Autoimmune uveitis is a major cause of visual impairment worldwide. It can occur as an isolated inflammatory disorder of the eye (idiopathic) or be associated with systemic autoimmune diseases. Common causes include juvenile idiopathic arthritis, ankylosing spondylitis, Behcet's disease, and sarcoidosis. Treatment involves corticosteroids and immunosuppressants like methotrexate or biologics that target TNF-α or IL-6. Left untreated, autoimmune uveitis can lead to complications affecting the retina, optic nerve and other structures in the eye. Careful management is needed to prevent vision loss.

1. Autoimmune uveitis
Marwa Abo Elmaaty Besar
Lecturer Of Internal Medicine
(Rheumatology Immunology Unit)
(Pediatric Rheumatology)

2. Uveitis:-
 Definition:-
 an inflammatory disorder involving the pigmented vascular coat of the eyeball.
 describe many forms of intraocular inflammation affecting the retina and its vessels,
the optic nerve, and the vitreous.
 A major cause of severe visual impairment.
 10-15% visual loss in USA.
 USA of 52.4/100,000 person-years.
 Younger adults; <40 years at presentation.
 Higher prevalence in females
Gritz DC, etal 2004

4. http://dx.doi.org/10.1136/postgradmedj-2017-134891
Classification:-

5. International uveitis study group recommendations for the evaluation of intraocular inflammatory disease, 1987
7%
59.5 %
18.3 %
14.8 %

6. Autoimmune Uveitis (AU)
 An isolated type of autoimmune uveal inflammation that can occur:
 Without other autoimmune manifestations
(idiopathic autoimmune uveitis: I-AU)
 With systemic autoimmune disease
(systemic disease associated autoimmune uveitis: SDA-AU).
 Affect one or both eyes.
 AU can affect only the eye or part of systemic disease, whose identification is helpful for
the choice of treatment.

7. Front. Immunol., 14 May 2021 | https://doi.org/10.3389/fimmu.2021.676046
The Eye’s Immune Privilege

8. Clinical features:-
 Clinical picture: according to which part of the uvea is involved;
 Eye redness and pain to light sensitivity, blurred vision (anterior uveitis).
 Declining visual acuity (retinitis or neuroretinitis).
 Asymptomatic (childhood posterior uveitis).
• (Symptoms usually occur suddenly and worsen rapidly)
• (Sometimes they develop gradually and become chronic or recurrent)
 Complications; at disease onset or later.
o Cataract (24 %),
o Retinal neovascularization (16.3 %),
o Chorioretinal scars (10.6 %),
o Cystoid macular oedema (8.6 %),
o Glaucoma/hypertension (7.7 %),
o Epiretinal membranes (4.8 %), and
o Retinal detachment (3.8 %)

9. The distribution of idiopathic and systemic disease associated autoimmune uveitis
Clin Exp Med (2016) 16:125–136

11. Eye involvement in Spondylarthritis:-
 The most frequent extra-articular manifestation, 25-30% PSA, 33% AS.
 Usually precede systemic involvement.
 Prevalence increase with:
o Increase duration of the disease.
o HLAB27 positive patients.
 Typical eye involvement;
o Sudden-onset, unilateral, anterior uveitis (iridocyclitis).
o Characterized by painful red eye with photophobia and blurred vision.
o Strong tendency for recurrent attacks, involvement of the contralateral eye.
Loh AR, etal 2010

12. Eye involvement in Bechet's disease:-
 30-70%
 Male, young patient (disease onset < 40 years).
 Eye involvement after 2-3 years of disease onset.
 1/4 patient; may be first presentation.
 The typical form is a chronic, relapsing, bilateral uveitis affecting both anterior and posterior
segments of the uvea (panuveitis).
 Retinal vasculitis and vitreitis, posterior uveitis.
 Hypopyon, anterior uveitis, is peculiar to ocular Bechet's disease
 Complication;
(haemorrhage, thrombosis, macular disease), leading to blindness, 25% patients.
Yurdakul S,etal 2008

13. Eye involvement in Juvenile idiopathic arthritis
(paediatric uveitis):-
 10% of children with JIA.
 Risk group:
o Girls 80%
o oligoarticular type
o Positive ANA, DRB1*0801(DRw8) allele.
 present with
o Bilateral iridocyclitis within 4 years from the onset of the disease.
o ‘‘White uveitis’ indolent and chronic course.
 Complication; band keratopathy, posterior synechiae, and ocular hypertension, total blindness.
Foeldvari I, etal 2014

14. Eye involvement in systemic sarcoidosis:-
 20–50 %
 Any part of the eye, including the orbit and the lacrimal gland.
 Presents with bilateral fat keratic precipitates, iris nodules, and anterior and
posterior synechiae.
 Long-term complications are common, cystoid macular oedema is the worst.
 Isolated ocular sarcoidosis
o Develops in older patients.
o Misdiagnosed as primary intraocular lymphoma.
Birnbaum AD, etal 2010

16. Corticosteroids
 Is the first-line therapy.
 Slow-release intraocular devices, systemic and topical usage.
 Topical corticosteroids are efficient in anterior uveitis, but poor activity in the other location.
 Intra-ocular injection steroid; (FDA 2010)
o In serious cases of anterior uveitis,
o Unilateral intermediate uveitis.
o An adjuvant in posterior uveitis
o Monitoring intraocular pressure (IOP)
o Limited diffusion of steroid particles
 The oral administration; initial treatment in non-infectious posterior uveitis and panuveitis
cases.
 1 mg/kg for 1 months then gradual tapering over 3-6 months. 10mg/day.
Seminars in Arthritis and Rheumatism 49 (2019) 438�445

17. T-Lymphocyte Inhibitors
Cyclosporine A
 Highly effective in uveitis secondary to several aetiologies: sarcoidosis, behçet, birdshot, pars planitis,
vogt-koyanagi-harada
 Immunosuppressive effects by binding to cyclophilin of T-lymphocytes.
 Topical or oral.
 2-5 mg/kg/day is best, (10mg/kg/day).
 Hypertension, hirsutism and nephrotoxic effects.

18. Antimetabolites:
Methotrexate (MTX)
 An antimetabolite of folic acid.
 Effective in uveitis associated with JIA, sarcoidosis, pemphigus, rheumatoid arthritis, etc.
 Complications; myelosuppression, hepatotoxicity.
 High rate of complication led 16% of patients to drop treatment.
Mycophenolate mofetil.
 USA, remission rate at six months was 53%, and 73% after one year.
 this drug was well-tolerated, and only 12% dropped treatment because of side effects.
Int. J. Mol. Sci. 2015, 1618778-18795; doi:10.3390/ijms160818778

19. Int. J. Mol. Sci. 2015, 16

20. Biologics:-
 Infliximab (TNF-α inhibitors):- uveitis secondary to JIA and Behçet, sarcoidosis,
Vogt-Koyonagi-Harada, Birdshot, etc
o Significant adverse effects
o Thrombo-embolism, lupus-like syndrome,
o The formation of solid tumours or lymphomata.
 Adalimumab (TNF-α inhibitors):-
o Effectiveness in uveitis secondary to JIA, reduce it in 35% of refractory uveitis.
o Efficient in uveitis secondary to behçet,sarcoidosis, birdshot, vogt-koyonagi-
harada, in uveitis refractory to usual treatment.
 Golimumab (TNF-α inhibitors):- effective in uveitis secondary to
spondyloarthropathy, vitritis and macular oedema secondary to Behçet, cases of
recalcitrant uveitis secondary to JIA.
 Interferon; IFN-α (α-2a, α-2b) and IFN-β (β-1a and β-1b), patients with refractory
Behçet uveitis but not recommended in uveitis secondary to sarcoidosis.

21. Biologics:-
 Tocilizumab (anti-IL-6 receptor antibody); effective for treating refractory uveitis to
Adalimumab, Infliximab or Abatacept, rebound inflammation after suspending therapy.
 Rituximab; a monoclonal antibody against antigen CD20 on the surface of B-cells, an
alternative to refractory uveitis who do not respond to other drugs.
 Daclizumab; a humanized mononuclear antibody against IL2, patients with refractory
posterior uveitis.
 Alemtuzumab; inhibit CD-52 present B, T lymphocytes, effective uveitis associated with
Leukaemia and Behçet patients.
 Others; (Bevacizumab, Ranibizumab or Aflibercept) antiVEGFs are usually injected
intravitreally, choroidal neovascularisation.
Int. J. Mol. Sci. 2015, 1618778-18795; doi:10.3390/ijms160818778

22. • Infliximab and adalimumab can be considered as first-line immunomodulatory agents for the
treatment of ocular manifestations of Behçet's disease.
• Infliximab and adalimumab can be considered as second-line immunomodulatory agents for the
treatment of uveitis associated with juvenile arthritis.
• Infliximab and adalimumab can be considered as potential second-line immunomodulatory agents
for the treatment of severe ocular inflammatory conditions including posterior uveitis,
panuveitis, severe uveitis associated with seronegative spondyloarthropathy, and scleritis in
patients requiring immunomodulation in patients who have failed or who are not candidates for
antimetabolite or calcineurin inhibitor immunomodulation.
• Infliximab and adalimumab can be considered in these patients in preference to etanercept,
which seems to be associated with lower rates of treatment success

24. Management
of Paediatric
Uveitis

27. Reference:-
 Gritz DC, Wong IG. Incidence and prevalence of uveitis in Northern California; the Northern
California epidemiology of uveitis study. Ophthalmology. 2004;111:491–500.
 Yurdakul S, Yazici H. Behcet’s syndrome. Best Pract Res Clin Rheumatol. 2008;22:793–809.
 Foeldvari I. Ocular involvement in juvenile idiopathic arthritis: classification and treatment. Clin
Rev Allergy Immunol. 1 Aug2014.
 Thorne JE, Woreta F, Kedhar SR, Dunn JP, Jabs DA. Juvenile idiopathic arthritis-associated
uveitis: incidence of ocular complications and visual acuity loss. Am J Ophthalmol.
2007;143(5):840–6.
 Loh AR, Acharya NR. Incidence rates and risk factors for ocular complications and vision loss in
HLA-B27-associated uveitis.Am J Ophthalmol. 2010;150:534–42.
 Birnbaum AD, Huang W, Sahin O, Tessler HH, Goldstein DA. Ocular sarcoidosis misdiagnosed as
primary intraocular lymphoma. Retina. 2010;30:310–6.