Chapter 141- Adamantiades- Behcet Disease Flashcards
1
Q
Allele associated with more severe prognosis and ocular involvement
A
HLA B51
2
Q
Viral agent associated with Behcet
A
HSV1
3
Q
Sensitive marker of disease activity
A
IL8
4
Q
Presenting sign in more than 80% of cases
A
Oral aphthous ulcers
5
Q
Major cause of morbidity
A
Ocular involvement
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
7
Q
BCD scoring of ___ indicates Behcet
A
4
8
Q
Positive pathergy test manifests within ____ hours as erythematous papule (> ___mm) or pustule at site of skin needle prick
A
48h, 2mm
9
Q
Risk factors for the development of superficial thrombophlebitis and vision loss
A
Recurrent erythema nodosum
HLA B51 positivity
10
Q
Risk factors for development of systemic involvement
A
- Superficial thrombophlebitis
- Ocular lesions
- Male gender
11
Q
Markers of severe prognosis
A
HLA B51 positivity
Male gender
Early development of systemic signs
12
Q
Most common signs of Behcet
A
Oral aphthous ulcers Genital ulcerations EN-like lesions Uveitis Arthropathy Papulopustules
13
Q
Mortality rate of males with systemic signs
A
0-6%
14
Q
Affects patients in their _____, M ____F
A
20s-30s, =
15
Q
Crucial for antigen binding and NK cell interactions
A
bW4 epitope
16
Q
Bacteria related to pathogenesis of disease
A
Streptococcus sanguis KLH 1 antigen
Mycoplasma fermentans MALP 404
17
Q
Major microscopic finding at most sites of active disease
A
Immune mediated occlusive vasculitis
18
Q
Primary target organ
A
Endothelium
19
Q
Endothelial damage is signified by increased plasma levels of ___, which signifies vasoconstriction and ___
A
Endothelin 1, thrombomodulin
20
Q
Standardize evaluation of mucocutaneous severity
A
Mucocutaneous Activity Index
21
Q
Presenting sign in 80%
A
Oral mucocutaneous ulcers
22
Q
Single acneiform lesions or follicular based pustules should NOT be considered relevant. True or False
A
True
23
Q
Most diagnostically relevant lesion
A
Posterior uveitis/ retinal vasculitis
24
Q
Describe characteristics of arthritis found in Behcet
A
NONerosive Asymmetric Sterile SeroNEGATIVE OLIGOarthritis
25
Principal feature of pulmonary involvement leading to coughing and hemoptysis
Pulmonary artery aneurysms
26
Ulcers in this area can lead to perforation and massive bleeding
Ileocolonic area
27
Characteristic HPx features
Vasculitis & thrombosis
28
Predominant HPx finding
Neutrophilic vascular reaction
29
Erythema without infiltration is considered a POSITIVE finding.
True or False
False
30
Pathergy reaction is PATHOGNOMONIC for the disease.
| True or False
False
31
Mucocutaneous manifestations occur first while joint manifestations occur last.
True or False
False, both occur first
32
Major life threatening complications
CNS and pulmonary, large vessel involvement;
| GI perforation
33
Onset in childhood predicts a poor prognosis. True or False
False, does not necessarily
34
Leading causes of morbidity
Ophthalmologic and neurologic sequelae ff by severe vascular and GI
35
More common manifestations of females
Genital ulcers
EN like lesions
Joint involvement
36
More common manifestations in males
Ocular, Cardiac, Vascular involvement
Superficial and deep venous thrombosis
Folliculitis
Papulopustular lesions
37
Medication that can be used during pregnancy
Prednisolone
38
Rapid relapse occurs after discontinuation of the ff medications:
Dapsone
Infliximab
IFN alpha
Cyclosporine
39
Treatment for recurrent oral ulcers
Irsoglandine 2-4mg/day
40
Treatement for refractory uveitis
Tacrolimus 0.05-0.2mg/kg/day PO
| Serum levels: 15-25ng/ml
41
Treatment for GI ulcers
Sulfasalazine 1.5-3g/day
42
Treatment for intestinal involvement in children
Thalidomide
2-3 mkday
ADE: neurotoxicity
43
Treatment for erythema nodosum
Methylprednisolone 40mg every 3 weeks every IM
44
Treatment for recent onset ocular disease
Azathioprine 2.5mkday
| CI: pregnancy, lactation, BMs supression, children, severe infection, hepatotoxicity
45
Treatment for positive pathergy reaction
Dapsone 100mg/day
ADE: metHgbinemia
Sol’n: Ascorbic acid 500mg/day
46
Treatment for arthritis and mucocutaneous lesions
Indomethacin 100mg/day PO
47
Treatment for progressive psychosis or dementia, arthritis, and severe mucocutaneous lesions
Methotrexate 7.5-30 mg once a week
48
Treatment for panuveitis
1. Intravitreal injection (TA 4mg)
| 2. Cyclosporine A
49
Behcet is a systemic vasculitis involving ___ types and sizes of blood vessels
ALL
50
There is __ prevalence due to chronic nature of disease and __ annual incidence
Increasing; low
51
Juvenile disease rates are __% in different ethnic groups
2 to 21%
52
An __ disease onset has been observed in children of patients with Behcet disease
Earlier
53
Polymorphisms in __ has been reported in Chinese population
IL23 R
54
New gene associations have been reported (4)
ERAP 1
CCR1- CCR3
KLRC4
STAT4
55
Provoking factor for initiation of the disease
Exposure to Streptococcus sanguis antigen
56
Involvement of __ is proposed as explanation for chronic infection leading to initiation of Behcet disease
IgA protease producing S. Sanguis
57
A possible role for bacterial stimulation of monocytes via __ producing neutrophil stimulating proinflammatory factors in Behcet disease
TLR2
58
Major role in the pathogenesis of Behcet
Immunologic mechanisms
59
Pathergy reaction is induced by:
Rapid accumulation of neutrophils (hyperchemotaxis) followed by T lymphocytes, monocytes: macrophages at needle prick sites
60
Behcet is a typical __ mediated inflammatory disease with elevated levels of cytokines IFN gamma, IL2, and TNF alpha
TH1
61
__ pathway may play important role in the pathogenesis of Behcet disease
IL17/IL23
62
Detected as autoantigens in Behcet disease
Tropomyosin
| 160kDa polypeptide kinectin
63
Lymphocytes express __ molecules which bind to endothelial cells in active disease
CD29
64
IGM antibody against endothelium __ has been found amongst Behcet patients
Alpha enolase
65
Cerebral manifestations of Behcet disease
Sterile meningoencephalitis
| Vasa neurorum
66
Cardiac involvement of Behcet disease
Endocarditis
| Pericarditis
67
Pulmonary involvement of Behcet
Embolism
Aneurysm
Hemorrhage
68
GI Involvement of Behcet
Gastritis
Ulcers
Pseudo-Crohn disease
69
Oral aphthous ulcers heal with scarring (92%)
| True or False
False, nonscarring
70
Spontaneous healing of apthae occurs within
4 days to 1 month
71
Skin lesions considered as diagnostically relevant in dx of Behcet
```
Pustular vasculitic lesions
EN- like
Sweet-like
PG-like
Palpable purpuric lesions of necrotizing vasculitis
```
72
Recurrent vasculitic changes can ultimately lead to
Ischemic optic nerve atrophy
73
Cases of systemic involvement like the ff are potentially fatal
Large vein thromboses
| Large artery aneurysms
74
Neurologic involvement usually presents with
Severe headache
75
Psychiatric symptoms of the ff are signs of involvement (3)
Depression
Insomnia
Memory impairment
76
Scintigraphic evidence of arthritis is found in __% of patients
50
77
EEG detects diffuse __ waves is a positive finding for Behcet
Alpha
78
Severe course in 10% presents with
```
Blindness
Meningoencephalitis
Hemoptysis
Intestinal perforation
Severe arthritis
```
79
Recurrent aphthae are most commonly treated with
```
Mild diet
Avoidance of irritating agents
Potent topical steroids
Local anesthetics
HA 0.2% gel BID for 30 days
```
80
Treatment for genital ulcers and skin lesions
Antiseptic creams and corticosteroids for up to 7 days
81
Treatment for recalcitrant ulcers
TA 0.1-0.5 ml/lesion
82
Toothpastes containing __ should be avoided
SLS
83
Treatment for oral ulcers
Rebamipide 300 mg/day PO
Apremilast 30 mg BID PO x 12 weeks
Ustekinumab 90mg SC at weeks 0,4,12
84
ADE of ustekinumab
Headache
85
Treatment for visual acuity and ocular attacks, acute hearing loss, thrombophlebitis
Cyclosporine A 5mkday PO
| ADE: CNS pathologic findings
86
Treatment for intermediate uveitis, panuveitis, posterior uveitis
Adalimumab 80mg SC, 40 mg every other week starting 1 wk after initial dose
87
Treatment for active disease
5-60mg/ day Prednisolone PO
88
Treatment for acute exacerbation
100-1000mg/day IV over 1-3days steroid
| ADE: psychosis, DM
89
The ff can be used for ocular lesions except
1. Anakinra
2. Canakinumab
3. Secukinumab
4. Almetuzumab
3. ineffective
90
The ff can be used to treat for papulopustular lesions
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
Caustic solutions for oral ulcers
0.5% methyl violet
0.5% hydrogen peroxide
1-2% silver nitrate
5-10% myrrh
92
Antiseptic for oral ulcers
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
Anesthetics for oral ulcers
0.5-1% tetracaine
1.5% mepivacaine
2-5% lidocaine
