Uveitis in juvenile idiopathic arthritis

1. UVEITIS IN JUVENILE IDIOPATHIC
ARTHRITIS (JIA)
Ahmed Elshafei, MD FRCS
Professor of Ophthalmology
Minia University
No financial intrest

2. DEFINITION
JIA (Juvenile idiopathic arthritis)
formerly JCA (Juvenile chronic
arthritis) or JRA (Juvenile
rheumatoid arthritis):
Arthritis (chronic, seronegative &
peripheral)
Before age 16 years,
Of at least 3 months duration
Other causes have been excluded

3. JIA is the most common
identifiable etiology of pediatric
anterior uveitis (80%) and up to
47% of all cases of pediatric
uveitis.
Most common in North America,
Scandinavia, United Kingdom, and
Germany

4. ETIOLOGY
Autoimmune disorder with
involvement of CD4+ T cells with mix
of genetic and environmental factors.
The cause of uveal inflammation is not
fully known.:
Immune reaction to ocular antigens
Genetic predisposition

5. Oligoarticular
pauciarticular Polyarticular
Systemic
onset )Still
disease)
40-60% 20-40% 10-20%
Common in girls
(5:1)
Common in girls
(3:1)
Equal in both
sexes
Peak onset: at age
of 2 years
Peak onset: at age
of 3 years
Appear at any age
High risk of uveitis Intermediate risk Low risk of uveitis
75% are ANA +ve 40% are ANA +ve 10% are ANA +ve

6. Oligoarticular
pauciarticular
Polyarticular Systemic onset
)Still disease)
4 or fewer joints 5 or more
joints
Symmetric
polyarthritis
Involves the knees
and, less
frequently, the
ankles and wrists.
Involves the small
joints of the hand,
less frequently,
the knee, ankle, or
wrist
Hands,
wrists, feet, ankles,
elbows, knees, hips,
shoulders, cervical
spine, and jaw
Rarely associated
with systemic signs
Moderate
systemic
symptoms:
anorexia, anemia,
& growth
retardation
Systemic onset: fever,
rash, leukocytosis,
lymphadenopathy,
hepatosplenomegaly.
Pericarditis, pleuritis,
&abdominal pain

7. Oligoarticular
pauciarticular
Polyarticular Systemic onset
)Still disease)

8. RECENT CLASSIFICATION
1-Persistent oligoarticular: (4 or less
affected joints throughout the disease)
2-Extended oligoarticular: (4 or less
affected joints during the first 6
months and 5 or more affected joints
thereafter
3-Rheumatoid factor +ve polyarticular
4-Rheumatoid factor -ve polyarticular

9. Patient with the highest risk of
developing uveitis is:

10. WORSE VISUAL
PROGNOSIS
12% of patients with oligoarticular-onset go
blind, due to chronic low-grade inflammation

11. HISTORY
Arthritis usually precedes uveitis.
The mean age of developing uveitis is
9 years in females and 13 years in
males.
Many patients are asymptomatic and
referred by a rheumatologist.

12. HISTORY
Typically, patients have no pain nor
photophobia and the eye is white.
Complications may develop before the
uveitis is detected.

13. SYSTEMIC MANIFESTATIONS
General: Fever, lymph-
adenopathy, fatigue, weight loss
Skin: Rash, nodules, nails
changes & psoriasis
 Anemia
Growth retardation, delay of 2ry
sexual characters

14. GIT: Hepatomegaly, diarrhea,
frequent bowel movements
Respiratory: Cough,
wheezing, pleural effusion
Cardiac: Chest
pain/discomfort, dyspnea,
pericardial effusion
SYSTEMIC MANIFESTATIONS

15. EXAMINATION
Conjunctiva: Most patients have no
conjunctival injection even during acute
exacerbations
Cornea: Small-medium KPs; rarely
mutton-fat KPs (African Americans), band
keratopathy

16. EXAMINATION
Uveitis:
90% Iridocyclitis rarely panuveitis or
vitritis
>90% Nongranulomatous uveitis
80% Bilateral
93% Chronic, 5% recurrent, 2%
acute monophasic

17. EXAMINATION
Uveitis:
Cells and flare
Chronic flare (very common)
Posterior synechiae
Pupillary membrane
Rarely, Koeppe nodules

18. Arthritis may diminish and
resolve with age; however, uveitis
often remains chronic and may
persist into adulthood.

19. Lens - Posterior subcapsular cataracts
IOP- 2ry glaucoma (open angle,
pupillary block, PAS), hypotony and
phthisis
Complications

20. Posterior segment complications:
 Anterior vitreous cells
 Vitritis
 CME, hypotony maculopathy
 Occasionally, disc neo-
vascularization that may respond
to systemic steroids
Complications

21.  Young children can develop
amblyopia from media
opacities or macular edema.
 Complications of systemic
corticosteroids in children: growth
retardation, weight gain,
pancreatitis, DM, hypertension,
cataracts, and glaucoma
Complications

22. FOLLOW-UP
Pauciarticular: Every 3 months, if
patient is ANA +ve  every 2
months
Polyarticular patients: 6-months
Systemic-onset: Annually

23. LAB STUDIES
1- Antinuclear antibody (ANA)
ANA = antibodies that react with
nuclei in tissue sections
demonstrated by the classic indirect
immunofluorescence test (IFA).
ANA-positivity in young girls with
pauciarticular JRA presents the
highest risk of developing uveitis.

24. LAB STUDIES
ANA-positivity is present in most
children with JRA and uveitis
It is present in 80% of those
without uveitis.
So, ANA negativity may help in
predicting that a child may not
develop uveitis

25. LAB STUDIES
2- Human leukocyte antigens
Human leukocyte antigen DR5 and
DR1
A subgroup of older boys with
oligoarticular arthritis have high risk
uveitis are HLA-B27 +ve and -ve for
RF and ANA.
A significant percentage of patients
with spinal involvement are HLA-
B27 positive.

26. LAB STUDIES
2-Rheumatoid factor
Many patients with JRA who
develop uveitis are RF negative.
A few adolescent girls who are RF
+ve have juvenile variant
rheumatoid arthritis, and they are
not at significant risk for ocular
disease.

27. LAB STUDIES
3-Other studies:
Syphilis serologies
Lyme titers,
Angiotensin-converting enzyme
(ACE)
serum lysozyme

28. IMAGING STUDIES
Nondestructive but chronic
articular changes

29. HISTOPATHOLOGY
Joints: hyperplastic synovium, with
subsynovial lymphocytic infiltration,
vascular endothelial hyperplasia, and
edema.
Eye: Lymphocytes, plasma cells, and
scattered giant cells infiltrate the iris
and ciliary body.

30. TREATMENT
Medical treatment
80% of patients respond to topical
corticosteroids
Regional corticosteroids: lack of
response to topical steroid and
posterior segment involvement.

31. TREATMENT
Medical treatment
Systemic corticosteroids: Monocular
involvement favors regional
corticosteroid, while bilaterality favors
systemic corticosteroids.
Oral NSAIDs are useful adjunct
during steroids tapering
Topical NSAIDs in CME

32. TREATMENT
Medical treatment
Cycloplegic-mydriatics to prevent
posterior synechiae
Systemic immunomodulatory
agents (methotrexate, azathioprine and
cyclosporine) in limited or no response
to systemic corticosteroids or when
unacceptable side effects occur
Infliximab or Adalimumab may also be
considered.

33. TREATMENT
Surgical treatment
Cataract surgery:
Delayed IOL implantation (1 year after
cataract extraction) reduced secondary
glaucoma and retrolental membranes.
Should be combined with posterior
capsulotomy and anterior vitrectomy
Should be done when the AC is free from
cells (not flare(
Hypotony is a known post-operative
complication

34. TREATMENT
Surgical treatment
Band keratopathy: EDTA (Ethylene
diamine tetra acetic acid) chelation
Glaucoma surgery: when medical
therapy has failed with progressive
optic nerve damage.
Vitrectomy: adjunct to cataract
surgery or if vitreous debris prevents
optimal vision & prevent phthisis due
to prolonged hypotony.

35. Consultations:
Team approach with consultation of:
Ocular immunology and uveitis
specialist
Pediatric rheumatologist
Child psychiatrist

36. THANK YOU
Thank You