Endocrine Abstracts

Introduction: Autoimmune polyglandular syndromes (APS) are rare polyendocrinopathies characterized by the failure of several endocrine glands as well as nonendocrine organs, caused by an immune-mediated destruction of endocrine tissues. The type IIIa APS is the association of thyroid autoimmune diseases, type 1 diabetes mellitus and other autoimmune conditions excluding Addison’s disease and hypoparathyroidism. Herein, we report the case of autoimmune hypothyroidism associated to type 1 diabetes, systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC) in a young woman.

Case report: A 35 years-old woman with a medical history of type 1 diabetes treated by insulin since the age of 9 years and autoimmune hypothyroidism diagnosed at the age of 25, presented to our internal medicine department with inflammatory polyarthralgia of the hands, wrests, ankles and shoulders accentuated in the last 4 months. The physical examination showed a butterfly shaped rash of the face, red spots on exposed areas that worsen with sun exposure and oral ulcerations. The blood investigations showed an accelerated erythrocyte sedimentation rate (VS) (80 mm/h), a normal blood white count with no lymphopenia and a normal serum calcium level. The X-rays of the aching joints didn’t show erosions or pinches. The immunology tests showed positive anti-nuclear antibodies (ANA) (1/400) and anti-double-stranded DNA (anti-DNA). The SLE was diagnosed according to the criteria of the SLICC with a score of 5 points, ACR with a score of 4 points, EULAR/ACR 2019 with 20 points. The patient was started on hydro-chloroquine 400 mg a day and corticosteroids 10 mg a day. Further investigations were pursued showing an immunological profile compatible with PBC that was inactive at the moment of the diagnosis. The diagnosis of APS type IIIa was made after excluding an adrenal insufficiency by a normal 250 µg synacthen test.

Conclusion: We report the case of a female with type 1 diabetes, auto-immune hypothyroidism, SLE and PBC, which is a very rare combination. We present this case as evidence for the coexistence of several different immune-mediated diseases in the clinical context of a APS type IIIa. Thus, a regular follow up of these patients is needed to detect and treat the other auto-immunes disorders at an early stage.