Trivia On Vasculitis: MCQ Quiz! Test

1. 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?

Anti-HIV antibodies

C-ANCA

Blood and urine Histoplasma antigen

Anti-glomerular basement membrane antibodies

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.

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.

2. 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?

Polyarteritis nodosa

Microscopic polyangiitis

Chrurg-Strauss syndrome

Henoch-Schonlein purpura

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.

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.

3. 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?

Polyarteritis nodosa

Microscopic polyangiitis

Rheumatic fever

Subacute bacterial endocarditis

Microscopic Polyangiitisp-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

Explanation

Microscopic Polyangiitisp-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

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

IgM immune complexes that induce vaculitis

Macrophage activation that triggers cytokine secretion with subsequent vascular leakage

Histamine release by mast cells in the skin

IgA immune complexes that induce vasculitis

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

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

5. 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?

Large vessel giant cell vasculitis with fragmentation of elastic lamina

Small vessel eosinophilic vasculitis

Medium vessel vasculitis with fibrinoid necrosis

Large vessel granulomatous vasculitis with massive intimal fibrosis

Medium vessel transmural vasculitis

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 ]]

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 ]]

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

Transmural inflammation with fibrinoid necrosis

Necrotizing granuloma

Eosinophilic granuloma

Granuloma with intimal proliferation and elastic fiber destruction

Microscopic Polyangiitisp-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)

Explanation

Microscopic Polyangiitisp-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)

7. 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?

Polyarteritis nodosa

Henoch Shonlein purpura

Microscopic polyangiitis

Churg-Strauss syndrome

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.

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.

8. 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?

Segmental necrotizing inflammation

Necrotizing inflammation with thrombosis and abscess formation

Necrotizing inflammation with eosinophilia

Granulomatous inflammation and AFBs

Granulomatous inflammation and treponemas

Granulomatous inflammation

(PATHOMA)--B. Takayasu Arteritis
1. Granulomatous vasculitis that classically involves the aortic arch at branch points
2. Presents in adults 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%)
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

Explanation

(PATHOMA)--B. Takayasu Arteritis
1. Granulomatous vasculitis that classically involves the aortic arch at branch points
2. Presents in adults 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%)
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