Uveitis can be an intimidating disease to encounter and manage, especially if you’re not well versed in the disease. Use these tips as a primer on uveitis so you know what’s what when you encounter it.
No. 1: A SYSTEMIC WORKUP MAY BE HELPFUL
If the patient’s uveitic condition is recurrent, recalcitrant, or bilateral, it might be prudent to consider a systemic workup. Generally speaking, I do not recommend a systemic workup on an isolated case of anterior uveitis that responds well to traditional treatment such as topical steroids with or without cycloplegia. Conversely, if the iritis exhibits a particularly severe response that is bilateral, has granulomatous features (mutton-fat keratic precipitates), does not respond to standard treatment, recurs after it is successfully treated, or if there is an underlying history of systemic disease, I would recommend a systemic workup.
Anterior uveitis has been associated with a myriad systemic diseases, including ankylosing spondylitis (AS), rheumatoid arthritis (RA), sarcoidosis, tuberculosis, systemic lupus erythematosus (SLE), Behçet disease, inflammatory bowel syndrome (IBS), juvenile idiopathic arthritis (JIA), syphilis, leukemia, Lyme disease, psoriasis, Wegener granulomatosis (WG), bartonellosis (cat scratch disease), and herpetic disease (zoster or simplex). It may also be drug induced.
A panel of tests to consider includes the following:
- Complete blood count (CBC) with differential for general health and conditions such as anemia, leukemia, etc.;
- C-reactive protein (CRP) for inflammatory diseases;
- Erythrocyte sedimentation rate (ESR), often in conjunction with CRP, for inflammatory disease;
- Antinuclear antibodies (ANA) for autoimmune diseases such as SLE;
- Rheumatoid factor (RF) for rheumatoid arthritis;
- Rapid plasma reagin (RPR), venereal disease research laboratory (VDRL), and fluorescent treponemal antibody absorption (FTA-ABS) tests for syphilis;
- Angiotensin-converting enzyme (ACE) for sarcoidosis;
- Chest x-ray, QuantiFERON-TB Gold, and purified protein derivative (PPD) for tuberculosis;
- Human leukocyte antigen B27 (HLA-B27) for AS, reactive arthritis (Reiter syndrome), Crohn disease, and psoriatic arthropathy;
- Bartonella antibody panel;
- Lyme titer;
- Babesia antibodies (IgG and IgM);
- Anaplasma antibodies (IgG and IgM); and
- Perinuclear and cytoplasmic antineutrophil cytoplasmic antibodies (P-ANCA and C-ANCA) for WG.
However, rather than ordering every lab test to rule out each of these entities, it is sometimes advantageous to tailor testing based on the patient’s demographics, symptoms, and most likely underlying systemic disease. For example, if a young white male has recurrent uveitis and reports back pain, HLA-B27 is critical to rule out AS. For a middle-aged black woman, sarcoidosis, SLE, or other types of autoimmune diseases might be more likely to be part of the differential diagnoses. In this instance, a priority list of laboratory tests would include ANA, RF, ACE, ESR, CRP, and CBC with differential.
No. 2: NOT ALL UVEITIS IS ANTERIOR
Up to 90% of all uveitis is anterior,1 but don’t forget that uveitis can affect other parts of the eye as well, presenting as intermediate, posterior, or panuveitis.
Anterior uveitis
Classic symptoms include pain, redness, and photophobia. Signs include circumlimbal flush, fine or mutton-fat keratic precipitates, and cells and flare to varying degrees in the anterior chamber.
Intermediate uveitis (also called pars planitis or iridocyclitis)
Common features include white blood cells in the vitreous near the inferior retina, that tend to be adherent to retinal tissue, called snowballs or snowbanks.
Posterior uveitis
The least common form of uveitis, this particular form involves inflammation of the retina and choroid caused by infectious or noninfectious disease. Patients typically present with a “fog in headlights” complaint of decreased vision and floaters without the classic symptoms of pain and photophobia associated with anterior uveitis.
Panuveitis
When all three areas, anterior, intermediate, and posterior, are affected, be careful to rule out infectious and noninfectious entities along with masquerading conditions such as neoplastic conditions.
No. 3: THE CHIEF TREATMENT FOR ANTERIOR UVEITIS IS TOPICAL STEROIDS
The mainstay of treatment for anterior uveitis is topical steroids with or without cycloplegia. I typically start treatment with a topical steroid every 1 to 2 hours for the first 1 or 2 days in order to reduce the patient’s inflammation as rapidly as possible. I start to taper the treatment once all cells have subsided, and I taper more quickly for traumatic or idiopathic uveitis. If the condition is linked to a systemic disease, I prefer to take the taper slowly, perhaps over a few weeks. I have encountered cases in which the patient has had a rebound inflammatory response, especially if the condition is systemically related and the patient was tapered off medication too quickly.
There have been rare instances in my clinical experience when oral steroids, steroid injections (either sub-Tenon or intravitreal), or steroid implants were needed. I do not always use cycloplegic agents, but I tend to use them based on the patient’s level of discomfort and severity of presentation.
For intermediate, posterior, and panuveitis, the treatment decision paradigms are more difficult than for anterior uveitis. In posterior uveitis, topical treatment is typically ineffective, and oral treatment in the form of immunosuppressive agents such as methotrexate may be needed to stabilize the response. Due to the systemic nature of these medications and the associated side effects, it is generally recommended that there be a collaborative team management approach with a rheumatologist as well as a retina or uveitis specialist comfortable with treating posterior uveitis. Steroid injections, oral steroids, or steroid implants are all viable strategies in these types of advanced uveitis.
YOU’VE GOT THIS!
An eye care prover encountering uveitis should be able to manage the disease with careful planning and an informed patient. Don’t be intimidated by uveitis: you have the knowledge to manage this disease, and you have the support of your uveitis colleagues should the case be particularly difficult.