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38 PUK continued from page 37 uveitis specialist with expertise in PUK," she said. Common signs to look for in- clude peripheral ulceration or infil- trates, said Sophie X. Deng, M.D., assistant professor of ophthalmol- ogy, cornea and uveitis division, Jules Stein Eye Institute, David Geffen School of Medicine, Univer- sity of California, Los Angeles. Plus, most patients will complain of severe photophobia even if the PUK is mild. "If you don't see these kinds of PUK is clearly identifiable in this patient Source: Sophie X. Deng, M.D. patients very often, it's easy to mis- take PUK for an inflammatory dis- ease like bad blepharitis or marginal keratitis," Dr. Deng said. When a 2-3 day course of steroids offers no im- provement, think beyond typical keratitis and consider PUK, she said. One helpful general rule of thumb for people with PUK—"it normally doesn't affect younger peo- ple, but mostly people around 50-80 years old," Dr. Deng said. "If the pa- tient comes in, notes contact lens wear, inadvertently slept with the lenses in, and is younger—in his/her 30s—I wouldn't think about PUK as the first diagnosis—I'd treat for in- fectious keratitis." Dr. de Luise agreed and noted An example of marginal (limbal) herpes simplex keratitis (mimicking PUK) Source: Vincent P. de Luise, M.D. herpes simplex also can mimic symptoms of PUK. "Most clinicians are used to seeing central dendrites," he said. "However, marginal (limbal) HSV keratitis can begin as a mar- ginal ulcer mimicking PUK, and throwing steroids at it will only ex- acerbate the problem." As with any disease that has nu- Mooren's ulcer caused this patient's PUK Source: Vincent P. de Luise, M.D. merous potential causes, a complete patient history is imperative. Dr. de Luise said that not only RA but also Wegener's granulomatosis, polyarteritis nodosa, and Crohn's disease can be associated with PUK. Systemic lupus erythematosus is more rarely associated with PUK. In some cases, PUK may be the first inkling a clinician has that a patient has developed an ocular manifesta- tion of a systemic vasculitis. Mooren's ulcer can manifest as PUK, but Mooren's is a diagnosis of exclu- sion, he said. PUK associated with RA is more common in women, but PUK seen in Mooren's ulcer is slightly more common in men, Dr. de Luise noted. Treatment plans For Dr. de Luise, PUK treatment can be remembered with a second mnemonic: "the Triple A—arrest the corneal disease by medical or surgi- cal means if appropriate, add sys- temic immunosuppression drugs if an underlying systemic disorder is causing the PUK, and alert the rheumatologist the immunosuppres- sion drugs need to be re-evaluated." Co-management with a rheuma- tologist is recommended, "especially when you're unsure how extensive the workup should be or uncomfort- able with prescribing and managing immunosuppressive drugs," Dr. Thorne said. If a patient is on med- ication for RA but develops PUK anyway, it's probable an increase in medication is necessary to get the systemic disease under better con- trol, she said. "[Stephen] Foster et al. pub- lished a paper in the journal Oph- thalmology almost 30 years ago on mortality in cases of PUK or scleritis in the setting of RA," Dr. de Luise said. "If we're wrong in our diagno- sis of PUK, the patient will likely not die of the condition. But if we're right—and the patient has an under- lying systemic vasculitis that we di- agnosed by identifying the PUK as a marker for systemic disease—that patient's life can be saved and pro- longed. Any time a PUK case is be- having suspiciously, get serologies —specifically RF [rheumatoid fac- tor], ANA [anti-nuclear antibody] and ANCA [anti-neutrophil cytoplas- mic antibody] titers, C-reactive pro- tein, Lyme titer (if the history suggests), FTA-Abs [fluorescent tre- ponemal antibody absorption], and a chest X-ray. Do a corneal scrape and culture for the possibility of a local infectious cause, such as limbal (marginal) HSV." Co-management is crucial to align the systemic immunomodula- tors with the topical drops, Dr. Deng said. "PUK means the systemic vas- culitis is there and the disease is not controlled. You need the rheumatol- ogist to change the systemic medica- tions to better manage the disease." On occasion, she's given systemic steroids "for inflammation control," also noting the higher mortality rate associated with poorly managed RA/PUK. "Clinicians can also admit the patient to the hospital for a pulse dose steroid first and monitoring. The patient can be started on other immune modulating agents," she said. "Oral non-steroidals are not very helpful in managing PUK, and topical non-steroidals can rarely lead to corneal melt," Dr. de Luise said. Cyclophosphamide, methotrexate (oral or subcutaneous), azathioprine, and tumor necrosis factor-alpha an- tagonists (infliximab, rituximab, etanercept) have all been used to ef- fectively manage PUK in co-existent RA, he added. EW FEATURE February 2011 Corneal diagnoses and systemic disease April 2012