Trivia On Vasculitis: MCQ Quiz! Test

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Trivia On Vasculitis: MCQ Quiz! Test - Quiz


Have you ever heard of vasculitis? Vasculitis is a condition that is typified by inflammation in the blood vessels. It is autoimmune and can lead to life-threatening complications if not adequately treated. This group of different types of vasculitis can lead to organ and tissue damage, especially if it affects the brain, lungs, kidneys, or other vital areas of concern. All the best of luck with this quiz, lets see how much you know.


Questions and Answers
  • 1. 

    A 57-year-old woman with unilateral headache, vision loss, and "morning joint stiffness" has a biopsy of her temporal artery done in your office.  What are the histologic findings you would expect to see in the biopsy?

    • A.

      Large vessel giant cell vasculitis with fragmentation of elastic lamina

    • B.

      Small vessel eosinophilic vasculitis

    • C.

      Medium vessel vasculitis with fibrinoid necrosis

    • D.

      Large vessel granulomatous vasculitis with massive intimal fibrosis

    • E.

      Medium vessel transmural vasculitis

    Correct Answer
    A. Large vessel giant cell vasculitis with fragmentation of elastic lamina
    Explanation
    Giant Cell (Temporal) Arteritis: Histology: granulomatous inflammation Media and intima Multinucleated giant cells Necrosis and fragmentation of the *******internal elastic membrane**** Later stage: intimal thickening and medial fibrosis Clinical Manifestations Superficial temporal artery is thickened, swollen and tender Unilateral headache Ipsilateral visual loss Polymyalgia rheumatica (pain and morning stiffness in the neck, shoulders, and hips) Fever, malaise Diagnosed by temporal artery biopsy Treat with anti-inflammatory drugs (corticosteroids) Prognosis is good subsides in 6 to 12 months if left untreated  blindness _______________________________________________________ Takayasu Arteritis: granulomatous inflammation with [[ massive intimal fibrosis ]] aortic arch and its major branches Gross appearance Irregular thickening (intimal fibrosis) of the vascular wall Intimal wrinkling Narrowing of the orifices of major arterial branches (carotid, subclavian) Microscopic appearance Giant cell granulomas Transmural mononuclear inflammation Later changes: [[ fibrosis and intimal proliferation ]]

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  • 2. 

    A 64-year-old Caucasian male is admitted to the hospital with fatigue, cough, hemoptysis, and difficulties with nasal breathing.  At admission, his vital signs are as follows: temperature of 38.1 C, the pulse of 114 bpm, BP of 145/95 mm Hg, and RR of 22 breaths/min.  Physical examination reveals moderate puffiness of the face and dullness on percussion and reduced breathing over the left lung. Chest x-ray examination reveals several cavitating opacities in the left lung. Urinalysis detects hematuria and mild proteinuria. PPD test reading in 72 hours reveals 5-mm erythema; sputum microscopy for AFB is negative. Two images below represent gross and microscopic appearance of the patient’s lung. Which of the following laboratory tests is most likely to be positive in this patient?

    • A.

      Anti-HIV antibodies

    • B.

      C-ANCA

    • C.

      Blood and urine Histoplasma antigen

    • D.

      Anti-glomerular basement membrane antibodies

    Correct Answer
    B. C-ANCA
    Explanation
    SMALL-VESSEL VASCULITIS A. Wegener Granulomatosis l. Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys 2. Classic presentation is a middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis. 3. Serum c-ANCA levels correlate with disease activity. 4. Biopsy reveals large necrotizing granulomas with adjacent necrotizing vasculitis (Fig. 7.4). 5. Treatment is cyclophosphamide and steroids; relapses are common. B. Microscopic Polyangiitis l. Necrotizing vasculitis involving multiple organs, especially lung and kidney 2. Presentation is similar to Wegener granulomatosis, but nasopharyngeal involvement and granulomas are absent. 3. Serum p-ANCA levels correlate with disease activity. 4. Treatment is corticosteroids and cyclophosphamide; relapses are common. C. Churg-Strauss Syndrome 1. Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart 2. Asthma and peripheral eosinophilia are often present. 3. Serum p-ANCA levels correlate with disease activity.

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  • 3. 

    Histologic examination of a renal biopsy reveals necrotic changes of glomerular capillaries and proliferation of the epithelium of the Bowman capsule. Which of the following diseases does the patient most likely have?

    • A.

      Polyarteritis nodosa

    • B.

      Henoch Shonlein purpura

    • C.

      Microscopic polyangiitis

    • D.

      Churg-Strauss syndrome

    Correct Answer
    C. Microscopic polyangiitis
    Explanation
    SMALL-VESSEL VASCULITIS A. Wegener Granulomatosis l. Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys 2. The classic presentation is a middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis. 3. Serum c-ANCA levels correlate with disease activity. 4. Biopsy reveals large necrotizing granulomas with adjacent necrotizing vasculitis 5. Treatment is cyclophosphamide and steroids; relapses are common. ****************B. Microscopic Polyangiitis ****************** l. Necrotizing vasculitis involving multiple organs, especially lung and kidney 2. Presentation is similar to Wegener granulomatosis, but nasopharyngeal involvement and granulomas are absent. 3. Serum p-ANCA levels correlate with disease activity. 4. Treatment is corticosteroids and cyclophosphamide; relapses are common. C. Churg-Strauss Syndrome 1. Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart 2. Asthma and peripheral eosinophilia are often present. 3. Serum p-ANCA levels correlate with disease activity.

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  • 4. 

    A 56-year-old Hispanic male presents to the outpatient clinic with reddish-blue lesions on his lower extremities, fever, muscle pain, and weight loss. He reports a history of acute viral hepatitis B three months ago. Physical examination reveals multiple red-purple nodules on the skin of both legs. Laboratory results for P- and C-ANCA are negative. Urinalysis reveals hematuria and proteinuria. Abdominal ultrasound reveals few 1 – 2 cm fluid-filled cavities in both kidneys. Which of the following is the most likely diagnosis?

    • A.

      Polyarteritis nodosa

    • B.

      Microscopic polyangiitis

    • C.

      Chrurg-Strauss syndrome

    • D.

      Henoch-Schonlein purpura

    Correct Answer
    A. Polyarteritis nodosa
    Explanation
    MEDIUM-VESSEL VASCULITIS A. POLYARTERITIS NODOSA 1. Necrotizing vasculitis involving multiple organs; lungs are spared. 2. Classically presents in young adults as hypertension (renal artery involvement), abdominal pain with melena (mesenteric artery involvement), neurologic disturbances, and skin lesions. ******Associated with serum HBsAg ****** 3. Lesions of varying stages are present. Early lesion consists of transmural inflammation with fibrinoid necrosis; eventually heals with fibrosis, producing a 'string-of-pearls' appearance on imaging 4. Treatment is corticosteroids and cyclophosphamide; fatal if not treated B. KAWASAKI DISEASE l. Classically affects Asian children < 4 years old 2. Presents with nonspecific signs including fever, conjunctivitis, erythematous rash of palms and soles, and enlarged cervical lymph nodes 3. Coronary artery involvement is common and leads to risk for (l) thrombosis with myocardial infarction and (2) aneurysm with rupture. 4. Treatment is aspirin and IVTG; disease is self-limited. C. BUERGER DISEASE l. Necrotizing vasculitis involving digits 2. Presents with ulceration, gangrene, and autoamputation of fingers and toes; Raynaud phenomenon is often present. 3. Highly associated with heavy smoking; treatment is smoking cessation.

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  • 5. 

    A 47-year-old Caucasian male seeks medical care because of transient hematuria and hemoptysis of one week duration. He had an acute bacterial pneumonia five weeks ago and from that time he has not felt well. Two weeks ago the patient noticed some bloody nasal discharge. He also complains that his left knee has been hurting and that red spots have appeared on his arms and legs. Physical examination reveals lower leg pitting edema, and many small, red, raised lesions on the skin of his extremities that are painless. BP is 150/95 mm Hg. Lab Findings:
    • Moderate leukocytosis with ordinary WBC differential count
    • UA: hematuria and proteinuria
    • Elevated serum P-ANCA level
    • Normal serum IgA level
    What is the most likely diagnosis?

    • A.

      Polyarteritis nodosa

    • B.

      Microscopic polyangiitis

    • C.

      Rheumatic fever

    • D.

      Subacute bacterial endocarditis

    Correct Answer
    B. Microscopic polyangiitis
    Explanation
    Microscopic Polyangiitis p-ANCA (MPO)
    Primarily affects:
    skin (purpura=rash)
    kidneys (glomerulonephritis)

    segmental necrotizing vasculitis
    *no granulomas*

    Age:  50 yrs
    M  F
    whites > blacks

    Thought to be hypersensitivity reaction to drugs, toxins, microbes, etc.

    respiratory tract (sinusitis, pneumonitis)
    GI tract
    Also known as:
    Hypersensitivity vasculitis
    Leukocytoclastic vasculitis
    Microscopic polyarteritis

    Vasculitis without asthma or granulomas
    *****Hypersensitivity reaction to drugs or toxins*************
    Antigens: drugs, e.g., aspirin, penicillin, thiazide diuretics, microorganisms, etc

    Segmental necrotizing vasculitis
    Polymorpho-/mononuclear infiltration
    Fibrinoid necrosis
    Kidneys: necrotizing and crescentic glomerulonephritis
    _________________________________________________________
    Clinical manifestations
    Palpable purpuric rash, lower extremities
    Nephritic syndrome
    Sinusitis, cough, hemoptysis
    +/- GI hemorrhage
    Treat by eliminating causative agent
    Good prognosis
    except in kidney and/or lung involvement

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  • 6. 

    What histological changes are most likely seen within the wall of affected vessels?

    • A.

      Transmural inflammation with fibrinoid necrosis

    • B.

      Necrotizing granuloma

    • C.

      Eosinophilic granuloma

    • D.

      Granuloma with intimal proliferation and elastic fiber destruction

    Correct Answer
    A. Transmural inflammation with fibrinoid necrosis
    Explanation
    Microscopic Polyangiitis p-ANCA (MPO)
    Vasculitis without asthma or granulomas
    Hypersensitivity reaction to drugs or toxins
    Antigens: drugs, e.g., aspirin, penicillin, thiazide diuretics, microorganisms, etc

    segmental necrotizing vasculitis
    **********no granulomas***************

    hypersensitivity reaction to drugs, toxins, microbes, etc. (rash)

    p-ANCA (MPO)

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  • 7. 

    A 34-year old Caucasian female presents with malaise, fever, arm pain, loss of appetite, and blurred vision. Physical examination reveals a diminished pulsation and low BP on the upper extremities; pulses and BP pressure on the lower extremities are WNL. Which of the following are the most likely morphologic changes within the affected arteries?

    • A.

      Segmental necrotizing inflammation

    • B.

      Necrotizing inflammation with thrombosis and abscess formation

    • C.

      Necrotizing inflammation with eosinophilia

    • D.

      Granulomatous inflammation and AFBs

    • E.

      Granulomatous inflammation and treponemas

    • F.

      Granulomatous inflammation

    Correct Answer
    F. Granulomatous inflammation
    Explanation
    (PATHOMA)--B. Takayasu Arteritis
    1. Granulomatous vasculitis that classically involves the aortic arch at branch points
    2. Presents in adults< 50 years old (classically, young Asian females) as visual and neurologic symptoms with a weak or absent pulse in the upper extremity ('pulseless disease'). ESR is elevated.
    3. Treatment is corticosteroids.

    (CONRANS ppt)
    Takayasu Arteritis: "pulseless disease"
    granulomatous inflammation with massive intimal fibrosis
    aortic arch and its major branches
    F > M (F ≈ 90%)
    < 40 (30) yrs of age
    Asians
    Mechanism: ?immune
    Gross appearance
    Irregular thickening (intimal fibrosis) of the vascular wall
    Intimal wrinkling
    Narrowing of the orifices of major arterial branches (carotid, subclavian)
    Microscopic appearance
    Giant cell granulomas
    Transmural mononuclear inflammation
    Later changes: fibrosis and intimal proliferation
    Clinical manifestations
    Related to decreased blood flow through carotids and subclavians ("pulseless disease")

    Treat with corticosteroids

    Prognosis is usually good
    Worse with stroke or aortic dissection
    Granulomatous inflammation

    Carotid, subclavian (aortic arch)

    Females

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  • 8. 

    Henoch-Schonlein purpura frequently follows a respiratory tract infection. What is the reason for the purpuric rash?

    • A.

      IgM immune complexes that induce vaculitis

    • B.

      Macrophage activation that triggers cytokine secretion with subsequent vascular leakage

    • C.

      Histamine release by mast cells in the skin

    • D.

      IgA immune complexes that induce vasculitis

    Correct Answer
    D. IgA immune complexes that induce vasculitis
    Explanation
    D. Henoch-Schonlein Purpura
    1. Vasculitis due to lgA immune complex deposition; most common vasculitis in
    children
    2. Presents with palpable purpura on buttocks and legs, GI pain and bleeding, and
    hematuria (IgA nephropathy); usually occurs following an upper respiratory
    tract infection
    3. Disease is self-limited, but may recur; treated with steroids, if severe

    IgA associated vasculitis
    Children (most common vasculitis in kids)
    Follows infection (viral, Strep A)
    IgA and complement immune deposits
    Involves skin, GI, renal, joints
    Self-limited

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  • Current Version
  • Mar 22, 2023
    Quiz Edited by
    ProProfs Editorial Team
  • Jul 16, 2012
    Quiz Created by
    Chachelly
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