Matthew Tennant

BASICS

DESCRIPTION

• Chronic iridocyclitis typically has an insidious onset and lasts longer than 6 weeks

• Diagnosis may be delayed as it is often asymptomatic

• Bilateral involvement is common

EPIDEMIOLOGY

Incidence

Idiopathic chronic iridocyclitis: 1 case per 300,000 population (1)

Prevalence

Idiopathic chronic iridocyclitis uveitis: 1 case per 14,000 population (1)

RISK FACTORS

Chronic systemic diseases associated with iridocyclitis.

Genetics

Increased prevalence with HLA-A2, DR8, DR11, DR12, DQA1^∗0401, ^∗0501, ^∗0601, and DPB1^∗0201 in association with early onset pauciarticular juvenile arthritis (2)

GENERAL PREVENTION

Patients at risk of developing chronic iridocyclitis should be routinely screened.

PATHOPHYSIOLOGY

Hallmark is infiltration of leukocytes out of microvasculature and into surrounding inflamed tissue.

ETIOLOGY

• Juvenile idiopathic arthritis (JIA)

• Sarcoidosis

• Fuchs heterochromic iridocyclitis

• Tuberculosis

• Syphilis

• Postsurgical uveitis

• Herpes simplex/zoster

• Ankylosing spondylitis

• Inflammatory bowel disease

• Behçet’s disease

DIAGNOSIS

HISTORY

• Many patients are asymptomatic until they develop complications such as cataract and/or band keratopathy

• Questions to ask:

– Onset and duration of symptoms – usual gradual onset with duration >6 weeks

– Past episodes/recurrences and response to treatment

– Review of systems- presence or absence of rash, joint pain, GI symptoms (e.g., bloody diarrhea)

PHYSICAL EXAM

• Red eye with episcleral injection, although, absence of episcleral injection is common

• Aqueous cell and flare

• Keratic precipitates – cellular deposits on corneal endothelium composed of epithelioid cells, lymphocytes, and polymorphs

• Characteristics and distribution provide clues to etiology

– Fuchs heterochromic iridocyclitis – stellate KP diffusely over entire endothelium

– Granulomatous disease – Mutton fat KP (large, greasy) usually more inferiorly

– Iris nodules

– Koeppe nodules – at pupillary border

– Busacca – anterior iris stroma

– Rarely iris crystals

– Posterior synechiae

– Peripheral anterior synechiae

– Granulomatous deposits (Berlin nodules) on trabecular meshwork

– Band keratopathy – from chronic inflammation

– Cystoid macular edema

– Elevated intraocular pressure from trabeculitis or PAS

– Hypotony can develop in longstanding iridocyclitis

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Initial lab tests

• Screening tests should be tailored to causes of chronic iridocyclitis

• CBC

• JIA – antinuclear antibodies, rheumatoid factor, x-ray of affected joints, HLA typing

• Sarcoidosis – angioconverting enzyme assay (ACE), lysozyme, chest x-ray

• TB – PPD skin test, chest x-ray, QuantiFERON gold

• Syphilis enzyme immunoassay

• HLA typing: Ankylosing spondylitis, inflammatory bowel disease

Follow-Up & Special Considerations

Patients with pauciarticular arthritis should have slit-lamp assessments every 3–4 months for the first 5 years of arthritis onset (3).

Imaging

• Chest x-ray or CT Scan – for TB, sarcoidosis

– x-ray of affected joints in JIA

– Gallium scan if indicated- sarcoidosis

Diagnostic Procedures/Other

Anterior chamber tap for PCR testing may be performed to rule out toxoplasmosis, HSV, VZV, TB if no other systemic disease is identified.

DIFFERENTIAL DIAGNOSIS

See “Etiology.”

TREATMENT

MEDICATION

First Line

• Topical steroid (prednisolone acetate 1%) frequency varies depending on severity

– Cycloplegic (scopolamine 0.25% b.i.d or Cyclogyl 1% b.i.d for significant synechiae and/or inflammation)

– Topical glaucoma therapy if IOP elevated

Second Line

Periocular/subtenons steroid (Triamcinolone acetonide 40 mg) or intravitreal steroid (2–4 mg). Triesence (preservative-free Triamcinolone)

ADDITIONAL TREATMENT

General Measures

• Local therapy for monocular disease – subtenons or intravitreal Kenalog/Triesence

• For bilateral disease consider systemic steroid treatment

• Consider corticosteroid sparing agent if inflammation is not controlled within 6 weeks of therapy initiation

Issues for Referral

• Patients with ocular sarcoidosis should be referred to a Pulmonologist

– Pediatric patients with chronic iridocyclitis need a Rheumatology evaluation

– Co-manage systemic steroid sparing agents with Rheumatology

Additional Therapies

• Steroid sparing agents for long term immunosuppression – delayed onset of action – typically 8–12 weeks (4)

– Immunosuppressive agents requires routine blood work for potential side effects and should be co-managed with a Rheumatologist

– Antimetabolites – Azathioprine (Imuran), Methotrexate, and Mycophenolate mofetil (CellCept)

– T-cell inhibitors – Cyclosporine and Tacrolimus

– Alkylating agents – Cyclophosphamide (Cytoxan) and Chlorambucil (Leukeran)

– Biologics

– IL-2 inhibitors – Daclizumab

– Anti-TNF alpha – Infliximab, Etanercept (Enbrel)

– Recombinant interferon alpha

– Antilymphocytic – Rituximab

SURGERY/OTHER PROCEDURES

• Cataract surgery can reactivate or worsen iridocyclitis. Preoperative treatment with systemic steroids 4 days prior to cataract surgery is recommended (5).

– Ideally uveitis should be inactive for at least 3 months prior to cataract surgery.

– When medical management of glaucoma fails, consider surgery with tube-shunt.

– Conventional glaucoma filtration surgery tends to fail in the setting of uveitis.

– Pars plana vitrectomy with membrane peel when epiretinal membrane present.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

• JIA is often asymptomatic and requires ongoing uveitis screening

– If the child is <7 years old and ANA positive, screening eye examination every 3–4 months for 4 years, then every 6 months for 3 years, then annually

– If the child is <7 years old and ANA negative, screening eye exam every 6 months for 4 years, then annually

– If the child is >7 years old regardless of ANA status, screening eye exam every 6 months for 4 years, then annually

PATIENT EDUCATION

Return immediately if iritis recurs, as early treatment reduces complications.

PROGNOSIS

• Remissions and exacerbations of inflammatory activity are common

– Reduction in vision can occur secondary to delayed presentation and complications of prolonged iridocyclitis

COMPLICATIONS

• Cataract

• Band keratopathy

• Glaucoma

• Cystoid macular edema

– Posterior synechiae

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