Abstract

Introduction: Autoimmune (AI) polyendocrine syndrome type 2 (APS-2) is an association of autoimmune conditions characterized by at least two of the following three conditions: type 1 diabetes mellitus (DM), AI thyroiditis, and primary adrenal insufficiency. APS-2 is polygenic, effects more woman than men, usually presents in adulthood, and results from loss of immune tolerance, where T cells become autoreactive with resulting lymphocytic infiltration and dysfunction of affected tissues and organs. Circulating autoantibodies can confirm the diagnosis. APS-2 is associated with other AI conditions (Celiac disease, primary ovarian insufficiency, systemic lupus erythematosus (SLE), rheumatoid arthritis, vitiligo, alopecia, myasthenia gravis, and AI gastritis). We describe a case of a woman with long standing SLE initially not recognized as APS-2 syndrome, and its associated disorders. Case: A 62-year old female with history of SLE complicated by pulmonary and renal involvement, hypothyroidism, AI hemolytic anemia, Raynaud’s phenomenon, fibromyalgia, was referred to endocrinology for worsening hyperglycemia. Patient had previous diagnosis of diabetes mellitus (DM) type 2; treated with metformin. She was taking chronic prednisone at varying doses (5mg BID to 60mg daily) depending on activity of SLE. The blood glucose levels significantly increased when the steroid dose was increased due to Lupus pneumonitis, and she subsequently developed autoimmune hemolytic anemia with hemoglobin dropping from 12.7 g/dL (normal=12.0-16.0) to 5.8 g/dL in less than one month. The glycosylated hemoglobin had increased from 5.7% to 8.0% with blood glucose values up to 400’s mg/dL, while hospitalized. It was assumed that insulin resistance had worsened due to high steroid doses of methylprednisolone 125mg every 8 hrs. She was then started with basal and bolus insulin. Although previously negative, the glutamic acid decarboxylase antibody (GAD65) became positive, suggesting type 1 DM. Discussion: Although, there are reports of SLE associated with APS-2, physicians should monitor for other autoimmune endocrinopathies, such as type 1 DM, autoimmune thyroiditis and adrenal insufficiency. Chronic treatment with steroids can mislead physicians in assuming that a patient is developing increasing insulin resistance due to steroids, and not make a correct diagnosis of newly developed type 1 DM. An interesting observation made in this case is also the timeline of Lupus flare and development of type 1 DM and the associated other complications - autoimmune hemolytic anemia and Lupus pneumonitis. It could be hypothesized that increased autoreactivity of the patient’s immune system foreshadowed SLE- flare and might have been responsible for production of additional antibodies (GAD65) leading to development of type 1 DM.

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