Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Rheumatology > 356 Vasculitis > Flashcards

356 Vasculitis Flashcards

1
Q

clinicopathologic process characterized y inflammation and damage to blood vessels

A

vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is the general pathophysiology of vasculitic syndromes

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

prominent hypothesized immune complexes in vasculitic syndromes

A

antineutrophil cytoplasmic antibodies (ANCA) and pathogenic T lymphocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

most widely accepted pathogenic of vasculitis

A

deposition of immune complexes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

hepatitis B antigen is associated with what systemic vasculitis

A

Polyarteritis nodosa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

vasculitis strongly associated with hepatitis C virus infection

A

cyroglobulinemic vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

disposition of immune complexes attract which compliment component

A

C5a which is strongly chemotactic to neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

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

A

ANCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Two major categories of ANCA

A

cytoplasmic ANCA, perinuclear ANCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

refers to ANCA with diffuse granular cytoplasmic staining

A

cytoplasmic ANCA or cANCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

major cANCA antigen

A

Proteinase 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

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

A

pANCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

major target of pANCA

A

myeloperoxidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

where does proteinase 3 and myeloperodase reside

A

azurophilic granules and lysosomes of resting neutrophils and monocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

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

A

IL-1 and TNF alpha

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

definitive diagnosis of vasculitis

A

biopsy of involved tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

cyclophasphamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

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

A

rituximab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

how to lessen methotraxate toxicity

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

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

A

true.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what is the typical lung involvement in granulamatosis with polyangitis

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

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

A

true.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

True or false. As with other forms of glomerulonephritis, immune complex deposition in granulomatosis polyangitis is found in renal lesions.

A

false.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

True or false. Chronic nasal carriage of Staph Aureus has been reported to be associated with higher relapse of granulomatosis polyangitis

A

true.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

True or faLse. Patient with granulamatosis with polyangitis have been found to ave increased incidence of venous thrombotic events but anticoagulation is not recommended.

A

true.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

How is granulatomsis with polyangitis diagnoses

A

necrotizing granulomatous vasculitis on tissue biopsy with compatible clinical features

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

what tissue biopsy yields the highest in granulomatosis with polyangitis

A

pulmonary tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

True or false. Presence of ANCA is comparable to tissue biopsy in granulomatosis with polyangitis

A

false. Presence of ANCA is adjunctive and should not substitute biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

mimicker of granulomatosis with polyangitis wherein there is extreme tissue destruction in sinuses and face

A

midline destructive disease such as those caused by upper airway neoplasm by extranodal natural killer cell/lymphoma (nasal type)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

mimicker of granulomatosis with polyangitis wherein patient presents with isolated midline destructive disease

A

cocaine induced tissue injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

differentiate between granulomatosis with polyangitis vs lymphomatoid granulomatosis

A

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
Q

treatment modality in granulomatosis with polyangitis leading to marked improvement in more than 90% of patients and complete remission in 75% of patients

A

cyclophasphamide

35
Q

True or false. ANCA titer may be used to assess disease activity

A

false. It can be misleading as patient with remission still have elevated ANCA titers

36
Q

Treatment approach in granulomatosis with polyangitis

A

two phases: induction and maintenance;

37
Q

What is the inducation phase treatment of granulamtosis with polyangitis

A

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
Q

when is plasmapharesis done

A

found to improve recovery in patients with creatinine greater than 5.8 mg/dl

39
Q

how is remission maintenance after cyclophosphamide done

A

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
Q

what is the optimal duration of maintenance therapy

A

usually given at a minimum of 2 years past remission after which consideration can be given for tapering over 6-12 month period

41
Q

chimeric monoclonal antibody directed against CD20 and used for induction of severe granulomatosis with polyangitis

A

rituximab

42
Q

what must be check prior to giving rituximab

A

hepatitis screening as it can bring about hepatitis B reactivation

43
Q

True or false. Methotrexate can be used together with glucocorticoids as alternative in the treatment of granulomatosis with polyangitis

A

true.

44
Q

True or false. Etanercept can be used as adjunctive therapy.

A

False. Etanercept has not been found to sustain remission when used as adjunct

45
Q

a dimeric fusion protein containing 75 kDa TNF receptor bound to IgG1

A

etanercept

46
Q

True or false. TMP SMX may be given to granulomatosis polyangitis with sinusoidal disease but not those with renal or pulmonary diease

A

true.

47
Q

disease manifestations of granulomatosis with polyangitis not responsive to systemic immunosuppresive treatment

A

subgliottic stenosis and endobronchial stenosis

48
Q

connote a necrotizing vasculitis with few or no immune complexes affecting small vessels such as the capillaries, venules or arterioles

A

microscopic polyarteritis

49
Q

how to differentiate microscopic polyarteritis with granulamatosis with polyangitis

A

in microscopic polyarteritis there is absence of granulomatous inflammation

50
Q

mean age of onset of microscopic polyarteritis

A

about age 57 with slightly more frequency in females

51
Q

prednisone dosage for polymyalgia rheumatica

A

10-20 mg/ day

52
Q

True or false. Arteriographic abnormalities are noted as much as 50% in coronary arteries in patients with Takayasu arteritis

A

False. Coronary artery are involved less than 10% of the time

53
Q

Most common affected artery in Takayasu arteriritis

A

subclavian artery at 93% followed by common carotid 58%

54
Q

inflammation and stenotic disease disease of medium and large-sized arteries characterized by a strong predilection for the aortic arch and its branches

A

takayasu arteritis

55
Q

commonly involved vessels in takayasu arteritis

A

disease involves medium and large sized arteries with a strong predilection for the aortic arch and its branches

56
Q

Frequently involved in the narrowing in takayasu arteritis

A

vasa vasorum

57
Q

True or false. In takuyasu arteritis pulses are commonly absent in the involved vessels particularly the subclavian artery

A

true.

58
Q

Typically clinical picture of takayasu arterities

A

young women who develops decrease or absence of peripheral pulses discrepancies in blood pressure and arterial bruits

59
Q

Imaging to help diagnose takayasu arteritis

A

complete aortic arteriography by catheter-directed dye

60
Q

What is the prognosis of takayasu arteritis

A

good with 5 year survival at 94%

61
Q

most common clinical manifestation of cyroglobulinermic vasculitis

A

cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis

62
Q

fundamental finding in cryoglobulinemic vasculitis

A

presence of circulating cryoprecipitates

63
Q

True or fase. Hypocomplementemia occurs in 90% of patients

A

true.

64
Q

treatment which provided evidence of benefit for cryoglobulinemic vasculitis

A

rituximab

65
Q

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

A

single organ vasculitis

66
Q

examples of single organ vasculitis

A

isolated aortitis, testicular vasculitis, vasculitis of the breast, isolated cutaneous vasculitis and primary CNS vasculitis

67
Q

defined broadly as inflammation of the blood vessels of the dermis

A

cutaneous vasculitis, hypersensitivity vasculitis, and cutaneous leukocytoclastic angiitis

68
Q

true or fasle. More than 70% of cases, cutaneous vasculitis occurs as part of a primary systemic vasculitis or as a secondary vasculitis

A

true.

69
Q

represents the most commonly encountered vasculitis in clinical practice

A

cutaneous vasculitis

70
Q

typical histopahologic feature of cutaneous vasculitis

A

presence of vasculitis of small vessels

71
Q

the most commonly involved vessels of idiopathic cutaneous vasculitis

A

postcapillary venules

72
Q

refers to the nuclear debris remaining from the neutrophils that have infiltrated in and around the vessels during the acute stages

A

leukocytoclasis

73
Q

hallmark of idiopathic cutaneous vasculitis

A

predominance of skin involvement

74
Q

uncommon clinicopathologic entity characterized by vasculitis restricted to the vessels of the CNS without apparent systemic vasculitis

A

primary CNS vasculitis

75
Q

how is primary CNS vasculitis confirmed

A

biopsy of brain or leptomeninges

76
Q

clinicopathologic entity characterized by recurrent episodes of oral and genital ulcers, iritis, and cutaneous lesions

A

Behcet disease

77
Q

characterized by interstitial keratitis together with vestibuloauditory symptoms

A

Cogans syndrome

78
Q

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

A

kawasaki disease

79
Q

treatment of kawasaki disease

A

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
Q

drugs that have been implicated in vasculitis

A

allopurinol, thiazides, gold, sulfonamide, phenytoin and penicillin

Rheumatology (15 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions