A case–control study on autoimmune polyendocrine syndromes in patients with systemic lupus erythematosus

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OBJECTIVES: This study aimed to investigate the prevalence of autoimmune polyendocrine syndromes in patients with SLE and to assess whether autoimmune polyendocrine syndromes predict higher disease activity or worse outcomes. METHODS: The clinical charts of 417 SLE patients (meeting SLICC 2012 and/or EULAR/ACR 2019 criteria)( referred to our centre between 2021 and 2023 were analysed. Autoimmune polyendocrine syndrome cases were identified using ORPHA definitions; 185 autoimmune polyendocrine syndrome-free SLE patients, randomly enrolled, served as controls. Demographic, clinical and serological data were collected. RESULTS: Forty-seven of 417 (11%) SLE patients had another autoimmune disease affecting the glands that allowed the diagnosis of autoimmune polyendocrine syndrome: 39 were diagnosed with Hashimoto thyroiditis, 6 with Graves' disease, and 3 with type 1 diabetes mellitus. Forty-five were affected by autoimmune polyendocrine syndrome type 3, and 2 by autoimmune polyendocrine syndrome type 4; no patients were diagnosed with autoimmune polyendocrine syndrome types 1 or 2. A comparison between the autoimmune polyendocrine syndrome-positive and -negative patients revealed no significant differences in clinical or serological features. At the last evaluation, ∼80% of both groups of patients were in clinical remission, and approximately half of the patients remained on steroid therapy. Autoimmune polyendocrine syndrome-positive patients had a slightly higher median SLICC Damage Index (SDI), although this was not associated with increased disease activity. CONCLUSION: The prevalence of autoimmune polyendocrine syndrome among SLE patients is significantly higher than in the general population (11% vs 0.005%), confirming the association between autoimmune thyroiditis and SLE. However, autoimmune polyendocrine syndrome-positive patients do not appear to have a more aggressive disease or develop more complications.

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