What is the treatment for a teenage boy with bilateral uveitis and elevated serum Angiotensin-Converting Enzyme (ACE) levels?

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Last updated: May 13, 2025View editorial policy

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From the Guidelines

For a teenage boy with bilateral uveitis and high serum ACE, which suggests sarcoidosis, I recommend starting with topical corticosteroid eye drops such as prednisolone acetate 1% every 1-2 hours while awake, tapering as inflammation improves, as per the guidelines outlined by the American College of Rheumatology 1.

Treatment Approach

  • Cycloplegic drops like cyclopentolate 1% twice daily should be added to prevent synechiae formation and reduce pain.
  • For systemic therapy, oral prednisone at 1-2 mg/kg/day (maximum 60-80 mg daily) is typically initiated, with a slow taper over 3-6 months based on clinical response, as suggested by the Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans 1.
  • In cases requiring long-term therapy or with steroid-related concerns, methotrexate (10-25 mg weekly) with folic acid supplementation (1 mg daily) is the preferred steroid-sparing agent, as recommended by the American College of Rheumatology 1.
  • For refractory cases, biologic agents like adalimumab (40 mg subcutaneously every 2 weeks) may be necessary, as indicated by the Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans 1.

Monitoring and Follow-up

  • Regular ophthalmology follow-up every 1-4 weeks initially is essential to monitor treatment response and adjust therapy, as emphasized by the American College of Rheumatology guidelines 1.
  • Multidisciplinary management with ophthalmology, rheumatology, and pulmonology is important for comprehensive care, as highlighted by the Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans 1.

Key Considerations

  • Untreated uveitis can lead to complications including cataracts, glaucoma, and permanent vision loss, as noted by the American College of Rheumatology guidelines 1.
  • The treatment approach should target both the ocular inflammation and underlying systemic disease, as suggested by the Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans 1.

From the Research

Treatment Options for Bilateral Uveitis with High Serum ACE

  • The patient's condition suggests ocular sarcoidosis, which is a leading cause of inflammatory eye disease 2.
  • Systemic corticosteroids are indicated when uveitis does not respond to topical corticosteroids or when there is bilateral posterior involvement, especially macular edema 2.
  • In cases where an unacceptable dosage of corticosteroids is required to maintain remission, additional immunosuppression may be used, especially methotrexate 2.
  • Monoclonal antibodies against tumor necrosis factor-α have been used in severe noninfectious uveitis, including sarcoid uveitis 2, 3.
  • Anti-TNF-α therapy has been shown to be effective in sarcoid uveitis patients refractory to conventional immunosuppressive therapy, allowing for a substantial reduction in prednisone dose 3.

Specific Treatment Considerations

  • Hydroxychloroquine may be an interesting option in sarcoidosis-associated uveitis, with a success rate of 55.6% in one study 4.
  • The incidence rate of flare decreased significantly with hydroxychloroquine treatment, from 204.6 to 63.8 per 100 person-years 4.
  • High serum ACE levels have been implicated in ocular sarcoidosis, and may be a useful marker for diagnosis 5.

Important Considerations

  • Corticosteroids are a powerful and important class of medications in the treatment of uveitis, but can have serious side effects and are not suitable for long-term therapy in most cases 6.
  • The choice of treatment should be individualized based on the patient's specific condition and response to therapy.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Research

Ocular Sarcoidosis.

Seminars in respiratory and critical care medicine, 2020

Research

Hydroxychloroquine Therapy in Sarcoidosis-Associated Uveitis.

Ocular immunology and inflammation, 2024

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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