AB0645 PREVALENCE AND DESCRIPTION OF AUTOIMMUNE POLYENDOCRINE SYNDROMES IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: EXPERIENCE OF A SINGLE CENTER

Abstract

BackgroundAutoimmune polyendocrine syndromes (APS) are a heterogeneous group of clinical conditions characterized by functional alteration of one or more endocrine glands and often associated with other systemic autoimmune diseases. Four types of APS have been described. APS-1 is a rare autosomal recessive disease due to mutation in the AIRE gene and characterized by at least two of the following manifestations: chronic mucocutaneous candidiasis, hypoparathyroidism and Addison’s Disease (AD). APS-2 is characterized by AD associated with Diabetes Mellitus Type 1 (DM1) and/or autoimmune thyroid disease. APS-3 involves the association of autoimmune thyroiditis and other organic or systemic autoimmune manifestations, excluding hypoparathyroidism and AD. APS-4 is characterized by autoimmune activity against an endocrine organ in combination with at least one more endocrine or non-endocrine organ. In literature, data regarding prevalence and incidence of APS are poor, especially in the Rheumatology field.ObjectivesThe primary objective of the work is to describe the prevalence of APS in a cohort of patients with Systemic Lupus Erythematosus (SLE) followed in our Department and to assess the possible association between organic autoimmune diseases and SLE. The secondary aim is to describe the timing of onset of the different autoimmune diseases and the presence of associated comorbidities and/or any feature that may predict the development of APS.MethodsIn this retrospective observational study we included the medical history, biochemical, and immunological data of patients older than 18 years of age with SLE who are actively followed at the Department of Rheumatology and Clinical Immunology of our hospital. Patients who, in addition to the diagnosis of SLE, had at least one other autoimmune disorder against an endocrine organ were included in the study.ResultsThe total number of SLE patients examined is 423, with a mean age at the last evaluation of 53 (±13), 393 were female (93%) and 30 male (7%). Forty-eight patients (11.3%) have at least one other endocrinological autoimmune disease that would allow the diagnosis of APS. The sample examined is composed of 46 (96%) females and 2 (4%) males (F:M=23:1). The mean age of the patients is 52 (±14) years; the mean age of SLE onset is 36 (±14) years. No cases of APS-1 nor APS-2 were found; 45 (94%) patients are affected by APS-3; while 3 (6%) by APS-4. Forty-three (90%) patients presented two autoimmune diseases and 5 (10%) presented three autoimmune diseases. The mean age at onset of the first disease is 31 (±12) years; the second manifestation is 39 (±13) years and the third manifestation is 37 (±11) years. Concerning the timing of the onset, SLE was the first manifestation in 17/48 patients, the second manifestation in 25 patients, the third manifestation in 1 patient and in 5 patients the diseases were diagnosed at the same time.Figure 1shows the different conditions according to the time of onset. The mean latency between the onset of the first manifestation and the onset of the second is 9 (±9) years. The mean latency between the onset of the second manifestation and the onset of the third is 4 (±3) years.Figure 1.Time of onset of APS different pathologiesConclusionIn our cohort of SLE patients, the prevalence of APS is quite high and occurs proportionally more in females than in males. Hashimoto’s Thyroiditis (HT) is the most represented disease, followed by Graves’ disease and DM1. In most cases, SLE is not the first autoimmune disease that appears in these patients. In most cases, the onset pathology is autoimmune thyroiditis. Furthermore, although the available data are scarce, in our cohort the latency between the onset of the first and second autoimmune pathology is significantly longer than the latency between the second and third manifestation.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.