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7th > Chapter 141- Adamantiades- Behcet Disease > Flashcards

Chapter 141- Adamantiades- Behcet Disease Flashcards

1
Q

Allele associated with more severe prognosis and ocular involvement

A

HLA B51

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2
Q

Viral agent associated with Behcet

A

HSV1

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3
Q

Sensitive marker of disease activity

A

IL8

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4
Q

Presenting sign in more than 80% of cases

A

Oral aphthous ulcers

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5
Q

Major cause of morbidity

A

Ocular involvement

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6
Q

International Criteria for Behcet Disease

A 
Ocular lesions (recurrent) - 2
Oral aphthosis (recurrent) - 2
Genital aphthosis (recurrent) - 2
Skin lesions (recurrent) - 1
CNS - 1
Vascular manifestations - 1
Positive pathergy test - 1
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7
Q

BCD scoring of ___ indicates Behcet

A

4

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8
Q

Positive pathergy test manifests within ____ hours as erythematous papule (> ___mm) or pustule at site of skin needle prick

A

48h, 2mm

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9
Q

Risk factors for the development of superficial thrombophlebitis and vision loss

A

Recurrent erythema nodosum

HLA B51 positivity

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10
Q

Risk factors for development of systemic involvement

A
  1. Superficial thrombophlebitis
  2. Ocular lesions
  3. Male gender
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11
Q

Markers of severe prognosis

A

HLA B51 positivity
Male gender
Early development of systemic signs

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12
Q

Most common signs of Behcet

A 
Oral aphthous ulcers
Genital ulcerations
EN-like lesions
Uveitis
Arthropathy
Papulopustules
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13
Q

Mortality rate of males with systemic signs

A

0-6%

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14
Q

Affects patients in their _____, M ____F

A

20s-30s, =

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15
Q

Crucial for antigen binding and NK cell interactions

A

bW4 epitope

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16
Q

Bacteria related to pathogenesis of disease

A

Streptococcus sanguis KLH 1 antigen

Mycoplasma fermentans MALP 404

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17
Q

Major microscopic finding at most sites of active disease

A

Immune mediated occlusive vasculitis

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18
Q

Primary target organ

A

Endothelium

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19
Q

Endothelial damage is signified by increased plasma levels of ___, which signifies vasoconstriction and ___

A

Endothelin 1, thrombomodulin

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20
Q

Standardize evaluation of mucocutaneous severity

A

Mucocutaneous Activity Index

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21
Q

Presenting sign in 80%

A

Oral mucocutaneous ulcers

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22
Q

Single acneiform lesions or follicular based pustules should NOT be considered relevant. True or False

A

True

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23
Q

Most diagnostically relevant lesion

A

Posterior uveitis/ retinal vasculitis

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24
Q

Describe characteristics of arthritis found in Behcet

A 
NONerosive
Asymmetric
Sterile
SeroNEGATIVE
OLIGOarthritis
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25
Q

Principal feature of pulmonary involvement leading to coughing and hemoptysis

A

Pulmonary artery aneurysms

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26
Q

Ulcers in this area can lead to perforation and massive bleeding

A

Ileocolonic area

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27
Q

Characteristic HPx features

A

Vasculitis & thrombosis

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28
Q

Predominant HPx finding

A

Neutrophilic vascular reaction

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29
Q

Erythema without infiltration is considered a POSITIVE finding.
True or False

A

False

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30
Q

Pathergy reaction is PATHOGNOMONIC for the disease.

True or False

A

False

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31
Q

Mucocutaneous manifestations occur first while joint manifestations occur last.
True or False

A

False, both occur first

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32
Q

Major life threatening complications

A

CNS and pulmonary, large vessel involvement;

GI perforation

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33
Q

Onset in childhood predicts a poor prognosis. True or False

A

False, does not necessarily

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34
Q

Leading causes of morbidity

A

Ophthalmologic and neurologic sequelae ff by severe vascular and GI

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35
Q

More common manifestations of females

A

Genital ulcers
EN like lesions
Joint involvement

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36
Q

More common manifestations in males

A

Ocular, Cardiac, Vascular involvement
Superficial and deep venous thrombosis
Folliculitis
Papulopustular lesions

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37
Q

Medication that can be used during pregnancy

A

Prednisolone

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38
Q

Rapid relapse occurs after discontinuation of the ff medications:

A

Dapsone
Infliximab
IFN alpha
Cyclosporine

39
Q

Treatment for recurrent oral ulcers

A

Irsoglandine 2-4mg/day

40
Q

Treatement for refractory uveitis

A

Tacrolimus 0.05-0.2mg/kg/day PO

Serum levels: 15-25ng/ml

41
Q

Treatment for GI ulcers

A

Sulfasalazine 1.5-3g/day

42
Q

Treatment for intestinal involvement in children

A

Thalidomide
2-3 mkday
ADE: neurotoxicity

43
Q

Treatment for erythema nodosum

A

Methylprednisolone 40mg every 3 weeks every IM

44
Q

Treatment for recent onset ocular disease

A

Azathioprine 2.5mkday

CI: pregnancy, lactation, BMs supression, children, severe infection, hepatotoxicity

45
Q

Treatment for positive pathergy reaction

A

Dapsone 100mg/day
ADE: metHgbinemia
Sol’n: Ascorbic acid 500mg/day

46
Q

Treatment for arthritis and mucocutaneous lesions

A

Indomethacin 100mg/day PO

47
Q

Treatment for progressive psychosis or dementia, arthritis, and severe mucocutaneous lesions

A

Methotrexate 7.5-30 mg once a week

48
Q

Treatment for panuveitis

A
  1. Intravitreal injection (TA 4mg)

2. Cyclosporine A

49
Q

Behcet is a systemic vasculitis involving ___ types and sizes of blood vessels

50
Q

There is __ prevalence due to chronic nature of disease and __ annual incidence

A

Increasing; low

51
Q

Juvenile disease rates are __% in different ethnic groups

52
Q

An __ disease onset has been observed in children of patients with Behcet disease

53
Q

Polymorphisms in __ has been reported in Chinese population

54
Q

New gene associations have been reported (4)

A

ERAP 1
CCR1- CCR3
KLRC4
STAT4

55
Q

Provoking factor for initiation of the disease

A

Exposure to Streptococcus sanguis antigen

56
Q

Involvement of __ is proposed as explanation for chronic infection leading to initiation of Behcet disease

A

IgA protease producing S. Sanguis

57
Q

A possible role for bacterial stimulation of monocytes via __ producing neutrophil stimulating proinflammatory factors in Behcet disease

58
Q

Major role in the pathogenesis of Behcet

A

Immunologic mechanisms

59
Q

Pathergy reaction is induced by:

A

Rapid accumulation of neutrophils (hyperchemotaxis) followed by T lymphocytes, monocytes: macrophages at needle prick sites

60
Q

Behcet is a typical __ mediated inflammatory disease with elevated levels of cytokines IFN gamma, IL2, and TNF alpha

61
Q

__ pathway may play important role in the pathogenesis of Behcet disease

62
Q

Detected as autoantigens in Behcet disease

A

Tropomyosin

160kDa polypeptide kinectin

63
Q

Lymphocytes express __ molecules which bind to endothelial cells in active disease

64
Q

IGM antibody against endothelium __ has been found amongst Behcet patients

A

Alpha enolase

65
Q

Cerebral manifestations of Behcet disease

A

Sterile meningoencephalitis

Vasa neurorum

66
Q

Cardiac involvement of Behcet disease

A

Endocarditis

Pericarditis

67
Q

Pulmonary involvement of Behcet

A

Embolism
Aneurysm
Hemorrhage

68
Q

GI Involvement of Behcet

A

Gastritis
Ulcers
Pseudo-Crohn disease

69
Q

Oral aphthous ulcers heal with scarring (92%)

True or False

A

False, nonscarring

70
Q

Spontaneous healing of apthae occurs within

A

4 days to 1 month

71
Q

Skin lesions considered as diagnostically relevant in dx of Behcet

A 
Pustular vasculitic lesions
EN- like
Sweet-like
PG-like
Palpable purpuric lesions of necrotizing vasculitis
72
Q

Recurrent vasculitic changes can ultimately lead to

A

Ischemic optic nerve atrophy

73
Q

Cases of systemic involvement like the ff are potentially fatal

A

Large vein thromboses

Large artery aneurysms

74
Q

Neurologic involvement usually presents with

A

Severe headache

75
Q

Psychiatric symptoms of the ff are signs of involvement (3)

A

Depression
Insomnia
Memory impairment

76
Q

Scintigraphic evidence of arthritis is found in __% of patients

77
Q

EEG detects diffuse __ waves is a positive finding for Behcet

78
Q

Severe course in 10% presents with

A 
Blindness
Meningoencephalitis
Hemoptysis
Intestinal perforation
Severe arthritis
79
Q

Recurrent aphthae are most commonly treated with

A 
Mild diet
Avoidance of irritating agents
Potent topical steroids
Local anesthetics
HA 0.2% gel BID for 30 days
80
Q

Treatment for genital ulcers and skin lesions

A

Antiseptic creams and corticosteroids for up to 7 days

81
Q

Treatment for recalcitrant ulcers

A

TA 0.1-0.5 ml/lesion

82
Q

Toothpastes containing __ should be avoided

83
Q

Treatment for oral ulcers

A

Rebamipide 300 mg/day PO
Apremilast 30 mg BID PO x 12 weeks
Ustekinumab 90mg SC at weeks 0,4,12

84
Q

ADE of ustekinumab

85
Q

Treatment for visual acuity and ocular attacks, acute hearing loss, thrombophlebitis

A

Cyclosporine A 5mkday PO

ADE: CNS pathologic findings

86
Q

Treatment for intermediate uveitis, panuveitis, posterior uveitis

A

Adalimumab 80mg SC, 40 mg every other week starting 1 wk after initial dose

87
Q

Treatment for active disease

A

5-60mg/ day Prednisolone PO

88
Q

Treatment for acute exacerbation

A

100-1000mg/day IV over 1-3days steroid

ADE: psychosis, DM

89
Q

The ff can be used for ocular lesions except

  1. Anakinra
  2. Canakinumab
  3. Secukinumab
  4. Almetuzumab
A
  1. ineffective
90
Q

The ff can be used to treat for papulopustular lesions

A

IFNalpha 6 x 106 IU thrice a week SC
ADE: psychotic signs, psoriasis, myopathy
Thalidomide 100-300mg/day
Etanercept 25mg SC twice a week orally

91
Q

Caustic solutions for oral ulcers

A

0.5% methyl violet
0.5% hydrogen peroxide
1-2% silver nitrate
5-10% myrrh

92
Q

Antiseptic for oral ulcers

A

0.1% triclosan
0.2% HA gel
1% hexetidine
1-2% chlorhexidine
Diclofenac in 2.5% HA
3% diclofenac
5% amlexanox oral paste
Tetracycline mouthwash 2mins 4-6 times a day
Doxycyline in isobutylcyanoacrylate

93
Q

Anesthetics for oral ulcers

A

0.5-1% tetracaine
1.5% mepivacaine
2-5% lidocaine

7th (23 decks)

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