The Uvea and Joints

Syed Atiqul HaqProfessor of Rheumatology

BSMMU

Rheumatological Associations

of Uveitis

RDs and UveitisSpondyloarthropathies (SpAs)

Juvenile idiopathic arthritis (JIAs)

Sarcoidosis

Behcet’s disease

SjÖgren syndrome

SLE

Systemic vasculitis

Relapsing polychondritis

Spondyloarthropathies

Spondyloarthropathies

Ankylosing spondylitis (AS)

Reactive arthritis (ReA)

Psoriatic arthritis (PsoA)

Enteropathic arthritis

Undifferentiated SpA

Features of Spondyloarthropathies

Young male

Axial pain

Family history

Pauciarticular

Big joints

Asymmetric

Enthesopathies

Rheumatoid factor negative

Radiological sacroiliitis

HLA-B27

Reactive Arthritis

• Preceding diarrhea/dysentery/ST-urethritis

• Conjunctivitis

• Acute onset

• Full remissions & relapses

Ankylosing Spondylitis

• Features of reactive, psoriatic, enteropathic

- absent

• Bilateral sacroiliitis

Uveitis in ReA & AS

• Acute, recurrent, may recur in the other eye

• Symptoms occur 1-2 days before clinical signs

• M:F= 2:1

• Resolves within 3 months

• Prognosis excellent

Enteropathic Arthritis

• Chronic diarrhea, recurrent abdominal pain

• Colonoscopic/imaging features of

– Ulcerative colitis

– Crohn’s disease

Psoriatic Arthritis

• FH of Psoriasis

• Psoriatic plaques - hidden

• Nail lesions

• Hand joint involvement, particularly DIPs

Uveitis in PsoA & Enteropathic Arthritis

• Chronic

• Insidious onset

• Bilateral

• Posterior to lens

• F>M

Juvenile Idiopathic Arthritis

Definition

• Arthritis in

– One or more joints

– Persisting 6 weeks or more

– Begins before 16th birthday

– Has no other known cause

Classification• Oligo-articular JIA: commonest

– Persistent

– Extended

• Enthesitis-related JIA (ERA)

• Polyarthritis:• RF negative

• RF positive

• Psoriatic arthritis

• Systemic onset (Still’s)

• Undifferentiated arthritis

Uveitis in JIA• Common (20-75%) in oligo-articular & ERA

– Occurs in 6% of polyarticular

• Other risk factors:

– Female gender

– ANA positive

• Eye & joint disease evolve independently

Characteristics of Uveitis in JIA• Anterior uveitis, rarely posterior

• Bilateral in 2/3rd

• Insidious onset, acute in ERA

• Chronic: lasts >2 yrs, often >15 yrs

– Remitting & relapsing 60%

– Persistent 20%

– Single episode 20%

Uveitis in Oligo-articluar JIA…

• Often asymptomatic

– Visual disturbance, photophobia, headache: rare

– Pain and redness in 25%

• Acutely painful in ERA

• Prognosis poor, potentially blinding

• Periodic slit lamp surveillance for 1st 4 years

– 3 mnthly 2 yrs, 6 mnthly 2 yrs, then yrly up to puberty

Complications of Uveitis in JIA(May be Diagnostic)

• Band keratopthy

• Posterior synechie

• Cataracts

• Glaucoma

• Blindness

Sarcoidosis

Cardinal Features• Usually a subacute illness: HO a few weeks

• Symptoms:

– Fever

– Cough

– Arthritis: mono- or oligo-articular, often ankle

• Signs:

– Bilateral parotid enlargement

– Erythema nodosum

– Uveitis

• CXR-PA view: Bilateral hilar lymphadenopathy

Sarcoidosis: Ophthalmic Features• 20-40% develop eye disease

• Ophthalmic features:

– Uveitis: commonest

– Dry eyes

– Optic neuritis

– Blepharitis

– Orbital disease

– Asymptomatic conjunctival or scleral nodules: diagnostic

– Retinal vasculitis: periphlebitis, focal cuffing of veins, new vessesls on disc or periphery

Sarcoidosis:

Characteristics of Uveitis

• Anterior in black, posterior in 25%, white and

elderly female

• Acute or chronic, relapsing

• Granulomatous mutton fat keratic precipitates

Mutton Fat Keratic Precipitates

Behcet’s Disease

Behcet’s: Cardinal Features• > 3 episodes oral aphthii/yr + > 2 of following:

– Recurrent genital ulceration

– Uveitis or retinal vasculitis

– Erythema nodosum, pseudofolliculitis, papulopustular lesions, acneform nodules

– Positive pathergy test

Behcet’s: Ocular Involvement• Eyes involved in 70-80% pts

– 25% become blind

• Eye conditions:

– Uveitis

– Retinal vasculitis

– Glaucoma

– Cataract

Uveitis in Behcet’s• Recurrent, 2/3rd cases bilateral

– Panuveitis commonest in both sexes

• Minority female may have isolated anterior uveitis

• Posterior frequent in male

– Often severe

– Resolves spontaneously

– Resolution is incomplete

• Blindness more often due to concomitant retinal vasculitis

Rheumatological Evaluation

of Uveitis

Steps of Evaluation

History

Examination

Laboratory investigation

Referral

History

Demographics, Joint PainAge <16: JIA

>16-young adults: SpAs

Sex Male: AS, ReA, ERAFemale: PsoA, Oligoarticular JIA

Recurrent, short episodes,Diarrhea, dysentery, ureth discharge prior to onset

ReA

Limb predominance LL: ReA, AS, UL: PsoA

Large vs. small Large: ReA, AS, Small: PsoA

Axial SpAs

Other Systems, Past & Family HistoryCough, breathlessness Sarcoidosis

Abdominal pain, bloody diarrhea Enteropathic

Oral and genital ulcers Behcet’s

Past HO joint pain, IBP, PRE, pso SpAs

FH of psoriasis PsoA

FH of RDs, painful red eye SpAs

Examination

Steps of Examination

General examination

Systemic examination (screening)

Examination of MSK system

Examination of the eye

General Examination

Bilateral Parotid Enlargement

Sarcoidosis

• Aphthous ulcer

Behcet’s disease

• Psoriatic Plaques

Psoriatic arthritis

• Psoriatic Nail Changes

Psoriatic arthritis

• Erythema Nodosum

Sarcoidosis

Behcet’s

Reactive arthritis

Post-yersinial

• Keratoderma Blenorrhagicum

Reactive arthritis

Systemic ExaminationFindings Suggested diagnosis

Bilateral pulm. creps Sarcoidosis

Regurgitant murmurs in precordium AS, ReA

Lump in RIF CD with enteropathic arthritis

Neuropthies (mononeuritis multiplex)

Sarcoidosis, Behcet’s

Examination of MSK System• Gait

• Tenderness in hand joints

• Restriction of movement of spine

• SI joint tenderness

• Tenderness at foot joints

• Enthesitis

INVESTIGATIONS

Routine

• Urinalysis

• Hb%, ESR, CBC

Selective Investigations

• CXR-PA view

• X-ray of SI joints

• ANA

• HLA-B27

• CT scan of chest

Indications for CXR-PA

• Fever

• Cough, breathlessness

• Parotid enlargement

• Erythema nodosum

X-Ray SI Joints• Views:

– X-ray pelvis AP view including both SI joints

– X-ray both SI joints oblique view

• Suitable for younger pts

• Indications:

– Adults with suspected SpAs

– Children with suspected ERA, when clinically not obvious

ANA• Oligo-articular JIA: assessing need for periodic slit lamp

• Features of SLE

– Young woman

– Fever

– Butterfly rash

– Hair loss

– Proteinuria

HLA-B27• In adults, suspected SpA

– Clinically not obvious

– SI joint X-ray normal/equivocal

• In children, confusion between oligoarticular JIA and ERA

CT Scan of Chest

• Eye features of sarcoidosis in the absence of

clinical or CXR evidence of systemic disease:

– Granulomatous mutton fat keratic precipitates

– Nodules on iris and choroid

– Associated retinal vasculitis, periphlebitis, focal

cuffing of veins, new vessels on disc or periphery

Management of

RD-Related Uveitis

Treatment Outline

• Initial treatment – Topical glucocorticoids

• Disease resistant to initial treatment – Oral glucocorticoids– Glucocorticoid Sparing Agents (GSA)

Indications for Oral steroid

• Refractory to local therapies, peri-ocular triamcinolone

– Refractory bilateral disease interfering with ADL

• Behcet’s disease

Protocols of Oral Steroid

• Short (+intermittent), 40 to 60 mg for 2 weeks:

– Acute uveitis associated with AS, ReA and ERA

• Long term: Refractory chronic, 4 phases:

– 40 to 60 mg till suppression of inflammation

– Gradual tapering

– Maintenance with minimum dose: 6-12 months

– Gradual withdrawal

Glucocorticoid Sparing Agents

Azathiprine

Methotrexate

Mycophenolate mofetil

Cyclosporin

Tacrolimus

Cyclophosphamide

Thalidomide

Infliximab

Indications For GSAs

• Severe refractory uveitis failed to respond to 40-60

mg/d prednisolone

• Remission not maintained with pred @ <10 mg/d

• Management of associated RD requires the GSA

Take Home

Uveitis→RD →TreatmentType of uveitis Probable RDs Steroid

protocolGSA

Acute AS, ReA, ERA Short course MTX

Chronic PsoAOligoarticular JIA

Long-term MTX

Long-term AZTEnteropathic

With retinal vasculitis

Sarcoidosis Short/long MTX

Behcet’s Short/longIV MethPred

ThalidomideInfliximab

Conclusions• The uvea and joints move hand-in-hand

• Eye involv & its pattern help in specific Dx

• Control of one does not lead to control of other

• For optimum management ophthalmologist

and rheumatologist should work hand-in-hand

THANK YOU