Demystifying Uveitis

Supplement to September 2017 GONE WITH THE FLARE Demystifying Uveitis THOMAS ALBINI, MD; PETER K. KAISER, MD; BYRON LADD, MD; JESSICA SHANTHA, MD; SUNIL SRIVASTAVA, MD; AND STEVEN YEH, MD Sponsored by GONE WITH THE FLARE GONE WITH THE FLARE DISCUSSION WITH THOMAS ALBINI, MD; PETER K. KAISER, MD; BYRON LADD, MD; JESSICA SHANTHA, MD; SUNIL SRIVASTAVA, MD; AND STEVEN YEH, MD Uveitis can be a confounding clinical entity, but when it is diagnosed early and treated appropriately, there is an opportunity to reduce the disease burden and save vision. The fact remains, however, that uveitis specialists cannot manage all uveitis cases alone, suggesting a need to develop greater collaboration with retina physicians in the interest of elevating the quality of care we can deliver. This thinking is the inspiration behind Gone With The Flare, a program developed in collaboration with Santen Inc., that has a twofold objec- tive: to demystify uveitis and to initiate a much larger discussion between uveitis and retina specialists about how best to serve uveitis patients. The discussion captured in the following pages, which is enhanced by example cases we have encountered while treating uveitis patients, is from a Gone With The Flare program held in May 2017. The inclusion of respected retina specialists on the panel provides unique perspectives and insights into the clinical challenges of uveitis, as well as ensures that the approach is broadly applicable. We hope readers will find the text useful and thought provoking, but most of all, we hope it will inspire even greater collaboration between uveitis specialists and retina specialists in the future. Thomas Albini, MD; Jessica Shantha, MD; Steven Yeh, MD Program Chairs, Gone With The Flare UVEITIS PATIENT INTAKE Peter K. Kaiser, MD: In terms of work up, the history and review Thomas Albini, MD: When a patient with uveitis comes into the of systems is much more thorough than what we perform on our practice, how does your interaction with that patient differ from traditional retinal patients. As Dr. Albini said, follow up is very dif- other retina patients? ferent, variable, and often times very intensive during the early diagnosis and management phase. This can be problematic for Byron Ladd, MD: The big difference is the time required to man- physicians who see patients in several offices. Moreover, there may age these patients appropriately. I would estimate that uveitis is be differences in what kind of testing one can do during the patient about 10% of my patient volume. Because uveitis is not my main encounter at the various offices. Calling patients back, especially if focus, the various steps in management take more clinic time. you are asking them to go to a different office, might cause delays in Uveitis is complex and requires a more thorough history, examina- delivering care. tion, and laboratory work up. My practice is a multispecialty group, and we divide the workload: Dr. Ladd: Dr. Kaiser brings up a good point, and it speaks to an our general ophthalmologists manage anterior uveitis and iritis, and important aspect of efficiently managing uveitis while still being I handle cases where the posterior segment is involved. That model thorough. It is imperative that testing is ordered in a thoughtful really arose from the fact that there is not a uveitis specialist within manner so we do not waste time chasing red herrings. That is mean- 100 miles, which created a need for us to manage uveitis patients. As ingful in terms of managing the patients that stay in the clinic and I understand from speaking with colleagues, most community-based when co-managing with consultants, whether that is a rheumatolo- retina specialists fill a similar need. gist, an infectious disease specialist, internal medicine practitioner, or an oncologist. When we coordinate care with others, it helps to Dr. Albini: I think a related point is that for most retina diseases, have a clear picture of the clinical and laboratory findings and how like diabetic macular edema, age-related macular degeneration they should be managed. (AMD), and retinal vein occlusion, there are established intervals for treatment and follow up. While there is always a need to individual- A STANDARDIZED APPROACH FOR UVEITIS ize care, our prognostic ability is much more decisive with those MANAGEMENT conditions. Whereas, in uveitis, it might be difficult to understand Dr. Albini: Uveitis is fundamentally a pathological inflamma- the tempo of the disease, and thus the need for follow up, especially tion of the uveal tract, and there are several ways to approach these during the first encounter. cases. Whether it is a uveitis specialist or a retina physician seeing the 2 SUPPLEMENT TO RETINA TODAY SEPTEMBER 2017 NP-SANTEN-US-0018 DEMYSTIFYING UVEITIS patient, a standardized and organized approach to patient management could be useful. To that end, we are proposing a uveitis decision tree that can serve as such a tool (Figure 1). Steven Yeh, MD: The guidance we are suggesting is really a consolidation of evidence- based practices that, hopefully, makes the approach to uveitis management much more straightforward. It is predicated on making a treatment decision, but is also helps in nar- rowing the differential diagnosis. The first step is to understand the anatomic location of the inflammation, whether it is anterior, intermediate, posterior, or panuveitis. Clinical parameters described by the Standardization of Uveitis Workshop (SUN) are useful in making this determination,1 and it is important to note that we want to assess the location of inflammation, not the complications. Next, we recommend establishing whether an infectious pathogen is involved, which is ideally done before starting treatment. Following this, assess the disease Figure 1. A proposed decision tree for evaluation of uveitis. See cut-out on page 16. course, which may be done on the basis of several factors, including laterality, morphology, host versus systemic disease, whether the etiology is infectious, and disease course—whether it is acute monophasic, acute recurrent, or chronic. For example, Behçet disease and posterior panuveitis with retinal vasculitis are examples of uveitic conditions associated with an acute recurrent pattern of inflammation that may lead to vision loss over time (Figure 2). On the other hand, sarcoidosis, birdshot retinopathy, and some forms of intermediate uveitis present with chronic inflammation that may be difficult to differentiate during the first encounter. Dr. Albini: I think it is important to real- ize that while achieving a diagnosis is optimal, more than 50% of cases are ultimately undifferentiated etiologies.2 That is not at all a sign of failure. In many cases of undifferentiated uveitis, also referred to as idiopathic, treatment may be initiated while the evaluation continues. Sunil Srivastava, MD: We recently did a sur- vey at the clinic showing up to 70% of cases seen Figure 2. Depiction of cycle of inflammation versus visual acuity over time. in community practices may be undifferentiated. Dr. Srivastava: Regardless of whether you are a uveitis Dr. Albini: From the standpoint of the uveitis specialist, when specialist or a community retina practitioner, I think it is prudent to you are referred a case of undifferentiated uveitis, is there a need to approach any case with a previous diagnosis with a grain of salt. The repeat the testing to see if something has been missed? clinician is attempting to build a clinical picture based on the result of SEPTEMBER 2017 SUPPLEMENT TO RETINA TODAY 3 GONE WITH THE FLARE CASE 1: TREATMENT SELECTION IN UVEITIS OF UNKNOWN ETIOLOGY • A 74-year-old white woman referred for initial consultation for panuveitis OD. • Medical history was significant for dermatomyositis (treated with monthly intravenous immunoglobulin and azathioprine); anterior uveitis 3 years prior (successfully treated with topical steroids) • Patient started on oral prednisone 60 mg/day and valacyclovir 500 mg BID; periocular triamcinolone acetonide (40 mg/mL) at time of diagnosis; after worsening inflammation, periocular triamcinolone acetonide (40 mg/mL) was readministered • Fundus photographs showed vitreous haze and vitritis, and full thick- A B ness white lesions with cellular reaction over the lesions in the right Figure 1. Vitreous haze, vitreous, and white lesionsin right eye. No eye, but no signals of active disease in the left (Figure 1). active dieseas shown in left eye. Thomas Albini, MD: Does anyone have any initial reaction to this case? Peter Kaiser, MD: The history worries me a little bit. This is a patient referred with no labs, valaciclovir was initiated, however, for a presumed viral etiology but there were two steroid injections. I would be con- cerned about starting steroids if infectious pathogens have not yet been ruled out. Sunil Srivastava, MD: This case highlights that you do not want to miss anything important or make things worse. The treating doctor could have stopped after the first triamcinolone injection because the inflammation worsened. Jessica Shantha, MD: Oral steroids are always something you can take away but you cannot take away the injection. Dr. Albini: Based on the administration of valaciclovir and the pre- Figure 2. Diffuse infectious processes corresponding with visual sentation with vitritis and retinitis, it could be that the treating doctor acuituy of CF. was worried about a possible viral process. What labs would you order if you saw this patient in your clinic? to screen for other infectious etiologies. The prednisone was tapered and intravitreal foscarnet was given every 3 days. Byron Ladd, MD: I see some bad retinitis, which is suggestive of After three doses of foscarnet plus one ganciclovir injection, the toxoplasmosis. Perhaps the immunocompromised status is a factor in patient reported that her vision had improved. A fundus photograph the high-grade inflammatory response.

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