Neurotrophic keratitis in autoimmune polyglandular syndrome type 1: a case report

Background Autoimmune polyglandular syndrome type 1 (APS-1) is a rare autosomal recessive disease. In patients with APS-1, the most frequently reported ocular manifestations are keratoconjunctivitis with dry eye and retinal degeneration. However, to our knowledge, no research studies have reported the relationship between APS-1 and neurotrophic keratitis (NK). Possible explanations such as limbus cell deficiency being the primary cause of APS-1 keratopathy are not applicable to our unusual case of the patient with APS-1 presenting as ocular surface disease with NK. Our case findings suggest a new explanation for the observed corneal pathology and a potential treatment for these patients. Case presentation A 27-year-old woman was referred to our hospital because of intermittent blurred vision and recalcitrant ocular surface problems in both eyes for many years. She has a history of autoimmune polyglandular syndrome type 1 (APS-1), which includes hypothyroidism, hypoparathyroidism, hypoadrenalism, and hypogonadotropic hypogonadism. In vivo confocal microscopy clearly demonstrated significant degeneration of the sub-basal nerve plexus and stromal nerve bundles in her corneas bilaterally. She was diagnosed with severe NK and ocular surface disease caused by dry eye. Treatment included the application of therapeutic soft contact lenses and punctual occlusion; however, both treatments had a limited effect. Conclusion Patients with APS-1 may have ocular surface disease and severe damage to corneal nerves. Regular follow-up and treatment focusing on the regeneration of corneal nerves is particularly important in these patients.

Indications of Scleral Contact Lenses in Ocular Surface Diseases

The use of scleral lenses is increased over the last decade. Not only it helps in the patient’s visual rehabilitation but also provides wearing comfort to the patient. Previously scleral lenses were mostly prescribed in cases where traditional therapies don’t work well. But nowadays due to advanced designing and customized fitting this lens are widely prescribed in various ocular surface diseases where these lenses not only improve patient comfort but act as an important tool in promoting healing of the corneal epithelium. Therapeutic Scleral lens indications include DED syndrome, exposure keratitis, graft-versus-host disease, Stevens-Johnson syndrome, ocular cicatri¬cial pemphigoid, persistent epithelial defects, neurotrophic keratitis, chemical burns, limbal stem cell deficiency etc.