September 2015 Online Photo Diagnosis
BY LUCIANO BASTOS
Sjögren’s Syndrome Post-LASIK
This image shows lissamine green staining in a case of Sjögren’s syndrome post-laser assisted in-situ keratomileusis (LASIK). The staining reflects the dryness at the anterior eye as viewed using white light during slit lamp biomicroscopy.
The patient is a 31-year-old attorney from Rio de Janeiro. He wore soft lenses for myopia until he started experiencing contact lens intolerance, dryness, and conjunctival hyperemia. He tried GP lenses, but was not able to adapt because of discomfort.
In 1996, he underwent LASIK surgery. After the procedure, he presented an overcorrection in both eyes. The surgeon performed a secondary, enhancement procedure to correct the remaining refractive error. The patient then achieved the desired 20/20 vision, but he felt that his eyes were constantly irritated, especially in dry environments1. Rio de Janeiro typically has warm to hot weather, and most people work in air-conditioned facilities. This significantly worsened his symptoms of dry eye, and he also felt the sensation of dry mouth. Visual acuity and vision quality deteriorated in the following years to 20/30 (OD) and 20/40 (OS).
The patient was referred to a rheumatologist who conducted a sublingual salivary gland biopsy. The histopathological diagnosis revealed a confidence interval (CI) of grade 2 for Sjögren’s syndrome. He then travelled from Rio de Janeiro to our clinic in Porto Alegre because he had heard of scleral lens fitting and the benefits that they can provide for dry eye conditions.
The patient first visited our clinic in 2010. At that time, he was instilling oral pilocarpine and artificial tear drops t.i.d. during the day and lubricant gel before sleeping. He also was using special lubricant spectacles when in air-conditioned environments. He further was using prepared artificial saliva orally several times a day.
We conducted some tests using fluorescein and lissamine green to assess the overall condition of the anterior eye. We followed the protocol of the SICCA ocular examination sequence,2 performing the Schirmer’s test I (without anesthesia), tear breakup time (TBUT), corneal fluorescein staining, and lissamine green staining, so we could evaluate the anterior ocular surface. The Schirmer’s test confirmed an unstable or abnormal tear pattern, but due to the variability of this test, we did not considered it alone. The TBUT was five to six seconds.
Figure 2. TBUT test after two minutes of the application of fluorescein dye.
Corneal epithelial staining is a dynamic and time-sensitive process; therefore, to ensure reproducibility, grading of the fluorescein pattern is consistently initiated between four to eight minutes follow instillation.2 In evaluating the fluorescein pattern, we observed punctate epithelial erosions (Figure 3) and also the area where the flap was made during the LASIK procedure. Using the Ocular SICCA scale, we scored the staining as grade 2 because there were more than 20 punctate epithelial erosions (grade 2 refers to six to 30 punctate epithelial erosions). Note that there is a patch of confluent staining at the intermediate, inferior paracentral area of the cornea near the edge of the flap.
Figure 3. Punctate epithelial erosions seen with cobalt blue light and yellow filter.
Figure 4. Fluorescein pattern after five minutes shows the flap border from the LASIK surgery.
We used lissamine green dye, which is considered the gold standard for evaluating ocular surface staining. It has substantial advantages compared to using rose bengal.3
The lissamine green test confirmed a grade 1 (defined by the presence of 10 to 32 dots of staining) ocular staining score (OSS). We counted at least 27 dots during the examination process, which bordered on a grade 2 OSS. An abnormal OSS is defined as a score of 3 or above. We concluded that because the patient was treating the condition with all resources available at the time, his condition was under control despite his understandable complaints. We did not used the red filter, which would increase the grading score.4
The patient was instructed to use preservative-free artificial tears and a prepared preservative-free gel during sleep. We also suggested that he try to improve the humidity in his home and work enviroments if possible, as this would help alleviate the symptoms. We referred the patient to an eyecare practitioner in Rio de Janeiro regarding the possibility of punctal occlusion.
We offered him the option of fitting scleral lenses, as this would help improve his corneal health. We explained that to gain all of the benefits of a scleral lens, the ideal design would have a diamater as large as possible so it could cover a larger portion of the ocular surface. However, the patient was not comfortable with trying a full scleral lens. We considered a 16.0mm diameter lens, but we decided not to test it as the patient was from another state and we were concerned that the lack of follow-up care could compromise the fitting. In addition, the patient achieved 20/25 binocular vision with eyeglasses, which was also a strong argument to delay the fitting and instead pursue it only if the dry eye condition worsened in the future.
In more severe cases of Sjögren’s syndrome, some doctors may precribe high-dose corticosteroids, but their chronic use may induce higher intraocular pressure and cataract. Topical cyclosporine (0.05%) can help avoid excessive use of the corticosteroids. Its use in Sjögren’s syndrome should be considered for more severe cases, and the treatment should last for at least six months. It is known that cyclosporine takes several months before it starts to produce results, so it is sometimes difficult for patients to comply because it can be costly, at least in Brazil. If cyclosporine is prescribed, it should be used in conjunction with other lubricant eye drops.5
Satisfactory binocular vision was achieved with prescription spectacle correction, so we instructed the patient to pursue punctal occlusion through a practitioner in his city. We have not heard from him so far. The ocular dryness secondary to his Sjögren’s syndrome was under control.
It is important to correctly quantify the severity of the symptoms in cases of Sjögren’s syndrome to establish a proper treatment. Scleral lens fitting is an optimal resource for treating the condition, especially if it is a severe case. However, less severe cases can be managed with proper treatment and patient education to assure that the patient will maintain the overal anterior eye surface physiological health to achieve a better quality of vision.
Mr. Bastos is the director and clinical instructor of specialty contact lenses at the Instituto de Olhos Dr. Saul Bastos (IOSB), and is the director and specialty lens consultant of Ultralentes, a small laboratory specializing in GP and scleral lens designs in Porto Alegre, RS, Brazil.
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5. Gehlen ML, Skare TL. Reumato-Oftalmologia. Sao Paulo. Tecmedd. 2007:38-41.