churg strauss syndrome
5 - Drug associated
3 - Systemic Lupus Erythematous
Patients with vasculitis can develop renal manifestations. What % of patients with vasculitis present with renal involvement?
1 - 2%
2 - 20%
3 - 45%
4 - 80%
2 - 20%
- BUT if untreated 80% of patients with vasculitis will develop glomerulonephritis
20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis. Which of the blood vessels is mainly affected?
1 - large vessels
2 - medium vessels
3 - small vessels
4 - all are affected
3 - small vessels
20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). Is this always symptomatic?
- no
- includes a range from normal eGFR to AKI to rapid progressing glomerulonephritis with need for dialysis
- can also see Pauci-immune crescentic GN
20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). How can these patients be diagnosed effectively?
1 - presence of anti-neutrophilic cytoplasmic antibody (ANCA)
2 - renal biopsy (Pauci-immune crescentic GN) histology
3 - imaging (CT sinuses, HRCT, CT brain)
4 - bronchoscopy, Lung function tests, nerve conduction studies
5 - all of the above
5 - all of the above
- typically patient presents as someone who just isn’t getting better, but other multi-systemic features that don’t align
20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). Which 2 of the following is important to rule out when thinking of a diagnosis of GN in a patient with vasculitis?
1 - CHF
2 - pulmonary hypertension
3 - TB
4 - infective endocarditis
3 - TB
4 - infective endocarditis
- essentially identify the cause of infection and treat
- then see if symptoms persist
20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). The 1st line treatment is induction immunosuppression. Which 2 of the following treatment options are used for 3 months to induce remission as part of the induction immunosuppression?
1 - high dose of steroids
2 - cyclophosphamide
3 - azathioprine
4 - mycophenolate mofetil
1 - high dose of steroids
2 - cyclophosphamide
20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). Following induction immunosuppression with 3 months of high dose steroids and cyclophosphamide. Which 2 of the following treatment options are used in maintenance, due to the high risk of relapse?
1 - methotrexate
2 - opioids
3 - azathioprine
4 - mycophenolate Mofetil
3 - azathioprine
4 - mycophenolate Mofetil
- retuximan should be considered if patient continues to relapse (depletes B and plasma cells)
- plasma exchange can be considered if creatinine is >500
What is the mortality rate in 1 year if patients with vasculitis and glomerulonephritis (GN) if left untreated?
1 - 100%
2 - 50%
3 - 25%
4 - 5%
1 - 100%
What is another condition that can cause pulmonary-renal syndrome?
1 - TB
2 - Good Pastures Syndrome
3 - SLE
4 - Infective endocarditis
2 - Good Pastures Syndrome
- autoimmune inflammatory condition affecting the lungs and kidneys
- causes haemoptysis in lungs and haematuria in the kidneys
- typically triggered by infection or agent
Good pastures syndrome (GPS) is able to cause pulmonary-renal syndrome. What is the trigger for GPS?
1 - IgE from mast cells is released and binds to type IV collagen
2 - IgG/IgM antibodies bind to type IV collagen
3 - antibody-immune complexes forms on type IV collagen
4 - cytotoxic T cells target and begin to degrade type IV collagen
2 - IgG/IgM antibodies bind to type IV collagen
- type 2 hypersensitivity that triggers the complement pathway
- type IV collagen is present in abundance in the basement membranes of the glomerulus and alveolar basement membranes
In good pastures syndrome (GPS), which can causes pulmonary-renal syndrome, the Fab region of the antibody binds to type IV collagen. The Fc portion then binds with what to trigger the complement pathway?
1 - C1
2 - C2
3 - C3
4 - C4
1 - C1
- complement pathway is then activated
In good pastures syndrome (GPS), which can causes pulmonary-renal syndrome, once the complement pathway is activated, C3a, 4a and 5a act as chemotactic agents and attract which cell?
1 - basophils
2 - cytotoxic T cells
3 - B cells
4 - neutrophils
4 - neutrophils
In good pastures syndrome (GPS), which can causes pulmonary-renal syndrome, once the complement pathway is activated, C3a, 4a and 5a act as chemotactic agents and attract neutrophils. What do the neutrophils then release that damages the collagen and therefore the basement membranes?
1 - peroxidase
2 - myeloperoxidase
3 - proteinase-3
4 - all of the above
4 - all of the above
- all cause free oxygen radicals that form and damage the basement membranes
Which of the following is NOT a common symptom in good pastures syndrome (GPS)?
1 - dysponea/cough
2 - glomerularnephritis
3 - proteinuria >3.5g
4 - haemoptysis
3 - proteinuria >3.5g
Which of the following antibody can be screened for to diagnose good pastures syndrome (GPS)?
1 - anti-glomerular basement (GBM)
2 - anti-neutrophil cytoplasmic antibodies (ANCA)
3 - cytoplasmic-ANCA
4 - proteinase 3-ANCA
1 - anti-glomerular basement (GBM)
How is good pastures syndrome (GPS) typically treated?
1 - high dose of steroids
2 - cyclophosphamide
3 - plasmapheresis
4 - all of the above
4 - all of the above
- typically does not relapse and is a 1 hit wonder
