Eyeworld

EW CORNEA 70 September 2014 PUK in rheumatoid arthritis Source (all): Vincent P. de Luise, MD, FACS Patient with cataract post PUK RF (rheumatoid factor), ANA (anti-nuclear antibody) and ANCA (anti-neutrophil cytoplasmic antibody) titers, C-reactive protein, Lyme titer (if the history suggests), FTA-ABS (fl uorescent treponemal 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 herpes simplex keratitis. Oral nonsteroidals (NSAIDs) are not very helpful in managing PUK, and topical NSAIDs can rarely lead to corneal melt. Oral cyclophospha- mide, methotrexate (oral or subcu- taneous), azathioprine, and tumor necrosis factor-alpha antagonists such as infl iximab, rituximab, and etanercept have all been used to ef- fectively manage PUK in the setting of systemic autoimmune disease. Cataract surgery in PUK Cataract surgery is essentially always elective. Therefore, the PUK must be properly managed and completely controlled, and the eye "white and quiet" for at least 3 months (ideally 6 months) before recommending that patient for anterior segment surgery. The cornea should be photo- graphed preoperatively and the clock hours of corneal thinning identifi ed and labeled. This will dictate where not to make the lim- bal relaxing incisions or the main corneoscleral incision in cataract surgery. Corneal thinning from PUK may also lead to astigmatism, which al (limbal) HSV keratitis can begin as a marginal ulcer mimicking PUK, and adding topical steroids will only exacerbate the problem. A comprehensive patient history is imperative. Rheumatoid arthritis (RA), Wegener's granulomatosis, polyarteritis nodosa, and Crohn's disease can be associated with PUK. Mooren's ulcer can manifest as PUK, but Mooren's ulcer is a diagnosis of exclusion. PUK associated with RA is more common in women, but PUK seen in Mooren's ulcer is more common in men. PUK treatment can be remem- bered with a second mnemonic, AAA or Triple A: 1. Arrest the corneal disease by med- ical or surgical means, if necessary. 2. Add systemic immunosuppres- sion drugs if an underlying systemic disorder is the cause. 3. Alert the rheumatologist the im- munosuppression drugs need to be reevaluated. Co-management with a rheumatologist is essential. In 1984, Foster and colleagues reported in Ophthalmology an increased incidence of mortality in cases of PUK or scleritis in the setting of RA. If we are wrong in our diagnosis of PUK, the patient will likely not die of the condition. But if we are right and the patient has an underlying systemic vasculitis that we diagnosed by identifying the PUK as a marker for systemic disease, that patient's life could be saved and prolonged. Anytime a PUK case is behaving suspiciously, get serologies— by Vincent P. de Luise, MD, FACS Identifying and managing PUK P eripheral ulcerative kerati- tis (PUK) is a term that can refer either to a number of different local ocular conditions or to an ocular manifestation of a systemic autoim- mune disorder. PUK in the setting of systemic autoimmune disease is often the sentinel sign of vasculi- tis—that the systemic disease has entered a dangerous and worsening phase that can result in devastating outcomes, both ocular and systemic. Identifying and managing PUK What can cause PUK? Many ocular and systemic diseases can result in a consequent de-epithelialization, destruction of corneal stroma, and PUK. One useful mnemonic to remember these different conditions is the device "VAST CRIMES." VAST CRIMES stands for Viral (herpes simplex), Autoimmune, Staphylo- coccal marginal, Terrien's/furrow degeneration, Contact lenses, Ro- sacea, Infectious (bacterial, spiro- chetal tuberculosis), Mooren's ulcer, Excise (excising cancerous growths around the limbus of the eye that rarely might present as a peripheral keratitis), and Sclerokeratitis. This is a useful mnemonic to help run down the list of potential causes. Herpes simplex virus (HSV) keratitis can indeed present as PUK. Most clinicians are used to seeing HSV as a central dendrite. However, margin- Management of cataracts in the setting of peripheral ulcerative keratitis C ases of peripheral ulcerative keratitis (PUK) are one of the dreaded clinical entities seen in ophthalmology practice because of their often-tenuous outcomes. Most commonly, PUK occurs in a patient with some sort of autoimmune condition, but it is imperative that the ophthalmologist keep in mind that infectious etiologies must also be ruled out. Identifying the etiology of the PUK, treatment of the ocular condition, and co-management with a rheumatologist to treat the underlying systemic cause are all part of the process. Once a patient's PUK is stable and he or she has a visually signifi cant cataract, there are unique surgical and perioperative manage- ment considerations in this diffi cult patient population. To go along with our feature topic of cataract surgery in patients with corneal comorbidities, this month's "Cornea editor's corner of the world" focuses on the management of cataracts in the setting of PUK. Vincent de Luise, MD, discusses the etiologies of PUK, management of the acute condition, considerations for the timing of cataract surgery, adjustments to surgical technique, and perioperative pearls for these patients. Clara C. Chan, MD, cornea editor Cornea editor's corner of the world

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