Endocrine Abstracts

Introduction: The majority of systemic diseases result from an autoimmune process. Primary adrenal insufficiency (Addisson’s disease AD) is also frequently caused by an autoimmune process. Their association is rare but can be seen in the framework of autoimmune polyendocrinopathies. We report 2 observations associating an autoimmune adrenal insufficiency with a systemic disease.

Observation 1: We describe the case of a 50-year-old patient initially admitted to the intensive care unit for vomiting, diarrhea and acute dehydration. Physical examination showed melanoderma and hypotension at 80/60 mm hg.A baseline blood sample revealed hyponatremia (127 mmol/l) and hyperkalemia (5.6 mmol/l). Thus, Acute adrenal insufficiency was suspected. The patient immediately received intravenous hydrocortisone hemisuccinate with hyperhydration. The diagnosis of AD was confirmed by a low plasma cortisol level (50 nmol/l) in the face of high adrenocorticotropin hormone (ACTH) level=1250 pg/ml). Concerning the etiological investigation, the detection of BK in the sputum analysis was negative, the chest X-ray was normal and the adrenal glands were eutrophic in the Computed tomography. However, the anti 21 hydroxylase antibodies were positive. In the framework of the search for other autoimmune diseases, we found a thyroid autoimmunity with positive ATPO of 412 UI/ml. Antinuclear antibodies, anti SSA, anti SSB and anti R052 were also positive. The patient was referred to the department of internal medicine for management of a Goujerot Sjogren syndrome in the context of very likely systemic lupus erythematosus. Observation 2 We report the case of a 27-years-old patient who was diagnosed three years ago with Behçet’s disease (BD) on the basis of the symptoms of BD triad, which included recurrent oral and genital aphthous, arthralgia and iritis. Recently, he consulted in our endocrinology department for recurrent hypoglycemia. The baseline cortisol level was low(152 nmol/l) and the adrenal insufficiency was confirmed by a stimulation test. ACTH level was high which indicated Addison’s disease.

Discussion: Gougerot-Sjogren’s syndrome is a systemic disease associated with a humoral immune response. The coexistence with AD has been reported in the literature. Several mechanisms could explain this association such as thrombosis of adrenal vessels, hemorrhage due to anti-phospholipid syndrome, vasculitis or direct organ involvement by an autoimmune process BD pathophysiology is controversial with conflicting data. It is believed that BD disturbs the regulation of immune system. Furthermore, few studies correlate BD with AD and found partially dysfunctional adrenal glands in BD patients.