A case of late onset polyglandular autoimmune syndrome.

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Conference Proceeding

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Endocrine Practice


Objective: Polyglandular Autoimmune syndrome (PAS) is a deficiency in the function of several endocrine glands due to an autoimmune cause. Type 3 PAS is characterized by a combination of autoimmune thyroiditis, type 1 diabetes and other autoimmune processes. This case is a rare late onset of type 3 PAS. Case Presentation: A 93-year old male with a medical history of CAD, stage 4 CKD, hypertension presented with dizziness leading to a fall. Patient was found to be in severe DKA with blood glucose of 805 and beta-hydroxybutyrate of 6.04. Patient has no known history of endocrinopathy. His blood sugar was normal during a previous hospitalization three weeks prior when he had an upper respiratory infection. Diagnosis of latent autoimmune diabetes of adults (LADA) was supported with elevated GAD antibodies and low C-peptide of 0.8. Further work-up revealed TSH of 15.71, free T4 of 0.6 and a positive Thyroperoxidase antibodies suggesting autoimmune thyroiditis. Patient did have episode of hypotension. However, morning cortisol level was not suggesting of adrenal insufficiency. Patient was diagnosed with type 3 PAS. Discussion: Environmental exposure or intrinsic changes in the gene were hypothesized as the triggers for PAS in the genetically susceptible population. Patient did have a respiratory infection before the diagnosis of LADA and autoimmune thyroiditis, which could trigger PAS if he is genetically susceptible. However, type 3 PAS typically happens in the fourth decade of life. A diagnosis at age 93 was considered a very late onset for LADA and type 3 PAS. It was not clear if the patient has type 2 PAS. Cortisol was collected after possible steroid use for hypotension workup, which can cause falsely elevated cortisol. A 21-hydroxylase antibody was collected for further workup. It is possible that this patient developed adrenal insufficiency, which could classify him as type 2 PAS. Conclusion: When a patient develops one autoimmune endocrinopathy, it is important to screen for others especially in sudden onset endocrinopathy. More comprehensive studies and research about PAS will reveal information and facts that can clarify the epidemiology and etiology which may help preventing foreseeable complications.



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