Ch. 22 - Uveitis in JIA Flashcards
Persistent uveitis with relapses in <3 months after discontinuing treatment
Chronic anterior uveitis
Repeated episodes of uveitis separated by periods of inactivity without treatment for >3 months in duration
Recurrent uveitis
Sudden onset and limited duration uveitis
Acute uveitis
Duration of persistent uveitis
> 3 months
Duration of limited uveitis
3 months or less
Characteristics of uveitis that commonly accompany JIA: Course, location, symptom, onset
Chronic, anterior, asymptomatic, nongranulomatous inflammation, insidious
Characteristics of uveitis that affects HLA-B27 associated diseases like ERA: Course, location, symptom
Acute, anterior, symptomatic
T/F: The choroid (posterior uveal tract) is commonly affected in rheumatic disease of childhood
F
Uveitis in these chronic arthritides are insidious, chronic, and frequently recurrent
Oligoarthritis, polyarthritis, or psoriatic JIA
T/F: Chronic arthritis appears to be particularly uncommon in Asian populations
T
Chronic uveitis is most frequent in what JIA subtypes
Oligoarthritis > RF - polyarthritis > PsA
Chronic uveitis is more common in which gender
F
Mean age of onset of chronic uveitis
4y/o
MC antibody associated with chronic uveitis
ANA
Age-associated risk of uveitis has been seen only in girls. What age group is particularly at risk?
Younger than 7y/o at disease onset
Components of the immune system that play vital roles in the development of uveitis in JIA
T lymphocytes and their products
Innate immune system
Probably B lymphocytes as well
Present in ocular fluids and tissues of adults with AS, the higher levels of which are associated with more severe uveitis
IL-1b, IL-2, IL-6, IFN-y, TNF-a
Levels of this molecule is specially elevated in the aqueous of patients with AS-associated uveitis
TNF-a
Serum levels of these molecules are higher when uveitis is active in adults with anterior, posterior, and panuveitis
IL-6 and 8
Influences the maturation of Th17 CD4+ T cells, which participate in autoimmune disease
IL-6
HLA alleles most strongly and consistently associated with chronic anterior uveitis in JIA
Genes in the class II region
HLA allele associated with acute anterior uveitis
Class I gene HLA-B27
T/F: Up to half of children with chronic uveitis have symptoms attributable to the uveitis
T
Symptoms of anterior uveitis
Redness, pain, headache, photophobia, change in vision
T/F: Uveitis can be detected in patients before onset of arthritis
T, in <10%
T/F: Insidious onset chronic uveitis is commonly bilateral
T, in 70-80%
T/F: Unilateral uveitis may persist for many years in some children before the other eye is involved
T
T/F: Slit-lamp biomicroscopy should be performed on all children with JIA at the time of diagnosis for early detection of chronic uveitis
T
MC symptoms of chronic uveitis
Change in vision and ocular pain and/or redness
Less common symptoms of chronic uveitis
Photophobia and headache
AAP: Frequency of screening of children at high risk for uveitis
Every 3 months
AAP: Frequency of screening of children at moderate risk for uveitis
Every 4-6 months
AAP: Frequency of screening of children at low risk for uveitis
Every 12 months
AAP: JIA group/s at low risk for uveitis
sJIA, all <7 years at onset 7 years after onset, all >7 years at onset 4 years after onset
AAP: JIA group/s at moderate risk for uveitis
All children >7years at onset except SJIA
Poly/Oligo ANA (-) <7years at onset
All high risk patients 4 years after onset of arthritis
AAP: JIA group/s at high risk for uveitis
Poly/Oligo ANA (+) <7years at onset
Children affected by JIA who should have yearly ophthalmologic examination indefinitely
All JIA patients with oligo, poly, and systemic disease
ILAR: Chronic juvenile arthritides at low risk for uveitis
ERA, RF (+) polyarthritis, systemic arthritis
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
T
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
T
Diagnostic signs of anterior uveitis on slit-lamp exam
Inflammatory cells
Increased protein concentration (“flare”) in the aqueous humor of the anterior chamber
Refers to the deposition of inflammatory cells on the inner surface of the cornea
Keratic precipitates
MC complications of chronic anterior uveitis
Synechiae > band keratopathy > cataract > glaucoma
Refers to inflammatory adhesions between the iris and anterior surface of the lens
Posterior synechiae
Complication of chronic uveitis that results in an irregular or poorly reactive pupil
Posterior synechiae
May be the 1st obvious clue to uveitis on ophtha exam, but is often a sign of disease of considerable duration or severity
Posterior synechiae
Deposition of calcium in the corneal epithelium and tends to be a late occurrence in the course of chronic uveitis
Band keratopathy
Band keratopathy initially presents as
Gray-to-brown depositions seen at the corneal limbus
Form of uveitis where the patient usually complains of visual floaters
Pars planitis
Uveitis where the primary site of inflammation is the vitreous
Intermediate uveitis
In posterior uveitis, the primary site of inflammation is the
Retina or choroid
Ocular inflammation that is predominantly anterior to the lens
Anterior uveitis
Adhesions between the iris and cornea
Anterior synechiae
Adhesions between the iris and anterior lens capsule
Posterior synechiae
Insidious onset vs acute onset uveitis: More common in JIA
Insidious onset
Insidious onset vs acute onset uveitis: Strongly associated with HLA-B27
Acute onset
Insidious onset vs acute onset uveitis: Strongly associated with ERA
Acute onset
Insidious onset vs acute onset uveitis: More common in males
Acute onset
Insidious onset vs acute onset uveitis: More often unilateral
Acute
Insidious onset vs acute onset uveitis: Often mistakenly attributed to a foreign body, infection, or an allergy
Acute onset
Insidious onset vs acute onset uveitis: Long-term sequelae uncommon and visual prognosis excellent
Acute onset
T/F: Anterior uveitis without evidence of joint or systemic involvement probably accounts for most children with anterior uveitis
T
T/F: Uveitis may complicate reactive arthritis
T
MC rheumatic diseases associated with uveitis
Oligoarthritis, PsA, poly RF-, and ERA
Kawasaki, Behcet
Rheumatic diseases that rarely present with uveitis
SLE, RF, PAN, HSP, granulomatosis with polyangiitis
Most characteristic lab abnormality found in children with arthritis and insidious chronic uveitis
Presence of ANA, usually in low titer (<1:640)
T/F: ANA and RF are uncommon in children with sudden onset uveitis
T
T/F: In children with acute anterior uveitis, HLA-B27 is common
T
T/F: Abnormalities of acute phase response reflect the extent and activity of uveitis
F, arthritis
These investigations should be included in all children with uveitis
ANA, UA
These investigations should be done for all children with uveitis that is acute and symptomatic
HLA-B27
These investigations should be done on all children with anterior uveitis that is bilateral and sudden in onset
Urine b2 microglobulin, BUN, Crea, ESR, CBC
These investigations should be done on patients with granulomatous uveitis
Mantoux test or quantiferon blood test
CXR if suspicious of tuberculosis
These investigations should be undertaken in children with posterior uveitis
Toxoplasmosis serology, CMV serology and PCR on aqueous fluid, HIV serology
Initial therapy for uveitis in JIA
Topical corticosteroids w/ or w/o mydriasis (to prevent synechiae); continue up to 3 months before gradually tapering; if not responding, ADD second line
Second line therapy for uveitis in JIA
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
Tests for monitoring of MTX toxicity
Liver enzymes, albumin, CBC q2 months
3rd line therapy for uveitis in JIA
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
T/F: Activity of uveitis parallels that of arthritis in JIA
F
T/F: Uveitis may occur for the 1st time after arthritis is in remission
T
Prognosis for uveitis is worse in what conditions
Onset of uveitis occurs before diagnosis of arthritis or shortly thereafter
Initial severe inflammatory response
Chronic inflammation
ANA -
M vs F: Uveitis tends to lead to worse sight loss
M
Large concretions of leukocytes attached to the endothelial surface of the cornea
Mutton-fat precipitates
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
Granulomatous uveitis
Collection of fluid in the macula, a common cause of vision loss in patients with uveitis
Cystoid macular edema
Fibrotic tissue that forms posterior to the lens capsule and impedes the visual axis
Cyclitic membrane
Opacification of the lens as occurs from aging, chronic oral or topical corticosteroid use, or chronic inflammation
Cataract
Optic nerve injury usually resulting from a chronic elevation of intraocular pressure
Glaucoma
Circumferential posterior synechiae resulting in impaired flow of aqueous humor and a marked elevation of intraocular pressure
Iris bombé
The irreversible end stage of chronic inflammation or infection of the uvea
Phthisis
Definition of uveitis remission
Inactive disease for >3 months after discontinuing all treatments for eye disease
MC reported complications of chronic uveitis
Synechiae > Band keratopathy > Cataract > Glaucoma
manifests as a bilateral, sudden-onset, anterior uveitis with eye redness and photophobia before or after the presentation of renal disease
Tubulointerstitial nephritis and uveitis (TINU) (second most com- mon type of childhood uveitis in Japan)
Infection that may result in arthritis and a neuroretinitis characterized by exudates in the retina (macular star)
Bartonella henselae infection
Infections that usually cause focal chorioretinitis
Toxoplasma and Toxocara infections
Infection that may cause ranulomatous uveitis affecting both the anterior and posterior uvea
Tuberculosis
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.
Vogt–Koyanagi–Harada syndrome
Conditions that may mimic uveitis and is caused by juvenile xanthogranuloma or infiltration of the uveal tract with retinoblastoma or leukemic cells
Masquerade syndromes
Indications for treatment of chronic uveitis associated with JIA
1) AC cell grade ≥0.5+
2) Fibrin in the anterior chamber or keratic precipitates with corneal edema
3) Loss of VA
Aim of treatment of chronic uveitis associated with JIA
Persistent AC cell grade 0
T/F Cataract, glaucoma, synechiae, and band keratopathy ALONE without signs of active uveitis DO NOT require antiinflammatory treatment
T
Poor prognostic factors that would indicate escalation of therapy in chronic uveitis associated with JIA
Poor initial vision
Cataract
Macular edema
Dense vitreous body opacification
Ocular hypotony
Glaucoma
Preferred initial treatment for chronic uveitis associated with JIA
GC eye drops preferrably prednisolone acetate 1% or dexamethasone phosphate 0.1%
Indications for introduction of systemic steroid-sparing DMARDs for uveitis associated with JIA
Failure to achieve uveitis inactivity (AC cell grade 0) within 3 months
Flare on weaning topical steroids
Systemic DMARDs is indicated at the outset in patients with the ff cxs
Male gender
Uveitis antedating arthritis
Poor initial vision
Glaucoma
Hypotony
Cataract
Band keratopathy
Posterior synechiae
Dense vitreous body opacification
Macular edema
First-line systemic DMARD for uveitis
MTX
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
0.5-1mkweek, max 30mg/week
3 months