MON-357 A Rare Case of Vogt-Koyanagi-Harada (VKH) Disease, Graves’ Disease, and Primary Ovarian Insufficiency (POI): Expanding the Spectrum of Multisystem Autoimmunity

Abstract Disclosure: A.N. Mukhtar: None. G.Y. Gandhi: None. Background: VKH disease is a rare T-cell-mediated autoimmune disorder that targets melanocyte-containing tissues, leading to uveitis, hearing loss, vitiligo, and meningeal inflammation. Early diagnosis and initiation of systemic corticosteroids, often combined with immunosuppressive therapy, are crucial for management. While VKH is primarily cell-mediated, Graves’ disease and POI are largely B-cell-driven disorders characterized by autoantibody-mediated dysfunction. Clinical Case: A 28-year-old female with VKH diagnosed at age 14 was diagnosed with Graves’ at age 20. Labs notable for TSH <0.006 µIU/mL (0.4-4.5), Free T4 3.51 ng/dL (0.8-1.8), Free T3 8.0 pg/mL (2.0-4.4), TSI 456 (0-139%), and TPO 3,558 IU/mL (0–34). A radioiodine uptake and scan showed increased uptake (5-hour: 86%, 23-hour: 82%) with a homogeneous distribution. She was treated with methimazole for two years, followed by radioiodine ablation (16 mCi). She developed hypothyroidism two months post-ablation and has since been euthyroid on levothyroxine. In December 2022, she developed idiopathic intracranial hypertension and started acetazolamide. After a few months, she experienced secondary amenorrhea. Gynecology suspected acetazolamide as a potential cause, leading to its discontinuation after over a year of use, but menses did not resume. An endocrine evaluation revealed a low estradiol 22 pg/mL and high FSH181.8 mIU/mL along with 17-hydroxyprogesterone 11 ng/dL (<200), DHEA-S 83 µg/dL (35-430), prolactin 8.9 ng/mL (4-23), testosterone 11 ng/dL (15-70). Given her autoimmune history, an immune-mediated ovarian failure was suspected, which could have been further confirmed by testing autoantibodies (such as anti-ovarian and anti-17α-hydroxylase antibodies); however, these tests are not widely available in clinical practice. Conclusion: This case highlights a rare presentation of VKH, Graves’ disease, and POI, suggesting that VKH may be part of a broader autoimmune polyglandular spectrum. Reports of VKH coexisting with hypothyroidism, diabetes, and adrenal insufficiency support this hypothesis. The HLA associations identified in VKH suggest a genetic predisposition. Altered T-cell subsets and cytokine profiles (elevated IL-2, IL-6) in VKH pathogenesis may contribute to cross-reactivity between melanocyte and endocrine autoantigens, leading to multisystem autoimmunity. Some reports suggest VKH may remit during pregnancy, possibly due to immune modulation in gestation. The role of acetazolamide in modulating neurotransmitter function and hormonal regulation requires investigation. Further research into the immunopathogenesis of VKH and its relationship to endocrine autoimmunity is warranted to enhance early recognition and personalized treatment. Presentation: Monday, July 14, 2025