Ch. 22 - Uveitis in JIA Flashcards

1
Q

Persistent uveitis with relapses in <3 months after discontinuing treatment

A

Chronic anterior uveitis

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2
Q

Repeated episodes of uveitis separated by periods of inactivity without treatment for >3 months in duration

A

Recurrent uveitis

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3
Q

Sudden onset and limited duration uveitis

A

Acute uveitis

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4
Q

Duration of persistent uveitis

A

> 3 months

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5
Q

Duration of limited uveitis

A

3 months or less

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6
Q

Characteristics of uveitis that commonly accompany JIA: Course, location, symptom, onset

A

Chronic, anterior, asymptomatic, nongranulomatous inflammation, insidious

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7
Q

Characteristics of uveitis that affects HLA-B27 associated diseases like ERA: Course, location, symptom

A

Acute, anterior, symptomatic

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8
Q

T/F: The choroid (posterior uveal tract) is commonly affected in rheumatic disease of childhood

A

F

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9
Q

Uveitis in these chronic arthritides are insidious, chronic, and frequently recurrent

A

Oligoarthritis, polyarthritis, or psoriatic JIA

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10
Q

T/F: Chronic arthritis appears to be particularly uncommon in Asian populations

A

T

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11
Q

Chronic uveitis is most frequent in what JIA subtypes

A

Oligoarthritis > RF - polyarthritis > PsA

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12
Q

Chronic uveitis is more common in which gender

A

F

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13
Q

Mean age of onset of chronic uveitis

A

4y/o

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14
Q

MC antibody associated with chronic uveitis

A

ANA

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15
Q

Age-associated risk of uveitis has been seen only in girls. What age group is particularly at risk?

A

Younger than 7y/o at disease onset

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16
Q

Components of the immune system that play vital roles in the development of uveitis in JIA

A

T lymphocytes and their products
Innate immune system
Probably B lymphocytes as well

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17
Q

Present in ocular fluids and tissues of adults with AS, the higher levels of which are associated with more severe uveitis

A

IL-1b, IL-2, IL-6, IFN-y, TNF-a

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18
Q

Levels of this molecule is specially elevated in the aqueous of patients with AS-associated uveitis

A

TNF-a

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19
Q

Serum levels of these molecules are higher when uveitis is active in adults with anterior, posterior, and panuveitis

A

IL-6 and 8

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20
Q

Influences the maturation of Th17 CD4+ T cells, which participate in autoimmune disease

A

IL-6

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21
Q

HLA alleles most strongly and consistently associated with chronic anterior uveitis in JIA

A

Genes in the class II region

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22
Q

HLA allele associated with acute anterior uveitis

A

Class I gene HLA-B27

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23
Q

T/F: Up to half of children with chronic uveitis have symptoms attributable to the uveitis

A

T

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24
Q

Symptoms of anterior uveitis

A

Redness, pain, headache, photophobia, change in vision

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25
Q

T/F: Uveitis can be detected in patients before onset of arthritis

A

T, in <10%

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26
Q

T/F: Insidious onset chronic uveitis is commonly bilateral

A

T, in 70-80%

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27
Q

T/F: Unilateral uveitis may persist for many years in some children before the other eye is involved

A

T

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28
Q

T/F: Slit-lamp biomicroscopy should be performed on all children with JIA at the time of diagnosis for early detection of chronic uveitis

A

T

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29
Q

MC symptoms of chronic uveitis

A

Change in vision and ocular pain and/or redness

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30
Q

Less common symptoms of chronic uveitis

A

Photophobia and headache

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31
Q

AAP: Frequency of screening of children at high risk for uveitis

A

Every 3 months

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32
Q

AAP: Frequency of screening of children at moderate risk for uveitis

A

Every 4-6 months

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33
Q

AAP: Frequency of screening of children at low risk for uveitis

A

Every 12 months

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34
Q

AAP: JIA group/s at low risk for uveitis

A

sJIA, all <7 years at onset 7 years after onset, all >7 years at onset 4 years after onset

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35
Q

AAP: JIA group/s at moderate risk for uveitis

A

All children >7years at onset except SJIA
Poly/Oligo ANA (-) <7years at onset
All high risk patients 4 years after onset of arthritis

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36
Q

AAP: JIA group/s at high risk for uveitis

A

Poly/Oligo ANA (+) <7years at onset

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37
Q

Children affected by JIA who should have yearly ophthalmologic examination indefinitely

A

All JIA patients with oligo, poly, and systemic disease

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38
Q

ILAR: Chronic juvenile arthritides at low risk for uveitis

A

ERA, RF (+) polyarthritis, systemic arthritis

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39
Q

T/F: Children with psoriatic arthritis are also at considerable risk for development of uveitis and should be followed at the same frequencies as children with oligo- or polyarticular JIA

A

T

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40
Q

T/F: Any child who has had uveitis should be considered to be at high risk, even if it has remitted, and continued surveillance is essential

A

T

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41
Q

Diagnostic signs of anterior uveitis on slit-lamp exam

A

Inflammatory cells
Increased protein concentration (“flare”) in the aqueous humor of the anterior chamber

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42
Q

Refers to the deposition of inflammatory cells on the inner surface of the cornea

A

Keratic precipitates

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43
Q

MC complications of chronic anterior uveitis

A

Synechiae > band keratopathy > cataract > glaucoma

44
Q

Refers to inflammatory adhesions between the iris and anterior surface of the lens

A

Posterior synechiae

45
Q

Complication of chronic uveitis that results in an irregular or poorly reactive pupil

A

Posterior synechiae

46
Q

May be the 1st obvious clue to uveitis on ophtha exam, but is often a sign of disease of considerable duration or severity

A

Posterior synechiae

47
Q

Deposition of calcium in the corneal epithelium and tends to be a late occurrence in the course of chronic uveitis

A

Band keratopathy

48
Q

Band keratopathy initially presents as

A

Gray-to-brown depositions seen at the corneal limbus

49
Q

Form of uveitis where the patient usually complains of visual floaters

A

Pars planitis

50
Q

Uveitis where the primary site of inflammation is the vitreous

A

Intermediate uveitis

51
Q

In posterior uveitis, the primary site of inflammation is the

A

Retina or choroid

52
Q

Ocular inflammation that is predominantly anterior to the lens

A

Anterior uveitis

53
Q

Adhesions between the iris and cornea

A

Anterior synechiae

54
Q

Adhesions between the iris and anterior lens capsule

A

Posterior synechiae

55
Q

Insidious onset vs acute onset uveitis: More common in JIA

A

Insidious onset

56
Q

Insidious onset vs acute onset uveitis: Strongly associated with HLA-B27

A

Acute onset

57
Q

Insidious onset vs acute onset uveitis: Strongly associated with ERA

A

Acute onset

58
Q

Insidious onset vs acute onset uveitis: More common in males

A

Acute onset

59
Q

Insidious onset vs acute onset uveitis: More often unilateral

A

Acute

60
Q

Insidious onset vs acute onset uveitis: Often mistakenly attributed to a foreign body, infection, or an allergy

A

Acute onset

61
Q

Insidious onset vs acute onset uveitis: Long-term sequelae uncommon and visual prognosis excellent

A

Acute onset

62
Q

T/F: Anterior uveitis without evidence of joint or systemic involvement probably accounts for most children with anterior uveitis

A

T

63
Q

T/F: Uveitis may complicate reactive arthritis

A

T

64
Q

MC rheumatic diseases associated with uveitis

A

Oligoarthritis, PsA, poly RF-, and ERA
Kawasaki, Behcet

65
Q

Rheumatic diseases that rarely present with uveitis

A

SLE, RF, PAN, HSP, granulomatosis with polyangiitis

66
Q

Most characteristic lab abnormality found in children with arthritis and insidious chronic uveitis

A

Presence of ANA, usually in low titer (<1:640)

67
Q

T/F: ANA and RF are uncommon in children with sudden onset uveitis

A

T

68
Q

T/F: In children with acute anterior uveitis, HLA-B27 is common

A

T

69
Q

T/F: Abnormalities of acute phase response reflect the extent and activity of uveitis

A

F, arthritis

70
Q

These investigations should be included in all children with uveitis

A

ANA, UA

71
Q

These investigations should be done for all children with uveitis that is acute and symptomatic

A

HLA-B27

72
Q

These investigations should be done on all children with anterior uveitis that is bilateral and sudden in onset

A

Urine b2 microglobulin, BUN, Crea, ESR, CBC

73
Q

These investigations should be done on patients with granulomatous uveitis

A

Mantoux test or quantiferon blood test
CXR if suspicious of tuberculosis

74
Q

These investigations should be undertaken in children with posterior uveitis

A

Toxoplasmosis serology, CMV serology and PCR on aqueous fluid, HIV serology

75
Q

Initial therapy for uveitis in JIA

A

Topical corticosteroids w/ or w/o mydriasis (to prevent synechiae); continue up to 3 months before gradually tapering; if not responding, ADD second line

76
Q

Second line therapy for uveitis in JIA

A

MTX (0.35-0.65mg/kg) by mouth or SQ once a week up to 3 months; if effective, continue MTX and taper topical steroids; monitor for toxicity; if unresponsive, ADD 3rd line

77
Q

Tests for monitoring of MTX toxicity

A

Liver enzymes, albumin, CBC q2 months

78
Q

3rd line therapy for uveitis in JIA

A

Anti-TNF-a, preferably IV infliximab (4-10mg/kg q6-8 weeks) or adalimumab (20-40mg SQ q1-2 weeks); if rapid control is not achieved, replace with MMF or cyclosporine

Local corticosteroid injections

79
Q

T/F: Activity of uveitis parallels that of arthritis in JIA

A

F

80
Q

T/F: Uveitis may occur for the 1st time after arthritis is in remission

A

T

81
Q

Prognosis for uveitis is worse in what conditions

A

Onset of uveitis occurs before diagnosis of arthritis or shortly thereafter
Initial severe inflammatory response
Chronic inflammation
ANA -

82
Q

M vs F: Uveitis tends to lead to worse sight loss

A

M

83
Q

Large concretions of leukocytes attached to the endothelial surface of the cornea

A

Mutton-fat precipitates

84
Q

Although these ocular findings are more common in a granulomatous disease such as sarcoidosis or tuberculosis, the histology of these lesions is not a granuloma

A

Granulomatous uveitis

85
Q

Collection of fluid in the macula, a common cause of vision loss in patients with uveitis

A

Cystoid macular edema

86
Q

Fibrotic tissue that forms posterior to the lens capsule and impedes the visual axis

A

Cyclitic membrane

87
Q

Opacification of the lens as occurs from aging, chronic oral or topical corticosteroid use, or chronic inflammation

A

Cataract

88
Q

Optic nerve injury usually resulting from a chronic elevation of intraocular pressure

A

Glaucoma

89
Q

Circumferential posterior synechiae resulting in impaired flow of aqueous humor and a marked elevation of intraocular pressure

A

Iris bombé

90
Q

The irreversible end stage of chronic inflammation or infection of the uvea

A

Phthisis

91
Q

Definition of uveitis remission

A

Inactive disease for >3 months after discontinuing all treatments for eye disease

92
Q

MC reported complications of chronic uveitis

A

Synechiae > Band keratopathy > Cataract > Glaucoma

93
Q

manifests as a bilateral, sudden-onset, anterior uveitis with eye redness and photophobia before or after the presentation of renal disease

A

Tubulointerstitial nephritis and uveitis (TINU) (second most com- mon type of childhood uveitis in Japan)

94
Q

Infection that may result in arthritis and a neuroretinitis characterized by exudates in the retina (macular star)

A

Bartonella henselae infection

95
Q

Infections that usually cause focal chorioretinitis

A

Toxoplasma and Toxocara infections

96
Q

Infection that may cause ranulomatous uveitis affecting both the anterior and posterior uvea

A

Tuberculosis

97
Q

A rare autoimmune disease possibly induced by immunity to the enzyme tyrosinase, leading to vitiligo, aseptic meningitis, encephalitis, uveitis, and hearing loss, as well as changes in the retinal pigment epithelium. Poliosis (patchy loss of pigment of eyelashes, eyebrows, or hair) is a characteristic finding.

A

Vogt–Koyanagi–Harada syndrome

98
Q

Conditions that may mimic uveitis and is caused by juvenile xanthogranuloma or infiltration of the uveal tract with retinoblastoma or leukemic cells

A

Masquerade syndromes

99
Q

Indications for treatment of chronic uveitis associated with JIA

A

1) AC cell grade ≥0.5+
2) Fibrin in the anterior chamber or keratic precipitates with corneal edema
3) Loss of VA

100
Q

Aim of treatment of chronic uveitis associated with JIA

A

Persistent AC cell grade 0

101
Q

T/F Cataract, glaucoma, synechiae, and band keratopathy ALONE without signs of active uveitis DO NOT require antiinflammatory treatment

A

T

102
Q

Poor prognostic factors that would indicate escalation of therapy in chronic uveitis associated with JIA

A

Poor initial vision
Cataract
Macular edema
Dense vitreous body opacification
Ocular hypotony
Glaucoma

103
Q

Preferred initial treatment for chronic uveitis associated with JIA

A

GC eye drops preferrably prednisolone acetate 1% or dexamethasone phosphate 0.1%

104
Q

Indications for introduction of systemic steroid-sparing DMARDs for uveitis associated with JIA

A

Failure to achieve uveitis inactivity (AC cell grade 0) within 3 months
Flare on weaning topical steroids

105
Q

Systemic DMARDs is indicated at the outset in patients with the ff cxs

A

Male gender
Uveitis antedating arthritis
Poor initial vision
Glaucoma
Hypotony
Cataract
Band keratopathy
Posterior synechiae
Dense vitreous body opacification
Macular edema

106
Q

First-line systemic DMARD for uveitis

A

MTX

107
Q

Based on the CARRA consensus treatment plan for both JIA-associated and idiopathic chronic anterior uveitis, both oral and SC MTX are treatment options at what dose and when to reassess for treatment efficacy

A

0.5-1mkweek, max 30mg/week
3 months