Endocrine Abstracts

Autoimmune glandular syndrome type 2 is a complex genetic condition where a triad of endocrinopathies are involved namely Addison’s disease, type 1 diabetes and/or autoimmune thyroid disorder. The disease predisposes one to a variety of other autoimmune associations. Here we report a rare presentation of a patient with APS-2 presenting with a 7-year history of progressive splenic atrophy leading to auto-splenectomy observed through imaging. We postulate that the underlying cause of this presentation is also of autoimmune nature. While APS-1 has been associated with asplenia and hyposplenism in literature, this is the first report of APS-2 associated with the same. Splenic hypofunction can increase susceptibility to encapsulated bacterial infection, with overwhelming post-splenectomy infection (OPSI) being a significant threat. It is crucial that clinicians recognize the importance of providing guidance on vaccinations, antibiotic chemoprophylaxis, and patient education for individuals with asplenia or hyposplenism. If patients with APS can experience progressive splenic atrophy, we suggest long term follow up with splenic function assessment. It is yet unclear whether pre-emptive screening with pitted red cell count has any clinical impact in this group of patients.