The use of topical cyclosporine A 0.05% as treatment for primary alacrimia in Allgrove syndrome

Introduction: The purpose is to report a case on the use of cyclosporine A 0.05% for primary alacrimia in Allgrove syndrome or triple A syndrome (alacrimia, achalasia, and adrenal insufficiency).

Case description: A 37-year-old man with achalasia treated surgically 11 years ago presented with sensation of a foreign body, irritation, and intermittent ocular redness for several years. Ophthalmological examination revealed bulbar hyperemia, Oxford grade 4 corneal staining, anisocoria, and optic atrophy. The patient was initially treated with washing with serum and lubricants. Due to the persistence of symptoms, treatment with cyclosporine A 0.05% was started observing a clinical improvement with a decrease in the symptoms caused by tear deficiency.

Conclusion: It is important to emphasize the relevance of establishing an early diagnosis through a complete multidisciplinary clinical examination and a study of adrenal function. The treatment of dry eye in these patients is difficult to manage, with topical immunomodulators such as cyclosporine A as a good alternative when lubricants are insufficient. To our knowledge this is the first case of subjective and objective improvement of dry eye using cyclosporine A 0.05% in a case of alacrimia in triple A syndrome. Allgrove syndrome presentation does not always manifest with the classic triad and some symptoms may be not present at the time of diagnosis. Ophthalmologist and pediatrician should consider this syndrome in patients with symptoms as complex as lack of growth, crying without tears, and convulsions.

Keywords: Anti-inflammatory agents; cornea/external disease; diseases of the ocular surface; examination techniques; examination techniques: stains; ocular surface agents.