HLA-B27-related anterior

Uveitis

Nicholas Jones

Manchester Uveitis Clinic

The Royal Eye Hospital

Manchester

Anterior means anterior only

IUSG classification:

Anterior uveitis = Iris

& pars plicata

AU Presentations:

A diagnostic approach

• Acute unilateral non-granulomatous AU

– (60-70% of all new patients)

• Acute bilateral non-granulomatous AU

– (1-2% of all new patients)

• Chronic bilateral, or granulomatous AU

– (10-20%)

• Subacute or chronic AU with unusual features

– (10%)

Acute unilateral non-granulomatous

AU – investigate ?

• History – Known medical diagnosis, treatment

– Ask: arthropathy, bowel, chest, skin, STD, recent illness, travel

• Signs of HLA-B27 positivity (Rothova): – Unilateral acute anterior uveitis

– Age <40 at first attack

– Recurrent attack

– Fibrin or cells +++, NO mutton-fat KP

– Associated AS or Reiter’s syndrome

• Investigations: HLA-B27 only (if necessary)

Typical HLA-B27 related AAU

• Unilateral, photophobia, ciliary congestion

• Posterior synechiae, low IOP, exudate

Severe HLA-B27 related AAU

• Plasmoid AC, fibrin web or clot

• Iris haemorrhage, bloody hypopyon

Severe HLA-B27 related AAU

• IOP very low (<8mmHg)

• Significant cell infiltrate in ANTERIOR vitreous

(including shed ciliary body pigment)

• Macular oedema quite common; occasional papillitis

• Poor or slow response to standard treatment

• Daily subconjunctival steroid +/- oral steroid +/- NSAID

Unusually severe, hyperacute

HLA-B27 related panuveitis

• Very poor visual acuity (<6/60)

• Severe panuveitis with plastic anterior uveitis

• IOP 0-5mmHg

• Aqueous tap for micro-organisms

• Very slow response to treatment

• Frequent relapses – cataract, pre-phthisis

• Oral steroid – oral immunosuppression

• Frequent HLA B27-related ankylosing spondylitis

Mercieca K et al. Ocular Immunology & Inflammation 2010;18:139-41

HLA-B27 related AAU -

Lost/rediscovered treatment skills

• Duke-Elder (1966): • “each attack leaves its mark, producing irreversible changes, and the

end-result is indistinguishable from an acute attack of destructive

severity which terminates in phthisis.”

• “Prompt treatment is therefore the

vital factor in the prognosis – rest,

full atropinisation at the earliest

possible moment, local and, if

necessary systemic steroid therapy..”

HLA-B27 related AAU -

Lost treatment skills

• Introduction of prednisolone acetate:

– Reduced rate of subconjunctival steroid injection

– Therefore reduced usage

of Mydricaine (atropine,

procaine, adrenaline)

• Under-use of atropine

• Under-use of local heat

HLA-B27 related AAU

Old skills regained ?

• Break the synechiae before the patient leaves:

• Vigorous mydriasis:

– Sub-conj Mydricaine or:

– Gt Atr 1% + PE 2.5%

• Then apply local heat:

– Microwaveable pads

– Water-filled glove

• Repeat if necessary!

Then look in the fundus!

Which topical steroid?

• There is very little hard evidence for the relative efficacy of

the various topical steroids • Jones NP. Anterior uveitis. In: Evidence-based ophthalmology, BMJ Books 2004

• There is an abundance of experience-based opinion that

Prednisolone acetate is the most potent

• Loteprednol

– Is it effective enough to treat substantial anterior uveitis?

– Is it reliably less likely to raise IOP (and does it matter)?

Chronic B27-associated uveitis

• Acute intermittent unilateral attacks of AU can:

– become fluctuating and chronic

– involve the posterior segment

– become bilateral

• Inflammation limited to the anterior segment is no less

sight-threatening than posterior uveitis

• Long-term oral immunosuppression is necessary in

some patients with B27-associated anterior or

panuveitis

• Undertreated chronic B27-associated uveitis often leads

to phthisis

Chronic B27 anterior uveitis

• If topical steroid causes IOP to rise:

– Don’t under-treat the inflammation. If you do, this will cause

angle fibrosis and glaucoma anyway.

– The inflammation MUST be controlled adequately:

– and then so must the glaucoma even if it needs tube surgery

– Change to oral immunosuppression if necessary

• Do not tolerate an unsatisfactory “half-way house”

where both uveitis and glaucoma are under-treated

Treating glaucoma in B27-

associated chronic anterior uveitis

• Prostaglandins may theoretically induce inflammation

– but in vitro, only latanoprost does this

– use travoprost or tafluprost by preference

• Many patients have chronic flare, with a high failure

rate for enhanced trabeculectomy

– Primary drainage tube surgery preferred

Managing cataract in B27-associated

uveitis

• Technically these can be among the most

difficult eyes to operate on

• Often heavy flare with risk of post-op

fibrinous uveitis

• Hit very hard with pre-operative and per-

operative steroid, including:

– Moderate-dose oral steroid one week pre-op

– IV methylprednisolone on day of surgery

– Intraocular triamcinolone

Can recurrent HLA B27-associated

AAU be suppressed?

• Sulphasalazine

– 10 pts with >=3 recurrences/yr – 1yr treatment

– Annual recurrence rate 3.4 – 0.9 • Munoz-Fernandez S et al. J Rheumatol 2003;30:1277-9

• Low-dose methotrexate

– 9 pts with >+3 recurrences/yr – 1yr treatment

– Annual recurrence rate 3.4 – 0.9 • Munoz-Fernandez S et al. Eye 2009;23:1130-3

A plea to the busy ophthalmologist:

• Examination findings at first presentation:

– Are at their most distinctive

– May never reappear in this form

– Should be meticulously recorded

– Consider photography

1814-2014