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Polyglandular Autoimmune Syndrome (PAS), also called polyglandular deficiency syndrome, encompasses a set of rare autoimmune conditions in which several hormone?producing glands progressively fail due to immune?mediated damage. The syndrome is broadly classified into types 1, 2, and 3, each with distinct age of onset, genetic background, and organ?specific patterns. PAS?1 (APECED) is an autosomal?recessive disorder linked to mutations in the AIRE gene and typically presents in childhood with chronic mucocutaneous candidiasis, hypoparathyroidism, and Addison’s disease. PAS?2 (Schmidt syndrome) is more common, polygenic, and strongly associated with HLA?class?II genes, often affecting women in young adulthood with adrenal insufficiency, autoimmune thyroid disease, and type 1 diabetes.

Pathophysiologically, PAS involves autoantibodies and T?cell?mediated destruction of endocrine tissues, leading to partial or complete gland atrophy and hormone deficiency. Non?endocrine autoimmune manifestations such as pernicious anemia, vitiligo, alopecia, and celiac disease may also occur. Diagnosis relies on clinical clustering of at least two organ?specific autoimmune endocrinopathies, supported by biochemical assays and autoantibody testing. Treatment focuses on lifelong hormone replacement (glucocorticoids, mineralocorticoids, thyroid hormones, insulin) plus management of associated infections and complications.