Abstract
Purpose: :
To report four cases of unusually severe keratitis sicca, secondary to dacryoadenitis, who eventually required systemic immunosuppressive therapy.
Methods: :
Observational case series of four patients with extremely severe dry eye syndrome, secondary to dacryoadenitis, who were profoundly disabled by pain and photophobia ( to the extent of staying in dark rooms) despite aggressive conventional therapy. Clinical data including visual acuities, other treatments administered for dry eye, systemic medical conditions, Schirmer and Rose Bengal staining results, conjunctival injection, and medications were recorded. All four patients were treated with systemic immunomodulatory therapy.
Results: :
All four patients were female, mean age at presentation 40 years ( range 22–58); and all had systemic autoimmune diseases: Systemic Lupus Erythematosus and Sjogren’s Syndrome ( n=2), Sjogren’s Syndrome (n=1) and Rheumatoid Arthritis and Psoriasis (n=1). Schirmer values at onset ranged from 0–2mm. All had failed aggressive lubrication, topical cyclosporine, punctual plugs. In 2 patients serum tears and hyphrecation were tried without success. Immunosupressive agents were used to control inflammation of the lacrimal glands: Methotrexate and Cyclosporine A (patient 1); Cyclosporine A (patient 2); Prednisone (patient 3); Methotrexate and Remicade (patient 4). Treatment with systemic immunomodulatory resulted in resolution of the acute inflammatory assault on the lacrimal glands and control of signs and symptoms in all four cases. Initial and final visual acuities were: 20/30 (OD) 20/40 (OS) to 20/20 OU in patient 1, 20/40 (OD) 20/30 (OS) to 20/25 OU in patient 2, 20/70 OU to 20/30 OU in patient 3 and 20/60 (OD) 20/50 (OS) to 20/30 OU in patient 4. Final Schirmer values ranged from 3–10; the fourth did not have it done.
Conclusions: :
Systemic immunosuppressive agents may be required in the treatment of primary and secondary Sjogren’s syndrome caused by systemic autoimmune conditions, otherwise resistant to conventional local therapy for dry eye disease.. We show that systemic immunomodulatory therapy leads to significantly improved tear production and resolution of the keratoconjunctivitis in such (rare) instances.
Keywords: immunomodulation/immunoregulation • keratitis • lacrimal gland