Moreover, patient health status often remains impaired, with persistent fatigue, depressive symptoms and even a higher risk of mortality, thus demonstrating the limitations of physiological replacement therapy 4- 7. Although this treatment is usually well adapted to prevention of an acute adrenal crisis, the frequency of crises has nonetheless been evaluated at 5–6 crises/100 patient years 2, 3. Mineralocorticoid replacement is also needed in patients with primary adrenal insufficiency to prevent orthostatic hypotension 1. Treatment requires systematic glucocorticoid replacement with the need to double the dose during an acute stress.

Hormonal diagnosis is based on an insufficient level of baseline cortisol and a failure to respond to a dynamic stimulation by ACTH (Synacthen) injection.

The main features of the disease are a low blood pressure level, fatigue and gastrointestinal upset as well as impaired quality of life and, in case of associated type-1 diabetes, an increased risk of mild and severe hypoglycaemia. Autoimmune adrenalitis may be isolated or associated with other autoimmune disorders as a component of polyendocrinopathy type 1 and 2. AI could be primary (mainly autoimmune adrenalitis, tuberculosis and congenital adrenal hyperplasia) or secondary (mostly due to hypothalamic–pituitary tumors). The overall prevalence is five in 10 000 of the population.

single photon emission computed tomography/computed tomographyĪdrenal insufficiency (AI) is a rare but potentially life-threatening disease 1.

Transplantation of the adrenal gland en bloc with the left kidney appears to be a good therapeutic option in patients with adrenal insufficiency awaiting kidney or kidney–pancreas transplantation. Functional imaging at 3 months showed good uptake of -metaiodobenzylguanidine by the adrenal graft. The adrenal graft functioned well at 12 months with a normalization of cortisol and aldosterone baseline levels.

One-year kidney and pancreas graft functions were satisfactory (estimated glomerular filtration rate: 55 mL/min/1.73 m 2 and HbA1c: 4.8%). We observed no additional morbidity due to the adrenal gland transplantation, as there were no surgical complications. The immunosuppressive regimen was not modified by the addition of the adrenal gland. En bloc left adrenal gland and kidney grafts were anastomosed on the left iliac vessels in normal vascular conditions and the pancreas graft was anastomosed on the right iliac vessels. We report here the 1-year outcome of the first simultaneous kidney–adrenal gland–pancreas transplantation in a 33-year-old patient with type 1 diabetes and concomitant autoimmune adrenal insufficiency. Replacement therapy sometimes fails to prevent an acute adrenal crisis and most often does not lead to restoration of well-being. Adrenal insufficiency is a rare but life-threatening disease.

Adrenal gland and kidney function