She Developed a Rare Eye Disease After a Strep Infection

A 53-year-old woman with severe eye symptoms had already seen multiple specialists before finally being diagnosed with a rare condition that’s more often seen in children and young adults.

The woman presented to a retina clinic with redness in her eyes, blurred vision, floaters, and light sensitivity. Doctors ultimately determined it to be post-streptococcal uveitis syndrome (PSUS), a rare, immune-mediated condition stemming from group A Streptococcus infection.

“To the best of our understanding, our patient in her 50s is the second oldest reported patient to present with a PSUS, and only the second patient above the age of 50 in the literature,” Norman Saffra, MD, of Albert Einstein College of Medicine and Episcopal Health Services in New York, and colleagues wrote in their case report and literature review published in BMJ Case Reports.

“It’s a good reminder for all practitioners that there are many complications possible from strep throats and post-strep syndromes, and specifically, ocular inflammatory disease from an autoimmune mechanism,” Saffra told MedPage Today.

“It’s usually kids who have strep throat, but you still have to think about it in adults, and this is a perfect example,” he added. “This adult presented with what should have been a childhood disease, reminding us that the uncommon presentation of common diseases is certainly more common, in our experience, than the common presentation of uncommon diseases.”

PSUS was first described in 1991 as an immune-mediated ocular inflammatory condition following group A β-hemolytic streptococcal infection, the authors wrote. While the precise pathophysiology is not yet clear, it’s thought to involve molecular mimicry, where immune responses directed against streptococcal antigens cross-react with retinal S-antigen, causing inflammation in the eye.

PSUS predominantly affects children and young adults, with onset 2 to 4 weeks after a pharyngeal strep infection. Diagnosis depends on elevated titers of anti-streptolysin O (ASO) and/or anti-deoxyribonuclease B along with the clinical findings, after exclusion of other possible etiologies, the authors noted.

In this case, the patient presented with symptoms of photophobia, conjunctival injection, blurred vision, and floaters in both eyes for about a week. Her medical history included gastro-esophageal reflux disease and hypertension, and ocular history included posterior vitreous detachment in the right eye, and bilateral posterior-subcapsular cataracts, but no previous episodes of uveitis.

Prior to developing these symptoms, she’d had an upper endoscopy for possible Barrett’s esophagus, and 1 day after that procedure, she had discomfort and inflammation in her throat. She was diagnosed with culture-positive streptococcal pharyngitis and treated with oral amoxicillin-clavulanate. But shortly after resolution of those symptoms, she began to have the ocular symptoms, first in the right eye, and then the left.

On presentation, visual acuity was 20/70 in the right eye, and 20/40 in the left; intraocular pressures were within normal limits. Examination of the anterior segment showed bilateral 1+bulbar conjunctival injection, 2+cells and flare and fine keratic particulates, the authors noted. The posterior segment exam showed bilateral findings of cystoid macular edema, 3-4+ vitreous cells, optic nerve head edema and vasculitis.

Once a diagnosis of uveitis was made, Saffra said, “now we had to get to, what triggered the uveitis?” And that’s where the adage of, “‘history, history, history,’ is so important.”

The previous physicians she had seen “did not connect the dots,” between her recent bout of strep throat and her symptoms, he said, “and so that’s what makes this case also worthy of sharing.”

Treatment included topical difluprednate 0.05% four times daily and cyclopentolate 1% twice daily in both eyes. Oral prednisone in a dose of 40 mg daily was given following posterior segment involvement, the authors noted.

The inflammation persisted for 8 months, preventing steroid tapering until the ASO titers began to normalize, they wrote. Ultimately, she had complete resolution of symptoms, and at 22 months follow-up, remained disease-free with visual acuity of 20/20 in both eyes.

The researchers add that PSUS has been reported to recur as well as have delayed presentation from the initial strep infection. “Ophthalmologists should be mindful of possible reappearance following resolution of symptoms,” they wrote.