Juvenile idiopathic arthritis and its associated uveitis

ABSTRACT

Introduction

Juvenile idiopathic arthritis is the most common chronic rheumatologic disease in children. Uveitis is the most common extra-articular manifestation of JIA, and it can be a sight-threatening condition.

Areas covered

In this review article, we discussed epidemiology, risk factors, clinical presentation, supportive laboratory tests, treatment options, and complications of Juvenile idiopathic arthritis and Juvenile idiopathic arthritis associated uveitis. We covered conventional immunomodulatory therapy and biologic response modifiers agents for different types of Juvenile idiopathic arthritis and their associated uveitis. Finally, we discussed the course of disease, functional outcome, and the quality of life of Juvenile idiopathic arthritis and Juvenile idiopathic arthritis-associated uveitis.

Expert opinion

Although clinical outcomes of Juvenile idiopathic arthritis and its associated uveitis have been improved over the past three decades by biologic response modifier agents, a significant proportion of patients require active treatment into adult life therefore screening and monitoring of these patients is required during the patient’s entire life. The limited number of food and drug administration approved biologic response modifier agents for the treatment of Juvenile idiopathic arthritis associated uveitis justify more randomized clinical trials with new medications in this field.

KEYWORDS:

• Arthritis
• biologic response modifier
• enthesitis
• immunomodulatory therapy
• JIA
• Juvenile idiopathic arthritis
• oligoarticular, polyarticular
• uveitis

Article highlights

• Juvenile idiopathic arthritis (JIA) is further divided into 7 relatively homogenous categories by the ILAR’s criteria: oligoarthritis, rheumatoid factor (RF) – negative polyarthritis, RF-positive polyarthritis, systemic arthritis, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis.

• Uveitis can be potentially a sight-threatening condition in patients with JIA.

• Chronic anterior uveitis is the typical form of JIA-associated uveitis and one of the most significant complications associated with JIA.

• Uveitis is most commonly seen with oligoarthritis (25%), followed by polyarticular (11%) category, although it can occur in any of the seven JIA arthritides.

• Young age of onset (<7 years) and antinuclear antibody (ANA) as the two strongest predictors of JIA-associated uveitis.

• The higher serum anti-histone antibody may be a weak predictor of uveitis incidence in female patients.

• Chronic intraocular inflammation can lead to synechiae, band keratopathy, cystoid macular edema, cataract, and eventually blindness.

• An interdisciplinary approach, including pediatric rheumatologist, ophthalmologists, psychologists, nurses, and the patient’s family forms the ideal model for the care for patients with JIA.

• Methotrexate is the first-line of treatment in JIA-associated uveitis and is recommended, orally or by subcutaneous route at a dose of 15 mg/m²/week.

• Adalimumab is a fully humanized TNF-α inhibitor and has been approved as the first biologic for many types of uveitis, including JIA-associated uveitis.

• Infliximab is a chimeric monoclonal antibody against TNF-α. Infliximab is not approved but is used off-label in polyarticular JIA

• Subcutaneous golimumab, a fully humanized anti-TNF- α, may be considered as an alternative anti-TNF agent for refractory JIA-uveitis.

• Intravenous tocilizumab, a fully humanized anti-IL6 agent has been approved for systemic and polyarticular JIA in patients over two years and older.

• There are limited data with regard to abatacept and rituximab for treatment of JIA-associated uveitis.

• JAK inhibitors are under investigation for systemic JIA, polyarticular JIA and its associated uveitis.

Declaration of interest

C Foster declares the following: Consultancies with Aldeyra Therapeutics (Lexington, MA), Allakos (Redwood City, CA), Bausch & Lomb Surgical, Inc (Rancho Cucamonga, CA), Eyegate Pharma (Waltham, MA), Genentech (South San Francisco, CA), Novartis (Cambridge, MA), pSivida (Watertown, MA). Grants or grants pending with Aciont (Salt Lake City, UT), Alcon (Aliso Viejo, CA), Aldeyra Therapeutics (Lexington, MA), Bausch & Lomb (Rochester, NY), Clearside Biomedical (Alpharetta, GA), Dompé pharmaceutical (Milan, Italy), Eyegate Pharma (Waltham, MA), Mallinckrodt pharmaceuticals (Staines-upon-Thames, UK), Novartis Pharmaceuticals (Cambridge, MA), pSivida (Watertown, MA), Santen (Osaka, Japan). Payment for lectures including service on speaking bureaus: Alcon (Aliso Viejo, CA), Allergan (Dublin, Ireland), Mallinckrodt pharmaceuticals (Staines-upon-Thames, UK). Stock or Stock Options: Eyegate Pharma (Waltham, MA). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

References

Additional information

Funding

This paper was not funded.