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356 Vasculitis Flashcards

1
Q

clinicopathologic process characterized y inflammation and damage to blood vessels

A

vasculitis

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2
Q

what is the general pathophysiology of vasculitic syndromes

A

mediated by immunopathogenic mechanisms that occur in response to certain antigenic stimuli

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3
Q

prominent hypothesized immune complexes in vasculitic syndromes

A

antineutrophil cytoplasmic antibodies (ANCA) and pathogenic T lymphocyte

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4
Q

most widely accepted pathogenic of vasculitis

A

deposition of immune complexes

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5
Q

hepatitis B antigen is associated with what systemic vasculitis

A

Polyarteritis nodosa

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6
Q

vasculitis strongly associated with hepatitis C virus infection

A

cyroglobulinemic vasculitis

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7
Q

disposition of immune complexes attract which compliment component

A

C5a which is strongly chemotactic to neutrophils

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8
Q

antibodies directed against certain proteins in the cytoplasmic granules of neutrophils and monocytes

A

ANCA

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9
Q

Two major categories of ANCA

A

cytoplasmic ANCA, perinuclear ANCA

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10
Q

refers to ANCA with diffuse granular cytoplasmic staining

A

cytoplasmic ANCA or cANCA

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11
Q

major cANCA antigen

A

Proteinase 3

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12
Q

refers to ANCA with more localized perinuclear or nuclear staining pattern in indicator neutrophils

A

pANCA

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13
Q

major target of pANCA

A

myeloperoxidase

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14
Q

where does proteinase 3 and myeloperodase reside

A

azurophilic granules and lysosomes of resting neutrophils and monocytes

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15
Q

cytokines that enhance adhesion of leukocytes to endothelial cells in the blood vessel wall

A

IL-1 and TNF alpha

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16
Q

definitive diagnosis of vasculitis

A

biopsy of involved tissue

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17
Q

drug used in vasculitis. Major toxic side effect. Bladder carcinoma

A

cyclophasphamide

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18
Q

drugs used in vasculitis. Major toxic side effect. Hepatitis B reactivation

A

rituximab

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19
Q

how to lessen methotraxate toxicity

A

folic acid 1 mg daily or folinic acid 5-10 mg once a week following methothrexate

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20
Q

True or false. Patients on immunosuppresive therapy with vasculitis should take TMP SMX as prophylaxis for PCP infection

A

true.

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21
Q

distinct clinicopathologic entity characterized by granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis

A

granulomatosis with polyangitis or Wegener’s

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22
Q

histopathologic hallmarks of granulomatosis with polyangitis

A

necrotizing vasculitis or small arteries and veins together with granuloma formation, which may be either intravascular or extravascular

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23
Q

what is the typical lung involvement in granulamatosis with polyangitis

A

lung involvement typically appears as multiple, bilateral, nodular and cavitary infiltrates

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24
Q

True or false. Granuloma formation on renal biopsy in wegener’s or granulomatosis polyangitis is rare

A

true.

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25
True or false. As with other forms of glomerulonephritis, immune complex deposition in granulomatosis polyangitis is found in renal lesions.
false.
26
True or false. Chronic nasal carriage of Staph Aureus has been reported to be associated with higher relapse of granulomatosis polyangitis
true.
27
True or faLse. Patient with granulamatosis with polyangitis have been found to ave increased incidence of venous thrombotic events but anticoagulation is not recommended.
true.
28
How is granulatomsis with polyangitis diagnoses
necrotizing granulomatous vasculitis on tissue biopsy with compatible clinical features
29
what tissue biopsy yields the highest in granulomatosis with polyangitis
pulmonary tissue
30
True or false. Presence of ANCA is comparable to tissue biopsy in granulomatosis with polyangitis
false. Presence of ANCA is adjunctive and should not substitute biopsy
31
mimicker of granulomatosis with polyangitis wherein there is extreme tissue destruction in sinuses and face
midline destructive disease such as those caused by upper airway neoplasm by extranodal natural killer cell/lymphoma (nasal type)
32
mimicker of granulomatosis with polyangitis wherein patient presents with isolated midline destructive disease
cocaine induced tissue injury
33
differentiate between granulomatosis with polyangitis vs lymphomatoid granulomatosis
in lymphomatoid granulomatosis there is lung, skin,CNS and kidney involvement in which atypical lymphocytoid and plasmacytoid cell infiltrate nonlymphoid tissue in an angioinvasive manner
34
treatment modality in granulomatosis with polyangitis leading to marked improvement in more than 90% of patients and complete remission in 75% of patients
cyclophasphamide
35
True or false. ANCA titer may be used to assess disease activity
false. It can be misleading as patient with remission still have elevated ANCA titers
36
Treatment approach in granulomatosis with polyangitis
two phases: induction and maintenance;
37
What is the inducation phase treatment of granulamtosis with polyangitis
Prednisone 1 mg/kg per day for first month with gradual tapering with discontinuation after 6- 9 months; this is given together with cyclophosphamide 2 mg/kg daily for 3 months
38
when is plasmapharesis done
found to improve recovery in patients with creatinine greater than 5.8 mg/dl
39
how is remission maintenance after cyclophosphamide done
After 3 months cyclophosphamide is swtiched to methotrexate or azathioprine; dosage of methotrexate is 0.3 mg/kg single weekly dose no to exceed 15 mg/week then increased to 2.5 mg/kg of up to 20-25 mg/week after 1-2 weeks; azathioprine dosage at 2 mg/kg per day
40
what is the optimal duration of maintenance therapy
usually given at a minimum of 2 years past remission after which consideration can be given for tapering over 6-12 month period
41
chimeric monoclonal antibody directed against CD20 and used for induction of severe granulomatosis with polyangitis
rituximab
42
what must be check prior to giving rituximab
hepatitis screening as it can bring about hepatitis B reactivation
43
True or false. Methotrexate can be used together with glucocorticoids as alternative in the treatment of granulomatosis with polyangitis
true.
44
True or false. Etanercept can be used as adjunctive therapy.
False. Etanercept has not been found to sustain remission when used as adjunct
45
a dimeric fusion protein containing 75 kDa TNF receptor bound to IgG1
etanercept
46
True or false. TMP SMX may be given to granulomatosis polyangitis with sinusoidal disease but not those with renal or pulmonary diease
true.
47
disease manifestations of granulomatosis with polyangitis not responsive to systemic immunosuppresive treatment
subgliottic stenosis and endobronchial stenosis
48
connote a necrotizing vasculitis with few or no immune complexes affecting small vessels such as the capillaries, venules or arterioles
microscopic polyarteritis
49
how to differentiate microscopic polyarteritis with granulamatosis with polyangitis
in microscopic polyarteritis there is absence of granulomatous inflammation
50
mean age of onset of microscopic polyarteritis
about age 57 with slightly more frequency in females
51
prednisone dosage for polymyalgia rheumatica
10-20 mg/ day
52
True or false. Arteriographic abnormalities are noted as much as 50% in coronary arteries in patients with Takayasu arteritis
False. Coronary artery are involved less than 10% of the time
53
Most common affected artery in Takayasu arteriritis
subclavian artery at 93% followed by common carotid 58%
54
inflammation and stenotic disease disease of medium and large-sized arteries characterized by a strong predilection for the aortic arch and its branches
takayasu arteritis
55
commonly involved vessels in takayasu arteritis
disease involves medium and large sized arteries with a strong predilection for the aortic arch and its branches
56
Frequently involved in the narrowing in takayasu arteritis
vasa vasorum
57
True or false. In takuyasu arteritis pulses are commonly absent in the involved vessels particularly the subclavian artery
true.
58
Typically clinical picture of takayasu arterities
young women who develops decrease or absence of peripheral pulses discrepancies in blood pressure and arterial bruits
59
Imaging to help diagnose takayasu arteritis
complete aortic arteriography by catheter-directed dye
60
What is the prognosis of takayasu arteritis
good with 5 year survival at 94%
61
most common clinical manifestation of cyroglobulinermic vasculitis
cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis
62
fundamental finding in cryoglobulinemic vasculitis
presence of circulating cryoprecipitates
63
True or fase. Hypocomplementemia occurs in 90% of patients
true.
64
treatment which provided evidence of benefit for cryoglobulinemic vasculitis
rituximab
65
defined as vasculitis in arteries or veins of any size in a single organ that has no features that indicate that is a limited expression of a systemic vasculitis
single organ vasculitis
66
examples of single organ vasculitis
isolated aortitis, testicular vasculitis, vasculitis of the breast, isolated cutaneous vasculitis and primary CNS vasculitis
67
defined broadly as inflammation of the blood vessels of the dermis
cutaneous vasculitis, hypersensitivity vasculitis, and cutaneous leukocytoclastic angiitis
68
true or fasle. More than 70% of cases, cutaneous vasculitis occurs as part of a primary systemic vasculitis or as a secondary vasculitis
true.
69
represents the most commonly encountered vasculitis in clinical practice
cutaneous vasculitis
70
typical histopahologic feature of cutaneous vasculitis
presence of vasculitis of small vessels
71
the most commonly involved vessels of idiopathic cutaneous vasculitis
postcapillary venules
72
refers to the nuclear debris remaining from the neutrophils that have infiltrated in and around the vessels during the acute stages
leukocytoclasis
73
hallmark of idiopathic cutaneous vasculitis
predominance of skin involvement
74
uncommon clinicopathologic entity characterized by vasculitis restricted to the vessels of the CNS without apparent systemic vasculitis
primary CNS vasculitis
75
how is primary CNS vasculitis confirmed
biopsy of brain or leptomeninges
76
clinicopathologic entity characterized by recurrent episodes of oral and genital ulcers, iritis, and cutaneous lesions
Behcet disease
77
characterized by interstitial keratitis together with vestibuloauditory symptoms
Cogans syndrome
78
acute febrile multisystem disease of children characterized by nonsuppurative cervical adenitis and changes in the skin and mucous membranes such as edema, congested conjunctivae, erythema of the oral cavity, lips and pals, and desquamation of the skin of the fingertips
kawasaki disease
79
treatment of kawasaki disease
IV gamma globulin 2 g/kg as single infusion over 10 hours together with aspirin 100 mg/kg per day for 14 days followed 3-5 mg/kg per day for several weeks
80
drugs that have been implicated in vasculitis
allopurinol, thiazides, gold, sulfonamide, phenytoin and penicillin

Rheumatology (15 decks)

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