Sjögren Syndrome Can Be Underlying Cause of Chronic Cicatrizing Conjunctivitis

Sjögren syndrome can be the underlying cause of chronic cicatrizing conjunctivitis, especially in cases of negative immunoreactant deposition in conjunctival biopsy, researchers found in a retrospective case series report published in The Ocular Surface. The conjunctivitis was “indistinguishable” from mucous membrane pemphigoid (MMP) in the 8 patients included in the study, according to researchers.

Investigators reviewed the medical records of 8 patients with “sicca syndrome” from 2 medical centers and recorded the patients’ cicatrizing conjunctivitis staging according to Foster’s 4-stage classification system of subconjunctival fibrosis, inferior fornix foreshortening, symblepharon, and ankyloblepharon and surface keratinization. They performed conjunctival biopsy to evaluate the patients for cicatrizing conjunctivitis and diagnosed Sjögren syndrome according to the American European Consensus Group’s 2002 6 criteria. Sjögren-specific antibodies A or B, or positive minor salivary gland biopsy results, were mandatory. Patients with secondary Sjögren syndrome, Sjögren syndrome suspects with incomplete testing, patients with Sjögren syndrome who did not have immunofluorescence studies of conjunctival biopsy specimen, and patients with other known causes of cicatrizing conjunctivitis were excluded.

All resulting 8 consecutive patients (all female, 7 White, mean age of 79 years) had immunoreactant deposition in conjunctival biopsy and either a positive minor salivary gland biopsy or positive serology for Sjögren syndrome.

The follow-up from the patients’ initial visit was a median of 52 months. At the initial visit, 5 patients did not have a known diagnosis of Sjögren syndrome and had been referred for dry eye, and their diagnosis was established in the follow-up. Conjunctival scarring was seen in 7 patients (5 with Foster stage 1; 2 with stage 2). Additionally, 6 patients had other extraglandular involvement, such as autoimmune thyroid disease and interstitial kidney disease. At the last follow-up, 5 patients had no signs of progression while 3 could not be adequately immunosuppressed initially, suffered from conjunctival inflammation, and had significant progression of the clinical findings.

A single patient who presented at stage 1 was undergoing treatment with azathioprine for her autoimmune thyroid disease and conjunctival inflammation, and her conjunctival scarring advanced to stage 2 during the follow-up (22 months after the initial visit), but it halted when rituximab was added to the regimen to treat her lymphoma.

Another patient initially presented with no scarring. Her conjunctival scarring progressed to stage 1 as of last visit (124 months before the follow-up) and was treated with hydroxychloroquine.

A third patient presented with stage 1 and interstitial kidney disease while on hydroxychloroquine and received treatment with mycophenolate mofetil for the kidney disease, which helped to halt the disease process at stage 2.

The 3 patients remained stable, the research shows. All patients received treatment for dry eye with topical remedies, including artificial tears, autologous serum tears, cyclosporine, tacrolimus, and topical steroids. Of the 5 patients who had corneal complications, 4 ultimately experienced vision loss.

“While systemic medications may be withheld in patients with stage 1 disease or in the absence of progression of cicatrization, it is the role of the ophthalmologist to monitor clinical findings through regular follow-ups and to collaborate with a rheumatologist in order to ensure that the patient does not have a biopsy-negative MMP which is a common occurrence,” the study says. “Diagnosis and treatment of a less frequent disease, MMP, is better established with proposed algorithms. We propose that cicatrizing conjunctivitis be regarded as a serious extraglandular manifestation of SS which seems to be associated with systemic manifestations of SS.”

No previously published research has detailed the cicatrizing conjunctivitis associated with the chronic autoimmune disorder, which is underdiagnosed in many dry eye patients because of its variable clinical presentation and nonspecific systemic manifestations, according to the report.

The researchers recommended that treatment involve adequate systemic immunosuppression. Limitations of the study include unknown frequency of the condition at the centers and the inability to rule out MMP based on negative immunofluorescence results.

Disclosure: Some study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.

Reference

Sharon Y, Cui D, Akpek E, Chu D. Cicatrizing conjunctivitis as an uncommon manifestation of primary Sjogren’s syndrome. Ocul Surf. Published online November 29, 2020. doi:10.1016/j.jtos.2020.11.014