A CASE-CONTROL STUDY ON AUTOIMMUNE POLYENDOCRINE SYNDROMES IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

PV211 / #363 Poster Topic: AS23 - SLE-Diagnosis, Manifestations, & Outcomes Background/Purpose Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can impact multiple organs, including joints, kidneys, skin, heart, blood cells, lungs, and nervous system. SLE patients face a higher risk of various comorbidities and treatment-related complications, with an increased mortality rate compared to the general population. Studies on observational cohorts have identified cardiovascular diseases, diabetes mellitus type 2 (T2DM), osteoporosis, certain types of cancer, and autoimmune endocrine disease, such as Hashimoto’s thyroiditis (HT), Graves’ disease (GD), type 1 diabetes mellitus (T1DM) and hyperparathyroidism, as common cause of morbidity in SLE patients. However, to our knowledge, no data currently exist on the connection between SLE and Autoimmune Polyendocrine Syndromes (APS), which are rare diseases characterized by multiple autoimmune conditions affecting at least 1 endocrine organ. APS 1 is due to gene AIRE mutations; APS 2 is characterized by Addison’s disease (AD) associated with HT or GD and/or T1DM; HT or GD with any other autoimmune diseases (excluding AD and hypoparathyroidism) fall under APS 3; APS 4 includes remaining combinations of autoimmune forms having an impact on endocrine organs. This study aimed to investigate the prevalence of Autoimmune Polyendocrine Syndromes (APS) in patients with Systemic Lupus Erythematosus (SLE) and to assess whether APS predicts higher disease activity or worse outcomes. Methods Clinical charts of 417 SLE patients referring to our Center between 2021 and 2023 were analyzed. APS cases were identified using ORPHA code definitions; 185 APS-free SLE patients, randomly enrolled, served as controls. Demographic, clinical and serological data were collected. Results Forty-seven (11%) SLE patients have another autoimmune disease affecting the glands that allows the diagnosis of APS: 39 were diagnosed with HT, 6 with GD, and 3 with T1DM. Forty-five patients were affected by APS type 3, and 2 by APS type 4; no patients were diagnosed with APS type 1 or 2. Table 1 show the sequence in which autoimmune diseases manifest. SLE was the first manifestation of APS in 22 patients (47%). HT was the first autoimmune manifestation for 21 (45%) patients, GD was the first for 2 (4%) patients and 2 women started with rheumatoid arthritis (2%) and autoimmune urticaria (2%), respectively. SLE was the second manifestation in 23 (49%) patients and the fourth for 2 (4%) patients. The comparison between APS+ and APS- patients, as shown in Table 2, revealed no significant differences in clinical or serological features, except for pulmonary hypertension (p=0.044) and renal microangiopathy (p=0.044). At the last evaluation, approximately 80% of both groups’ patients were in clinical remission and approximately half of the patients were still on steroid therapy. APS+ patients had a slightly higher median damage index (SLICC-SDI), although this was not associated with increased disease activity. Table 1. Sequence of autoimmune diseases presentation in patients with SLE and APS Table 2. Comparision of data in SLE patients with and without APS Conclusions The prevalence of APS among SLE patients is significantly higher than in the general population (11% vs 0,005%), confirming the well-known association between autoimmune thyroiditis and SLE. However, APS+ patients do not appear to have a more aggressive disease or develop more complications. The only clinical conditions statistically associated with APS (renal microangiopathy and pulmonary hypertension) are so rare that no definite conclusions can be drawn. Limitations of the study include a small sample size and single-timepoint data, highlighting the need for larger multicenter studies to clarify the link between SLE and APS.

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