What is the initial approach to treating a patient with HLA (Human Leukocyte Antigen) B27 associated uveitis and genital lesions?

Management of HLA-B27 Associated Uveitis with Genital Lesions

For patients with HLA-B27 associated uveitis and genital lesions, topical corticosteroids should be used as initial treatment for both manifestations, with systemic immunosuppressive therapy such as colchicine indicated for recurrent genital ulcers and TNF inhibitors (adalimumab or infliximab) for refractory uveitis.

Initial Assessment and Diagnosis

• HLA-B27 associated uveitis with genital lesions suggests a possible diagnosis of Behçet's syndrome or another spondyloarthritis with extra-articular manifestations 1
• Acute anterior uveitis (AAU) associated with HLA-B27 typically presents as unilateral, fulminant inflammation with cellular infiltration and fibrinous exudation 2
• The condition predominantly affects young adults between 20-40 years of age and has a high tendency for relapse 2
• Genital ulcers in this context may represent mucocutaneous manifestations of an underlying systemic inflammatory condition 1

Treatment Approach for Uveitis

Topical Therapy (First Line)

• Topical glucocorticoids should be used as initial treatment to control ocular inflammation 1
• For HLA-B27 associated acute anterior uveitis, topical treatment is often sufficient as it is typically episodic and self-limiting 1
• Monitor patients with controlled uveitis on stable therapy no less frequently than every 3 months 1
• If tapering or discontinuing topical glucocorticoids, ophthalmologic monitoring should occur within one month after each change 1

Systemic Therapy (For Refractory Cases)

• If uveitis is refractory to topical treatment, consider systemic immunosuppressive therapy 1
• For uveitis affecting the posterior segment, systemic therapy such as azathioprine, cyclosporine-A, interferon-alpha, or TNF inhibitors is recommended 1
• Adalimumab has FDA approval for non-infectious intermediate, posterior, and panuveitis in adults 3
• Monoclonal antibody TNF inhibitors (adalimumab and infliximab) are preferred over etanercept for uveitis management 1

Treatment Approach for Genital Lesions

• Topical steroids should be used for the treatment of genital ulcers 1
• Colchicine should be tried first for prevention of recurrent genital ulcers 1
• For refractory cases, consider systemic immunosuppressives such as azathioprine, thalidomide, interferon-alpha, TNF-alpha inhibitors, or apremilast 1

Integrated Management Approach

1. Initial therapy:

   • Topical corticosteroids for uveitis 1
   • Topical steroids for genital ulcers 1
   • Colchicine for prevention of recurrent genital ulcers 1

2. For refractory uveitis:

   • Progress to systemic immunosuppressive therapy 1
   • Consider methotrexate as first-line systemic agent 1
   • For inadequate response, add monoclonal antibody TNF inhibitors (adalimumab or infliximab) 1, 3

3. For persistent genital lesions:

   • Consider systemic immunosuppressives as used for refractory uveitis 1
   • TNF inhibitors can address both uveitis and mucocutaneous manifestations 1, 3

Monitoring and Follow-up

• Patients with controlled uveitis should be monitored every 3 months 1
• When changing topical glucocorticoid therapy, follow up within one month 1
• When changing systemic therapy, ophthalmologic monitoring should occur within two months 1
• Regular assessment for associated systemic diseases is essential as up to 40% of patients with HLA-B27 positive uveitis have undiagnosed systemic disease 2

Important Considerations

• HLA-B27 positive patients have a high risk for other genetically associated diseases, including spondyloarthritis, inflammatory bowel disease, and psoriasis 2
• Approximately 58% of patients with HLA-B27 associated uveitis have an underlying systemic disorder, which is often undiagnosed before the onset of ocular disease 4
• Though typically described as affecting young white men, women and non-whites may also develop HLA-B27 associated uveitis 4
• Complications of untreated uveitis include posterior synechiae, cataracts, glaucoma, and vision loss 1, 5

Multidisciplinary Approach

• Collaboration between ophthalmologists, rheumatologists, and dermatologists is essential for optimal management 1, 2
• Referral to a rheumatologist is recommended for all patients with HLA-B27 positive uveitis 6
• Early detection through regular screening and appropriate treatment can improve outcomes and prevent complications 1