Endocrine Abstracts

Introduction

Autoimmune Polyendocrine Syndrome (APS) is defined by the presence of 2 or more autoimmune induced endocrine failures in a single patient. There are 4 types of APS: type1 including chronic muco-cutaneous candidiasis, hypoparathyroidism, Addison disease (AD) and ectodermal dystrophy, APS type 2 and 3 containing autoimmune thyroide disease (AITD), type1 diabetes (DT1) with (type2) or without adrenal failure (type3) and type 4 not falling into the above categories. The aim of our study was to precise the prevalence of APS in a group of patients with AITD and their clinical and serological evaluation.

Methods

Retrospective study including 46 patients suffering from APS among 113 with AITD was performed in internal medicine and/or endocrinology department hospital from January 2000 to December 2017.

Results

46 patients (40%) with AITD had APS. 60% of population was female (sex-ratio = 1.87). The mean age was 34.13 years [14–65 years]. We noticed history of thyroid disease in 26% of cases and family auto-immune disorder in 8.6%. In our study, we identified 7 cases of APS type2 and 39 cases of APS type3. AITD was type Grave’s disease in 10 cases, Hashimoto thyroiditis at hypothyroidism stage in 23 cases, hyperthyroidism stage in 6 cases and euthyroidism in 7 cases. On clinical presentation, APS manifests itself as one of the major autoimmune diseases: adrenal failure and/or DT1 and/or hypo/ hyperyroidism in all cases. Diabetes preceded APS in 50% of cases. Minor autoimmune disorder type Vitiligo was diagnosed preceding the development of APS in 2 cases and concomitantly in 1 case. Thyroid gland was enlarged in 67% of cases. Serum analysis showed diabetes in 56.5%, hypothyroidism in 50%, hyperthyroidism in 35%, euthyroidism in 15% and adrenal insufficiency in 15.2% of patients. Immunologic investigations showed Thyroid peroxidase autoantibodies in 91%, thyroglobilin-autoantibodies in 43%, Glutamic-Acid-Decarboxylase autoantibodies in 67%, TSH-receptor autoantibodies in 21.7%, adrenal autoantibodies in 16.2%, antinuclear antibodies in 6.5%, antigliadin antibodies in 4.3%, anti-ovarian, antiparietal-cell and anti-transglutaminases auto antibodies in 2.1% respectively. Multiple autoimmune syndrome (type3) was retained in 6 patients (13%). Extra endocrine auto-immune disorders founded were: vitiligo (3 cases), lupus, celiac disease and pernicious anemia (1 case each).

Conclusion

Prognosis of APS depends on individual glandular failures. Considering the high incidence of APS in patient with family and/or personal autoimmune history, regular screening in specialized centers must be performed. In our series containing 46 cases of AITD with APS, type3 was more observed (84.8%) unlike other studies in which APS type2 was more prevalent.