Endocrine Abstracts

Introduction: The reactivation of autoimmune disorders has been described in patients with hypercortisolism after normalization of cortisol secretion. This phenomenon is probably related to the loss of immunosuppressive effect of endogenous glucocorticoids. This is a report of two patients with Takayasu arteritis (TA) and Cushing’s disease (CD), a novel association never described in literature, in whom TA was exacerbated after normalization of cortisol secretion.

Case report: Two women, 55 and 53 years old, presented with a clinical syndrome suggestive of hypercortisolism, in whom a diagnosis of CD was performed (urinary free cortisol: 188 and 264 mcg/day respectively), reported a history of TA, complicated by acute coronary syndrome, peripheral vascular disease and chronic renal failure. Not considered candidates for pituitary surgery, they were treated with inhibitors of adrenal steroidogenesis. Six months later, they had a normalization of daily cortisol excretion (urinary free cortisol: 98 and 118 mcg/day) but complained typical symptoms of a systemic vascular inflammatory process: fatigue, weight loss, fever and arthromyalgia; in one of the two patients, the strong inflammatory factor, inhibiting erythropoiesis, in association with iron and vitamin B12 deficiency, caused severe anemia (Hb 4 g/dl), requiring blood transfusions. The clinical picture, the strong increase of inflammatory markers such as VES, C-reactive protein and fibrinogen, and signs of vascular insufficiency such anisosphygmia in femoral and tibial pulses, in bilateral carotid axes and absence of right brachial and radial pulses, have suggested a process of reactivation of the TA. Treatment with corticosteroids was necessary to relieve the clinical symptoms of TA and to reduce the inflammatory markers.

Conclusions: These cases, particularly challenging because of the two concomitant disorders, CD and TA, confirm the crucial role of cortisol secretion reduction in the reactivation of the autoimmune process, supporting the hypothesis that TA activity was suppressed by endogenous glucocorticoids excess. This evidence suggests that it is mandatory to investigate on the possible development or reactivation of autoimmune diseases after normalization of cortisol secretion in patients with a history of Cushing’s syndrome.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.